Children With Seizures: A Guide For Parents, Teachers, And Other Professionals

Children with Seizures

by the same author Kids in the Syndrome Mix of ADHD, LD, Asperger’s, Tourette’s, Bipolar, and More! The one stop guide for parents, teachers, and other professionals Martin L. Kutscher MD, with contributions from Tony Attwood PhD and Robert R. Wolff MD Hardback ISBN 1 84310 810 0 Paperback ISBN 1 84310 811 9

of related interest Asperger's Syndrome A Guide for Parents and Professionals Tony Attwood Foreword by Lorna Wing ISBN 1 85302 577 1

Tics and Tourette Syndrome A Handbook for Parents and Professionals Uttom Chowdhury Foreword by Isobel Heyman ISBN 1 84310 203

Understanding Autism Spectrum Disorders Frequently Asked Questions Diane Yapko ISBN 1 84310 756 2

The ADHD Handbook A Guide for Parents and Professionals on Attention Deficit/Hyperactivity Disorder Alison Munden and Jon Arcelus ISBN 1 85302 756 1

Children with Seizures A Guide for Parents, Teachers, and Other Professionals

Martin L. Kutscher MD Foreword by Gregory L. Holmes MD

Jessica Kingsley Publishers London and Philadelphia

First published in 2006 by Jessica Kingsley Publishers 116 Pentonville Road London N1 9JB, UK and 400 Market Street, Suite 400 Philadelphia, PA 19106, USA www.jkp.com Copyright © Martin L. Kutscher 2006 Foreword copyright © Gregory L. Holmes 2006

Disclaimer: This information is for educational purposes and does not constitute medical advice; nor is it a substitute for discussion between patients and their doctors. This text is not intended to be all-inclusive or to set medical standards. Like most areas of information, recommendations and knowledge about the care of seizures are subject to debate and likely to change over time. The views of cited references do not necessarily represent the views of the authors. Not all of the medication usages discussed in this book are US FDA approved. The right of Martin L. Kutscher to be identified as author of this work has been asserted by him in accordance with the Copyright, Designs and Patents Act 1988. All rights reserved. No part of this publication may be reproduced in any material form (including photocopying or storing it in any medium by electronic means and whether or not transiently or incidentally to some other use of this publication) without the written permission of the copyright owner except in accordance with the provisions of the Copyright, Designs and Patents Act 1988 or under the terms of a licence issued by the Copyright Licensing Agency Ltd, 90 Tottenham Court Road, London, England W1T 4LP. Applications for the copyright owner’s written permission to reproduce any part of this publication should be addressed to the publisher. Warning: The doing of an unauthorised act in relation to a copyright work may result in both a civil claim for damages and criminal prosecution.

Library of Congress Cataloging in Publication Data Kutscher, Martin L. Children with seizures / Martin L. Kutscher ; foreword by Gregory L. Holmes. p. cm. Includes bibliographical references and index. ISBN-13: 978-1-84310-823-8 (pbk.) ISBN-10: 1-84310-823-2 (pbk.) 1. Epilepsy in children. 2. Convulsions in children. I. Title. RJ496.E6K87 2006 618.92’853—dc22 2005035130

British Library Cataloguing in Publication Data A CIP catalogue record for this book is available from the British Library ISBN-13: 978 1 84310 823 8 ISBN-10: 1 84310 823 2 ISBN pdf eBook: 1 84642 490 9

Printed and bound in Great Britain by Athenaeum Press, Gateshead, Tyne and Wear

To children and their parents, For their resiliency and courage. And to my amazing wife.

CONTENTS FOREWORD INTRODUCTION

9 11

PART 1: OVERVIEW OF SEIZURES AND THEIR CARE

1.

Overview of Seizures

17

2. What to Observe During a Seizure

26

3. What to Do During a Seizure

30

4. Accident Precautions

32

5. Medical Evaluation of Seizures

34

6. To Treat or Not, and When to Stop?

42

7. Treatment with Medications

47

8. Treatment Options other than Medication

66

9. For Kids to Read with Their Parents: What Are Seizures, Anyway?

70

10. Chapter on (and for) Teens with Epilepsy

76

Marissa A. Broadley and Martin L. Kutscher

PART 2: INDIVIDUAL EPILEPSY SYNDROMES

11.

Introduction to the Epilepsies

12. Idiopathic Focal Epilepsies: Benign Childhood Epilepsy with Centrotemporal Spikes (BCECTS)

87 91

Marissa A. Broadley and Martin L. Kutscher

13. Idiopathic Focal Epilepsies: Childhood Occipital Epilepsies (COE)

95

14. Symptomatic Focal Epilepsies: The Temporal Lobe Epilepsies

100

Martin L. Kutscher with Zachary Gottlieb

15. Symptomatic Focal Epilepsies: Other Types

104

16. The Generalized Epilepsies: Childhood and Juvenile Absence Epilepsy

108

Martin L. Kutscher with Eric Kutscher

17. The Generalized Epilepsies: Juvenile Myoclonic Epilepsy (JME)

113

Marissa A. Broadley

18. The Generalized Epilepsies: Idiopathic Epilepsy with Generalized Tonic-clonic Seizures Only

116

19. The Generalized Epilepsies: Infantile Spasms (West Syndrome)

118

20. The Generalized Epilepsies: Lennox–Gastaut Syndrome

126

21. Seizures Not Requiring the Diagnosis of Epilepsy: Febrile Seizures

132

22. The History of Epilepsy: A Timeline

139

Eric Kutscher REFERENCES

141

FURTHER READING

144

ABOUT THE AUTHORS

147

SUBJECT INDEX

148

AUTHOR INDEX

151

FOREWORD

My introduction to epilepsy occurred during my freshman year of high school. This experience remains quite vivid even 40 years later. One day during our homeroom period, a classmate had a generalized tonic-clonic seizure. She fell out of her chair, began jerking violently, and had urinary incontinence. Along with my other classmates, I was stunned and frightened, having no idea as to how to help her. Our teacher was as frightened and helpless as we were, and provided no aid or comfort to the student or our class. Fortunately, the seizure was short-lived, and my classmate was carried out of the room and taken to the nurse’s office—much to our relief. Sadly, this was only the first of several seizures to occur in our classroom. The response of our class to subsequent seizures was to pretend that the seizures were not occurring and ignore the attacks. As a consequence of our ignorance, we also shunned our classmate, avoiding her as much as we could. Despite her desire to be a normal student, we never gave her the opportunity to be a normal high school student. While I do not know what happened to her, I do know that due to our ignorance about her condition, her high school experience must have been emotionally devastating. I only wish that our teacher and ourselves had had access to accurate information about epilepsy at that time. Knowing the truth about epilepsy would have dramatically changed our attitude and approach to our unfortunate classmate. Dr. Kutscher, in this marvelous book, dispels many of the myths of epilepsy and provides a wonderful review of all aspects of the disorder. He effortlessly moves from diagnosis to treatment to prognosis. Employing humor, common sense, and an outstanding knowledge of epilepsy, Dr. Kutscher’s book is truly a tour de force. Teachers, parents, siblings, and friends of children with epilepsy will benefit greatly from this book. While the book is written for the non-physician, I believe that many healthcare workers would benefit from this wealth of current and

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accurate information. Certainly, physicians caring for children with epilepsy will want to recommend the book to families and teachers. Children with Seizures provides the reader with an excellent review of the types of seizures, their causes, and treatment. While epilepsy spares no race, gender, or age, there are unique features of epilepsy in children. Dr. Kutscher comprehensively covers the childhood aspects of epilepsy, emphasizing practical issues that arise on a daily basis with children with seizures. While the diagnosis and treatment of childhood epilepsy can be challenging, Dr. Kutscher brings remarkable clarity to the condition. Dr. Kutscher’s book provides answers to questions that often go unanswered at doctors’ appointments. Important topics such as the rationale for diagnostic tests, role of generic medications, dietary and surgical therapy, and school issues are all discussed in depth. It is unlikely that readers will put down the book with unanswered questions. Is epilepsy always a benign condition? No. While most children with epilepsy do well, some children with epilepsy will suffer from recurrent seizures despite excellent neurological care. While many books on epilepsy directed to non-physicians tend to ignore the more serious conditions, this is not the case here. Dr. Kutscher provides current information on both the benign conditions and the difficult to control epilepsies. For individuals involved with children with severe epilepsy, this book provides information that cannot be obtained elsewhere. Hippocrates was the first to dispel the notion that epilepsy was a sacred disease, cast upon individuals by the gods. Hippocrates argued that epilepsy was no different from other chronic disorders and should not be shrouded in mystique. Thanks to the efforts of compassionate physicians such as Dr. Kutscher, epilepsy is no longer a mysterious disorder cloaked in ignorance. I am delighted that Dr. Kutscher has provided us with this gem. My only regret is that such a book was not available 40 years ago. Gregory L. Holmes MD Professor of Medicine (Neurology) and Pediatrics Dartmouth Medical School Chief of Neurology Dartmouth-Hitchcock Medical Center Lebanon, New Hampshire

I N T RO D U C T I O N

If you’re reading this… Let’s face it: seizures can be scary to watch. If you made it to this book, then you—or someone you know—probably has been through an experience where it seemed like the child might be seriously ill, or even dying. That experience is finished now, but it may take months for family and friends to get over it. The good news is that for most children with seizures, everything usually works out fine.

Knowledge is an antidote to fear One way to handle a problem (and the fear it engenders) is to become knowledgeable about the area. This book will give you the information that you need to approach a child’s seizures from a position of strength. Along the way, we will explain some of those technical terms that you will be hearing. The knowledge will help you to feel more in control. As we discuss the material, we’ll try to be reassuring, informal, hopeful, and even upbeat.

The scope of the problem There are a lot of people in this world who need this information. One out of ten people will have a seizure at some point in their life. In the US, there are 2,500,000 people affected by seizures (Epilepsy Foundation of America 2005). At any given moment, one out of every 150 people will have a seizure disorder (Devinsky 2002, p.48). Let’s put that into perspective: it means that as many as 600 fans at the Rose Bowl stadium have a seizure disorder. You might ask, “If so many people have epilepsy, why don’t I know lots of them?” The simple answer is that you do. You just don’t know who they are, because they are usually normal people who lead normal lives.

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Who is this book for? This book is intended as an educational resource for parents and other family members, as well as for teachers and therapists who work with children who have seizures. This would include classroom teachers, special education teachers, psychologists, nurses, speech therapists, physical therapists, and occupational therapists. Although this book is specifically not a training manual for physicians, it will, we hope, serve as a useful resource for doctors to recommend to the family and to others who are involved in the child’s care.

Organization of this book Part 1 provides an overview of seizures and the care of children who have them. We will learn about types of seizures, what causes them, what to look for during a seizure, and what to do during a seizure. We’ll discuss how seizures are evaluated, the role of tests such as electroencephalograms (EEG) and MRI (Magnetic Resonance Imaging) scans, the decision to treat with medication or not, and for how long to treat. Anticonvulsant and other therapies are discussed. Next, we’ll address some of the emotional and social issues facing the kids and their parents. There are chapters that kids and teens can read by themselves. Parents will want to read those chapters as well—preferably with their child—since they contain very important information regarding topics such as video games, dating, and driving. In Part 2, we will cover individually some of the most common epilepsy syndromes in detail. How is each syndrome diagnosed? Which epilepsy syndromes need treatment? What medications work best for each of these syndromes? What can we expect for the future? Finally, we will provide selected further readings (books and Internet sites) along with references.

Cause for humility on the part of doctors It is easy for a doctor to sit calmly at his or her computer to write about someone else’s child having a seizure. It is another thing to watch your own child go through episodes that can seem very frightening, especially when you are alone or without support. I have the greatest respect for parents who carry on with what they have to do. I am also humbled by what we, as doctors, still do not know for sure. As I have prepared this book, I have tried to keep in mind what my professor said to us on the first day of medical school: “One third of what I am going to teach you this year is wrong… Unfortunately, I don’t know which third.” Studies involving real people

INTRODUCTION

13

may not achieve the predictable results of a simple chemistry experiment. Nonetheless, there is still much that we do know, which can successfully help us plan our expectations and course of action.

Is it going to be okay? Before we start, you may just want to quickly know, “Is it going to be okay?” The quick and oversimplified answer is “Typically, yes!” Most children outgrow their seizures. Unlike adults, most children’s seizures are not due to something bad such as a brain tumor or stroke. Typical seizures are unlikely to cause harm to the brain. Only a minority of children with seizures have mental retardation or cerebral palsy—but they are not caused by typical seizures, and you would have probably known about those problems already. So, take a deep breath. Find out what you need to know, and do what you need to do. You will make it through this: there’s no other choice. We’ll try to keep it brief. Good luck!

Part 1

OV E RV I E W O F SEIZURES A N D TH E I R C A R E

Chapter 1

OV E RV I E W O F S E I Z U R E S

What is a seizure? In four words, a seizure is a “temporary brain short circuit.” The brain cells get caught up in a reverberating cycle, and cause the body to enact the events that we interpret as a “seizure.” Here’s a bit more detail. The brain consists of billions of neurons, communicating with each other via electrical impulses. When one brain cell is sufficiently stimulated, it sends an electrical current down its long, wire-like axon. When this message reaches the end of the axon, the neuron releases a chemical neurotransmitter that floats across a synapse and lands on a receptor of the next cell—in turn, causing it to fire. Meanwhile, other neurons are putting a damper on all of this activity, trying to keep everything from getting out of control. On occasion, these dampening activities are insufficient, and the brain cells essentially get themselves into a reverberating short circuit. The brain cells fire—causing the muscles that they control to contract. This leads to the movements we witness during a typical seizure. Fortunately, the brakes usually regain control. Each time such an event occurs, the person is said to have experienced a seizure. Sometimes, the part of the brain that is involved in the seizure controls something other than muscle activity. In such cases, the person having the seizure may experience an unusual sensation, have an altered level of alertness, or do whatever is controlled by that part of the brain. Sometimes, the seizure focus spreads from one part of the brain to others, causing the manifestation of the seizure to change as the seizure evolves. A seizure may have up to three parts: possibly an introductory “aura,” the actual “ictal” seizure event, and possibly a “post-ictal” state. If present, the aura is the patient’s initial experience while the seizure is just beginning. During an aura, the uninvolved part of the brain is “watching” the other part of the brain have the

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CHILDREN WITH SEIZURES

seizure. Once the short circuit has spread as much as it will, the rest of the actual brain short circuit is called the “ictal” event or “seizure.” After the actual seizure itself, there may or may not be a period of transiently altered brain function called the “post-ictal” period. During the post-ictal period, the parts of the brain that were involved in the seizure may have already spent all of their energy, and might temporarily be unable to perform. The post-ictal period usually consists of sleepiness and/or temporary muscle weakness, and needs to be distinguished from the actual seizure itself. So, that is all that a seizure is. An electrical short circuit in the brain. Nothing more. Nothing less. Nothing to be embarrassed about.

What is “epilepsy?” Saying that a person has “epilepsy” just means that he or she has more than one seizure. That’s it. By itself, epilepsy has nothing to do with being developmentally or psychologically abnormal. The term can be used for people who have a chemical predisposition towards recurrent seizures (“idiopathic epilepsy”), or for people whose seizures are symptoms of some more identifiable cause such as head trauma or meningitis (“symptomatic epilepsy”). Unfortunately, the word “epilepsy” still carries a stigma for some people—even though epilepsy is just a medical issue like asthma or diabetes. Some people may find it easier to use the term “seizure disorder.”

How does the term “seizure” relate to the term “epilepsy?” Note that the term “seizure” refers to the specific neurological event, such as a staring spell or a jerking spell. In contrast, we will refer to “epilepsy” as a syndrome. The term “epilepsy syndrome” refers to a full syndrome consisting of the: type(s) of seizures seen in that syndrome, EEG findings, typical age of patient, typical response to different anticonvulsants, typical prognosis, etc. For example, “absence seizure” refers to the specific event of staring for several seconds. However, “childhood absence epilepsy” (previously called “petit mal epilepsy”) refers to a syndrome of young, neurologically normal children whose EEG shows 3-per-second spike-wave discharges and who are likely to outgrow their seizures. Determination of the correct type of epilepsy syndrome helps guide the work-up, choice of anticonvulsants, and expected outcome for the child. The rest of this chapter will be about the different types of seizures. The epilepsy syndromes are discussed in Part 2.

OVERVIEW OF SEIZURES

19

Introduction to “generalized” and “partial” seizures Seizure events are broadly classified into two types, depending upon how they start. Generalized seizures are those that start from essentially the entire brain all at once. In contrast, partial (also called “focal” or “local”) seizures are those that start from one part of the brain. This distinction between generalized and partial seizures is important for several reasons. It affects first the observations we must make during a seizure; second the medical work-up; and third the treatment of a child with seizures. 1.

We must observe if the seizure has any signature of a focal onset: Did the seizure start in or affect one part of the body more than the others? Was there an “aura,” i.e. a warning at the beginning of the seizure? This is important since a well-defined aura means that the event began as a focal seizure, because the aura of a seizure is actually just its focal onset. In contrast, a generalized seizure does not have a well-defined aura. Why? Well, we will need a slight diversion to explain: consciousness requires the proper functioning of (a) the brainstem and (b) at least one of the two cerebral hemispheres. The brainstem—which is located at the base of the brain—switches the brain “on” and “off,” such as during sleep cycles. Once turned “on,” the cerebral hemispheres make the conscious observations. A generalized seizure affects the entire brain at its onset, and thus affects large portions of both cerebral hemispheres at once. There is no longer at least one brain hemisphere available to be conscious. In other words, in a generalized seizure, there is “no one there” to be aware of the spell. Determination of an aura, then, is extremely useful. We should remember to ask both the observer and the child about the specific onset of the seizure. (Note, though, that the absence of an identifiable aura does not necessarily rule out a focal seizure: the aura or focal onset of a seizure may be so brief that it may not be noticed before there is spread to the rest of the brain. Also, young children may be unable to verbalize an aura, and may just start acting unusually or run to their parent.)

2.

The medical work-up may be affected, since focal seizures are more likely to have underlying focal structural abnormalities.

3.

The treatment is affected, since differing anticonvulsants treat different kinds of seizures.

Each of these broad categories (generalized versus focal seizures) can be further divided into individual kinds of seizures. In Table 1.1, we show the simplified classification of the seizures to be discussed in this chapter. First, we will provide a brief description of the several types of generalized seizures, and then a

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description of the several types of focal seizures. Brief videos of the major seizure types can be viewed at www.epilepsyontario.org (see Further Reading).

Types of generalized seizures Table 1.1 A simplified classification of seizure types

• Generalized seizures (that start initially throughout the whole brain)

° ° ° °

Absence seizures Tonic-clonic seizures Myoclonic seizures Atonic seizures

• Focal seizures (that start initially in part of one hemisphere)

° Simple partial seizures (consciousness remains normal during the seizure)

° Complex partial seizures (consciousness is altered during the seizure)

° Focal seizures that then secondarily generalize

By definition, generalized seizures start diffusely throughout the brain. Thus, all generalized seizures share in common the absence of a well-defined aura, and an altered level of consciousness. The generalized seizures are classified into absence, tonic-clonic, myoclonic, and atonic types. These types are also called “primary generalized seizures,” as distinct from “secondarily generalized” seizures which start in one location and then spread (see next section).

Absence seizures Typical absence seizures are very “clean” seizures. Like all generalized seizures, there is no well-defined aura. They consist of brief (3–30 second) staring spells, accompanied by a halting of activity. Typically, the person does not fall to the ground during an absence seizure. The person freezes in mid-activity, and stares with a vacant, glazed look. Sometimes, there is some mild eye fluttering, mild lip movements, or twitches. There is no post-ictal state after a typical absence seizure. This type of seizure used to be called “petit mal.” There are also “atypical absence” seizures, which may be prolonged or have other unusual, prominent features.

OVERVIEW OF SEIZURES

21

Tonic-clonic seizures A “tonic” seizure refers to the continuous stiffening of the extremities. As the patient’s own seizure brakes come on, the stiffening becomes only intermittent and the patient is seen to have twitching movements. A “clonic” seizure refers to these rhythmic jerking movements that come from the quick contractions alternating with slower relaxation of the muscles. A “tonic-clonic” seizure, then, is one that starts with continuous tonic stiffening, and is then followed by a clonic phase of rhythmic jerks. As the seizure winds down, the clonic jerks become slower and slower—until they stop. Tonic and clonic seizures usually cause clenching of the teeth, and sometimes biting of the tongue or cheek. Loss of consciousness (from a seizure or from other reasons) may also cause a loss of urine control—depending on how much fluid is in the person’s bladder at the time. Tonic-clonic seizures used to be called “grand mal.” Note that partial seizures may have such rapid secondary spread that they may be clinically indistinguishable from true primary generalized tonic-clonic seizures. Myoclonic seizures Myoclonic seizures are brief, startle-like jerks, often occurring in irregular flurries. They might be quick, forward flexion movements that resemble a startle; or, they may be quick, backward extension movements. Myoclonic seizures tend to be associated with drowsy states and waking up. This type of seizure is relatively rare. Atonic seizures Atonic seizures are also called “akinetic seizures” or “drop attacks.” It is as if someone just cut the string to a marionette puppet. The loss of muscle tone is so brief that consciousness has been regained by the time the child hits the floor. The sudden falls place the child at risk for injury. Sometimes a helmet is required for safety. This type of seizure is quite rare.

Types of partial seizures By definition, partial seizures begin in one part of the brain. In effect, they are one big aura. Partial seizures may also be referred to as “focal” or “local” seizures. The partial seizures are classified into three types: simple partial, complex partial, and partial seizures with secondary generalization.

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Simple partial seizures Simple partial seizures begin focally in one hemisphere and, by definition, do not impair the level of consciousness. They may consist of virtually any task of which the brain is capable—such as jerking of just one extremity, abnormal sensation of one part of the body, seeing spots, a feeling of fear or déjà-vu, etc. Complex partial seizures Complex partial seizures (previously called “psychomotor seizures” or “temporal lobe seizures”) begin focally in one hemisphere and do impair the level of consciousness. There is usually a well-defined aura followed by a confused trance. Typically, there is also lip smacking, eye fluttering, or fumbling/picking movements of the hands. These seizures usually last at least several minutes. Complex partial can sometimes be confused with absence seizures, since both types are marked primarily by a trancelike state. They can often be distinguished by the following criteria in Table 1.2. Table 1.2 Distinguishing absence from partial complex seizures Distinguishing criteria

Absence seizure

Partial complex seizure

Aura

Absent

Present (It is an aura!)

Duration of seizure

3–30 seconds

Several minutes or more

Lip smacking, fumbling, eye fluttering

Absent or mild

Prominent

Post-ictal confusion

Absent

Prominent

Partial seizures with secondary generalization A partial seizure may later spread to involve the rest of the brain—a process called “secondary generalization.” Although the secondary generalization may be the most striking feature to the family, it is the partial onset of these seizures that matters most to the doctor. A “Jacksonian march” is the label given to a seizure that spreads along the brain’s cortex—with resultant spread of the clinical seizure along the opposite side of the body. The children may be aware of this focal spread along one side, and then lose consciousness as the seizure spreads to the other hemisphere during secondary generalization.

OVERVIEW OF SEIZURES

23

Note that seizures that start initially throughout the brain are called “primarily generalized,” whereas seizures that start focally and then spread to the rest of the brain are referred to as “focal seizures with secondary generalization.”

Whatever happened to the names “petit mal” and “grand mal?” The names “petit mal” and “grand mal” are gone, along with the imprecise knowledge about seizure types that led to them. Tonic-clonic seizures used to be called “grand mal” (meaning “big malady” in French). Everything else was lumped together as “petit mal” (meaning “little malady” in French). However, we have just seen that there are lots of “smaller” seizures: there are true absence seizures, but also myoclonic, atonic, partial simple, and partial complex seizures as well. Thus, the term “petit mal” is not specific enough, and should be abandoned in favor of the correct modern classification as above.

What is “status epilepticus?” “Status epilepticus” is the medical term for a “long” seizure. How long is “long?” That depends upon whom you ask. Status epilepticus is defined by some neurologists as lasting more than 20 minutes; others would use a 30-minute cut-off. The seizure activity can be either one continuous spell lasting more than that time, or a series of shorter seizures without regaining consciousness in between them. Status epilepticus refers to the length of the seizure, not the type of seizure. Thus, for example, the prolonged seizure can consist of clonic activity, absence staring, simple partial, or complex partial activity. Note that, to a neurologist sitting calmly in his or her office, a seizure that lasts five minutes is still considered relatively brief—although those five minutes may seem like an eternity to those watching the spell. Timing the event with a watch is often very helpful to help accurately measure a seizure’s true length.

Status epilepticus is a medical emergency Fortunately, even such prolonged seizures are unlikely to harm the child. Nevertheless, they are not exactly good for you, either. In certain settings, your doctor might recommend having available a rectal form of diazepam (marketed in the US as Diastat) to be administered if the seizure lasts more than a few minutes. Diastat is a pre-filled, pre-measured plastic “syringe” that can be inserted rectally to abort a seizure on its way to becoming prolonged. The procedure is painless, and there are no needles involved! Although diazepam (Valium) given by rapid intravenous

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infusion can cause serious side effects such as respiratory failure or low blood pressure, the rectal form is absorbed just slowly enough that the medication is usually medically quite safe. Rectal diazepam can be sedating, though, which may make it harder to evaluate the child in order to see if there is anything else going on. Some families are comforted by the knowledge that they have the ability to help end a seizure. For others, the constant need to carry around the syringe creates an unwelcome impression of constant urgency. Children who present in status epilepticus are at much higher risk for any possible future recurrences to also be prolonged. In fact, some authors have concluded that the risk of having a future episode of status epilepticus “is limited largely to children whose first seizure was prolonged” (Hirtz 2003, p.169). However, if a child’s first seizure is status epilepticus, his or her risk for the recurrence of any brief seizure is not higher than for children whose first seizure was brief.

What events can mimic seizures? Part of classifying an event is to determine if it is even a seizure at all. There are a host of intermittent non-seizure events that can mimic or be confused with the different types of seizures. Your doctor might consider:

•

Cardiovascular events such as fainting or arrhythmias. Cardiac involvement may be suggested especially if there is any history of light-headedness, dizziness, or palpitations in the child; or a family history consistent with arrhythmia or sudden death. A fainting spell can be followed by a few twitches, or can sometimes even cause an actual subsequent seizure. Urinary incontinence is suggestive of seizures, but can occur with syncopal spells as well.

•

Breath holding spells can cause sudden loss of consciousness in young children, associated with stiffening, pallor, or blue color change. Unlike seizures, these spells are characteristically precipitated by crying, fright, or pain. A breath holding spell can also be followed by a few twitches, and can also rarely induce a subsequent seizure.

•

Migraines. Both migraines and seizures can begin with an aura and be accompanied by headache and vomiting. Some migraines can even be associated with confusion. (See Chapter 13, on childhood occipital epilepsy.)

•

Gastro-esophageal reflux can cause a painful heartburn that may make an infant stiffen or pause like a seizure. This is called “Sandifer’s syndrome,” and should be considered when the spells consistently follow an infant’s feedings.

OVERVIEW OF SEIZURES

25

•

Behavioral rage attacks can usually be distinguished from seizures. Seizures are not precipitated by frustration or anger, and any violent activity in a seizure is a confused random flailing or fighting off. In contrast, the diagnosis of a behavioral disorder is strongly suggested by provoked, coherent, goal-directed, and offensive attacks.

•

Tic disorders can usually be differentiated by the very consistent appearance of each type of tic, and by the appearance of multiple kinds of typical tics. There is no alteration of consciousness during tics.

• •

Movement disorders can also be confused with seizures. Sleep disorders such as night terrors can raise the question of seizures. Seizures can also be confused with the sudden sleepiness or drop attacks of narcolepsy.

•

Hypoglycemia and other metabolic disorders can cause or be confused with seizures.

• • • •

Substance abuse can cause intermittent unusual behaviors.

•

Pseudo-seizures (also called “non-electrical seizures”) are events created by the patient to look like true seizures, but are not. It is often difficult to determine whether or not the patient is actually consciously aware that he/she is “faking” the events. There is often some identifiable depression, anxiety, stress, or even abuse in the family. Typically, children with pseudo-seizures do not hurt themselves during a spell, do not turn blue, and do not loose control of their urine. Also, compared to true seizures, pseudo-seizures are more likely marked by irregular thrashing and side-to-side rolling movements, screaming or crying during the spell, remaining alert despite abnormal movements in all four extremities, being intermittently responsive during the spell, and lacking in any post-event sleepiness or confusion. To make it more confusing, though, twenty percent of patients with pseudo-seizures also have true seizures (Devinsky 2002, p.91). Often, a video EEG—to actually capture the spells on the EEG—is required to determine which of these events are true electrical seizures.

Chills due to fever do not interfere with normal levels of consciousness. Normal infant startles are discussed in the chapter on infantile spasms. Attention Deficit Hyperactivity Disorder (ADHD) is discussed in the chapter on absence epilepsy.

Chapter 2

W H AT T O O B S E RV E DURING A SEIZURE

There is rarely a pediatric neurologist around when the child is having a seizure. We are not usually asked to wait in other people’s living rooms just in case their child has a spell. So, how does your doctor actually know what happened? Someone else—that’s you—acts as the doctor’s eyes and ears. The more that you can observe, then the more accurately the doctor can decide whether or not the event was a seizure; and, if so, what kind. It is true that evaluations such as a physical exam, electroencephalogram, or MRI may help. However, the history of the event(s) is key. Ultimately, the diagnosis of a seizure is a clinical decision based largely on the event’s description. So, Table 2.1 shows the helpful observations that you can make.

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Table 2.1 Observations during a seizure Feature of the spell

Importance of that feature

Was there an aura? An aura is the prodrome or “warning” to the seizure.

A well-defined aura identifies the seizure as being of focal onset. (Note that even though a seizure has no identifiable aura, it may still be of focal onset.)

How did the seizure start? Did the seizure start in just one part of the body and then spread, or did it involve the whole body equally from the onset?

A localized onset to the seizure makes the seizure likely to have started in one specific part of the brain. (Note that the focal onset may spread so quickly to the rest of the brain—and thus, body—that even though a seizure has no observed focal start, it may still be of focal onset.)

Are there any typical triggers?

This is helpful in many types of seizures and other disorders. For example, a pattern of prominent crying or fright before the spell in a young child makes “breath holding spells” a possible diagnosis. Association with feeding of an infant may be a clue to gastro-esophageal reflux. Association with flashing lights or sleep state may indicate certain kinds of seizures.

Were there palpitations (feelings of a rapid heart beat) or a feeling of light-headedness?

These symptoms might suggest a cardiac problem.

Were there:

These markers help classify the seizure. They are often symptoms of a partial complex seizure, but can also sometimes be seen less prominently in an absence seizure.

• smacking or licking of the lips

• eyelid fluttering • picking or fumbling hand movements? Was the person able to respond to any outside stimulus?

In a moment of simple inattention such as boredom, the person will respond to a stimulus such as being called loudly or having his or her shoulder shaken gently. However, in an absence seizure, there will be no response at all to being called; and in a partial complex seizure, there may be a confused response.

Were there stiffening (“tonic”) and/or later harsh jerking (“clonic”) movements?

Rhythmic clonic movements are strong markers for a seizure event, although a few jerks can follow other conditions such as syncope.

Was the jaw clenched or was the tongue bitten?

A clenched jaw is a strong marker for a seizure event, as is tongue biting—although the latter can also occur with any kind of fall.

Continued on next page

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Table 2.1 cont. Feature of the spell

Importance of that feature

Was there incontinence of urine or bowels?

Incontinence is typically considered a marker for a seizure, but can occur with fainting spells as well.

Was there any color change or breathing problem?

These symptoms indicate cardiovascular/respiratory aspects of the spell—either as a cause or an effect of the event.

How long did the actual seizure last?

When these events happen, they seem to last for an eternity. Try to gauge the right time range. Did it last 1 second, 10 seconds, 5 minutes, or what? Looking at your watch would be helpful—if you can gather your wits to do so.

Was there a “post-ictal” state?

This refers to the period of confusion and/or muscle weakness that may follow certain seizures. The presence helps classify the type of seizure. Be sure to distinguish between the seizure itself, and the post-ictal stage.

Was there a headache?

The presence of a headache can be very important sometimes in determining an acute cause of the seizure. A headache can sometimes routinely follow a seizure, but may also indicate another problem such as bleeding or infection of the brain. Headaches occurring before the seizure are of even more concern.

Have there been spells suggestive of other types of seizures?

Whenever one type of seizure is found, a search for other seizure types should be undertaken. Have there been spells of startle-like jerking of the arms (myoclonic seizures) occurring especially shortly after awaking in the morning? Have there been staring spells suggestive of absence seizures? These events may have gone unnoticed, but are suggestive of certain epilepsy syndromes.

Are there symptoms of nocturnal seizures?

The seizures of some epilepsies occur typically during sleep. Clues of an unwitnessed night-time seizure might be:

• • • • • •

waking up with a bitten tongue waking up with blood/extra saliva on the pillow falling out of bed shaking movements heard from the room unexpected bed-wetting waking up confused or with a headache.

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29

Try your best Who knew that there was so much to look for? Who knew that anyone would ask you about such things? Who can keep a level head and make such observations while simultaneously trying to care for a child during a scary event? No one notices everything. Now, at least, you know what to look for. Try your best.

Chapter 3

W H AT T O D O DURING A SEIZURE

For better or worse, there is not much that an observer can do to alter the outcome of a seizure. That being said, there are a number of measures to help assure safe passage through the spell. Don’t worry. It’s mostly common sense, and is not that complicated. The following suggestions are adapted from The Epilepsy Foundation of America (2005).

•

Stay calm! Everybody functions better in a calm environment—you, other observers, and even the person who is recovering from the seizure.

•

Provide safety from physical injury:

° ° ° °

•

Try to soften the fall. Cradle the person’s head with your hands, a towel, a folded jacket, etc. Clear the area around the person of sharp objects. Do not try to physically stop the movements.

Protect the airway:

° Loosen tight clothes around the neck. ° Turn the person on his/her side (if possible) to prevent choking on fluids or food in the mouth. ° DO NOT PLACE ANYTHING IN THEIR MOUTH. Do not put your fingers in the person’s mouth, or force the person’s mouth open. People do not “swallow their tongue” during a seizure. Forcing something into a patient’s mouth may result in his or her broken teeth, or uselessly bitten fingers—yours!

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31

•

Do not attempt mouth-to-mouth or cardio-pulmonary resuscitation (CPR) except in the unlikely chance that breathing does not resume when the seizure stops. Failure to spontaneously resume effective breathing after a seizure indicates a complication such as choking, head or neck injury, or a heart attack. CPR will not effectively get air into the person’s lungs while he or she is still in an active tonic-clonic seizure.

•

Stay with the person until the seizure stops. Be sure the person can get to a safe destination.

•

For patients having a non-convulsive seizure:

° If needed, guide the patient to safety. Be aware that some types of seizure may cause the patient to be physically unpredictable. ° Explain to bystanders what is happening. People unfamiliar with these seizures might think the person is on drugs.

•

Call for medical assistance if:

° ° ° °

the seizure lasts more than five minutes the seizure recurs there is slow recovery or breathing problems afterwards there is no way to know if there is a previous history of seizures (i.e. if there is no Medical Alert ID tag or anyone who knows about the child’s seizures) ° the patient is pregnant, has another medical problem, or is injured. As always, each situation has to be individualized, and common sense should be used.

Chapter 4

AC C I D E N T P R E C AUT I O N S

Balancing risks and restrictions Once a child has had a seizure, most people worry about what could happen to the child if he or she had a seizure while in a dangerous location. Perhaps you feel that the safest thing would be to put the child in a padded bubble. But would that really be the safest? No. Being overly restrictive would ruin the sense of well-being and normalcy that is the goal of treating the child and family. Indeed, being overly restrictive merely guarantees a bad emotional outcome. Restrictions placed on a child with seizures need to be individualized, taking into account the status of the child’s seizure control. For each child/family, there is a different set of benefits to allowing an activity vs. the psychological repercussions of restricting it. The doctor is your advisor and has suggestions. However, as long as no one else is put at risk, the parents (sometimes along with the child) need ultimately to decide what to do, based on what is in the child’s ultimate physical and emotional best interest. Life has its risks, whether or not you have seizures. Common sense can be a good guide.

Reasonable accident precautions There are certain situations which might prove particularly dangerous places should a seizure occur. Typical warnings to avoid such risks might include the following, especially if it has not yet been shown that the seizures have been under good long-term control:

•

No biking around cars. It’s one thing to fall off a bike while wearing a helmet. That is a risk we all take. It’s another thing to fall off a bike into the path of a moving automobile. (Children need to demonstrate good seizure control before they should bike at all.)

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•

Consult your doctor about local driving laws (see Chapter 10 on teens with epilepsy).

•

No climbing higher than the child’s height. Typically the play set at school is allowed once seizures are under control. However, does the child really need to climb the rope to the top of the gym ceiling?

•

Provide careful and close supervision in and near water, including the bathtub. (Once the child reaches an appropriate age, showers are usually left unsupervised.) At the pool, someone needs to be watching the child so that they can alert the lifeguard. Lakes and especially oceans are areas of significant danger—a child could quickly disappear and be hard to find, especially if not wearing a life jacket. Remember, shallow water is no protection.

•

Lower hot water temperature in the house to below scalding temperature—in case the child falls into the faucet during a seizure.

•

Video games and flashing lights might be restricted by your doctor (see Chapter 9, For Kids to Read with Their Parents).

•

Routine sports are typically allowed. Sports at high risk for head trauma might be particularly risky for those children whose seizures involve a period of confusion. Such a period of confusion could make them vulnerable to injury—such as from an oncoming 250-pound peer. Once the seizures are well controlled, the risks/benefits of high contact sports for an individual child will need to be discussed. There is no evidence that heading a soccer ball will precipitate a seizure (Freeman, Vining and Pillas 2002, p.348).

•

Use common sense!

Chapter 5

M E D I C A L E VA LUAT I O N OF SEIZURES

The medical work-up of a child with suspected seizures involves a history, physical, and neurological examination by the doctor; and possibly a picture of the brain, an electroencephalogram (“brainwave test”), and blood tests. Other tests may be indicated to further evaluate the cause of seizures, or to investigate possible diagnoses other than seizures. Chapter 9, For Kids to Read with Their Parents, has a kid-friendly description of the EEG and MRI.

History, physical, and neurological examinations The evaluation of a child with a seizure begins with a careful history, physical, and neurological examination. For a first scary-looking spell, this will usually occur in the emergency room. For more subtle spells, which have typically been recurrent already, the initial evaluation may take place in the doctor’s office or clinic. First, a general physical examination can be done, including checking blood pressure and listening for abnormal cardiac sounds or heart rates. Examination of the skin may reveal birthmarks such as multiple coffee-colored skin spots, areas devoid of skin pigment, or port wine colored birthmarks around the eye. These skin markings may give a clue to associated neurological conditions. The neurological examination is a painless check of how different brain parts are functioning. Unlike the dermatologist, who can check the skin just by looking at it, neurologists obviously cannot utilize direct touch while examining the brain’s structure. We examine, instead, the brain’s function through a series of maneuvers that collectively comprise the neurological examination. Each test checks the function of a different part of the brain. For example, when the doctor checks to see if a child can detect being touched on the right arm, he or she really is

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35

determining if the child’s left parietal lobe sensory area of the brain is functioning properly. Here are the highlights of the neurological exam. Its not as simple as below, but the outline will de-mystify the process for you and your child. Remember: it’s painless!

•

Cranial nerves. The cranial nerves are a series of twelve nerves that come out from the base of the brain. They control functions carried out by the head, which include:

° Eye function. This includes checking the nerves that control vision as well as eyeball and pupil movements. ° Ophthalmoscopic exam. The doctor looks inside your eyes primarily to look for signs of raised pressure inside the head. How? The pupil is really a clear hole in the eyeball that we use to look out. (The pupil is black because it is dark inside the eyeball.) The doctor shines a light through the pupil with a special scope, which allows the doctor to examine the inside coating of the eyeball called the retina. In particular, we check the area where the optic nerve from the brain pierces into the back of the eyeball—called the “optic disc.” If there is raised pressure inside the skull (from a brain tumor, for example), there may be blurring or swelling of the nerve or vessels in the area. The ophthalmoscopic exam can sometimes pick up clues to other conditions, such as bleeding in the brain or hypertension. ° Sensation and movement of the face, swallowing, and neck muscles. ° Hearing (and occasionally also smell or taste).

•

Motor (muscle strength). We check the parts of the brain that control muscle movements (primarily the frontal lobes) by testing the strength and tone of muscle groups throughout the body.

•

Sensory. We check the parts of the brain that control sensation (the parietal lobes) by testing sensation throughout the body.

•

Reflexes. What is the doctor actually checking with that reflex hammer? When the doctor taps on your knees, there is a local two-way arc between the muscles that are stretched by the tap and the spinal cord. However, the brain has a role in the reflexes as well. Abnormal brain functions can cause the reflexes to be either increased or decreased in the opposite side of the body. The brain even controls what happens when the bottom of a person’s foot is scratched.

•

Cerebellar. The cerebellum sits behind the brainstem and controls balance and coordination. This is the area being tested when you touch

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your nose or perform complicated movements. The cerebellum is rarely directly involved in seizures.

•

Gait. Testing how people walk—regular, on their toes or heels, or as if on a tightrope—evaluates multiple brain functions.

•

Developmental. We check the child’s cognitive and language development.

Pictures of the brain: CT, MRI, and PET scans If a child with a new onset seizure is taken to the emergency room, the brain image that will probably be done is a CT scan (also called a CAT scan for “Computerized Axial Tomography.”) In general, this may initially be done without any intravenous contrast injection. When evaluating for seizures, this emergency non-contrast CT scan may typically be followed up by a contrast enhanced CT scan—or, quite preferably, with an MRI scan (Magnetic Resonance Imaging). The advantages and disadvantages of CT vs. MRI scans are summarized in Table 5.1 below:

Table 5.1 Comparison of CT and MRI scans CT scan

MRI scan

Emergency study

Elective study

Takes a few minutes

Takes 30 minutes or more

Can be done without sedation on young children

Usually requires sedation for children less than five years of age

Can detect most emergency masses, but can miss small lesions

Typically more accurate at detecting lesions

Better at detecting fresh blood

Less sensitive at detecting fresh blood

Uses X-rays

Uses magnet and radio waves

Requires intravenous contrast for best study. The contrast dye used for CT scans has some occasional risks

When used, MRI contrast dye is usually very safe

Poor ability to image the temporal lobes (which are a frequent source of seizures) and the bottom parts of the brain

Visualizes the entire brain well

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37

In short, MRI scans are more sensitive, do not require X-rays, and avoid the use of CT contrast material. Typically, these features make the MRI the neuro-image of choice for most non-emergency indications. PET scans (“Positron Emission Tomography”) and SPECT scans (“SinglePhoton Emission Computed Tomography”) are highly specialized tests, which are usually reserved to confirm or look for active or inactive metabolic areas in the brain that might be amenable to procedures such as surgery. A PET scan uses an intravenous injection of a mildly radioactive substance that has been attached to a chemical normally used in the brain, such as glucose. Sensitive radiation detectors are then used to determine which parts of the brain take up the radiation-tagged glucose (or other chemical). A SPECT scan measures the uptake of a different kind of low-radiation tracer injected by vein into the bloodstream. The scan looks for areas of increased blood flow that suggest the seizure’s location of origin. To be useful, a SPECT scan should be done during or immediately after a seizure—a technical feat in itself.

The electroencephalogram (EEG) An electroencephalogram (EEG) records electrical activity from the brain, just like an electrocardiogram (EKG) records electrical activity from the heart. After measuring the child’s head for placement of the leads, multiple thin wires are attached to the child’s scalp using paste. The wires are used solely to record—not stimulate—the brain’s activity. Leads are also attached to correlate heart activity with the brain activity. During the EEG, a series of procedures are performed to help bring out certain features. This typically includes asking the child to open or close his or her eyes, asking the child to hyperventilate (over-breathe), flashing a strobe light; and often, trying to capture times when the child is awake, drowsy, and/or asleep. The information of an EEG complements the information obtained from a MRI or CT. Whereas the MRI or CT is a picture that shows physical structure, the EEG is an electrical test that shows electrical function. By way of analogy, taking a CT scan is like taking a photograph of a telephone; whereas taking an EEG is like picking up the receiver and listening to what happens. Children’s EEGs are particularly difficult to interpret because of all of the normal variation that occurs during a child’s development. Children should be reassured that the EEG is totally painless and harmless. Most kids find the worst part to be washing the paste out of their hair afterwards. You can help children who might be scared to prepare for the test by play-acting at home. Take some shaving cream and yarn, and pretend to glue “wires” to each other’s head.

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Figure 5.1 Schematic of EEG findings

What can we find on the EEG? An EEG can yield several types of findings. See Figure 5.1.

•

Seizure spikes. Most routine half-hour EEGs do not happen to capture an actual seizure, since actual seizures usually occur quite infrequently. Even so, the routine EEG done in between seizures still has a good chance of detecting abnormalities called “spikes” or “spike and waves” in patients who have epilepsy. A spike is a particularly sharp jerk occurring during the EEG tracing. Sometimes, the spike is followed by a large, smooth “wave.” Finding such spikes is suggestive of a seizure disorder, although by no means is it proof. Sometimes, people have abnormal EEGs and will never have a seizure; and, as above, many people with epilepsy will have a normal EEG in between seizures. The ultimate decision as to whether a person has a seizure disorder is based on the overall clinical picture, not solely upon the EEG.

•

Actual seizures. On occasion, a routine EEG might actually happen to capture a seizure. Whereas a “spike” is just an isolated moment of abnormal brain activity, a “seizure” is a longer, organized event where spikes start out small, build up into a rhythm, and eventually gradually diminish in size. Capturing an actual seizure on the EEG essentially proves a seizure disorder.

MEDICAL EVALUATION OF SEIZURES

•

39

Localized or generalized brain dysfunction. In addition to showing the features of seizure disorders, the EEG can also show areas of the brain where the waves are slower, of lower voltage, or less organized than expected. Such findings would be indicative of “brain dysfunction.” Sometimes, these findings are seen throughout the brain. Other times, they are localized and indicate that one part of the brain is functioning abnormally—perhaps due to a structural abnormality.

As we shall see in Part 2, certain patterns on the EEG are associated with particular epilepsy syndromes. The location of spikes can help determine if the seizure is generalized (the spikes come from everywhere all at once) or focal (the spikes come from one part of the brain).

Types of EEG The EEG should ideally be done in a manner that recreates the conditions when the seizures have occurred. In particular, seizures that occur during sleep should best be evaluated with an EEG that includes sleep. In fact, EEGs are generally most likely to detect abnormalities if they include awake, drowsy, and sleeping states: spikes can be activated by sleep deprivation, by the act of falling asleep during the EEG, and by the state of being asleep. In particular, children with benign childhood epilepsy with centrotemporal spikes (also known as benign rolandic epilepsy) may have totally normal EEGs during the waking state that become markedly abnormal during sleep. Even young children who will need to be sedated will benefit from sleep deprivation the night before, as the sedative works much better on children who are already sleepy. There are several ways to perform an EEG:

•

Routine awake EEG. In a cooperative patient, an EEG is done in the following conditions:

° ° ° °

•

during quiet wakefulness, eyes closed during quiet wakefulness, eyes open during a flashing strobe light, which triggers some types of seizure spikes during hyperventilation (heavy breathing), which triggers absence seizures, and helps bring out abnormally functioning areas.

Sleep deprived EEG. In a sleep deprived EEG, the adult stays up all night before the EEG. In children, we typically allow several hours of sleep; or, simply bring in the child well past naptime. Children should not nap while traveling to the EEG lab! In addition to the above conditions, the patient is given enough time to fall asleep during the EEG. After

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recording a period of sleep, the patient is aroused while the EEG continues.

•

Sedated EEG. In order to get some children to tolerate multiple wires being glued onto their head, some children will require an oral sedation. Note that, unlike during an MRI, children do not need to be perfectly still during the EEG (although that would be nice). Discuss the issue of sedation for this procedure with your physician.

•

Video EEG. Sometimes, the EEG needs to be done continuously in the hospital’s EEG suite for an extended period of time—including multiple overnights—to capture daytime spells, nocturnal spells, or subclinical seizures that might interfere with language. A video camera also records the events. The video EEG is performed when it is essential to actually capture a spell on the EEG in order to determine whether or not it is a seizure. It is also usually part of the evaluation of any possible epilepsy surgery. Parents should press the “event” button whenever there is clinical behavior that they find suspicious.

•

Continuous ambulatory EEG. If the video picture component is not required for the prolonged study, sometimes it can be done at home with ambulatory equipment. The information from the leads is stored on a small recorder that is typically worn on the child’s waist, and is reviewed when the recorder is returned to the EEG lab. Like the video EEG, an accurate event log is essential. The ambulatory equipment may not be quite as sensitive for some types of seizures as the in-patient monitoring, and does not include a video portion for the doctors to see. Also, some children need the support of the in-patient EEG technicians in order to keep the leads on.

If one EEG does not give the required information, repeating the study significantly increases the yield and accuracy of detecting a seizure disorder. EEGs are typically performed at the time of initial diagnosis; when there are any changes or “surprises” during treatment; and when it is time to consider tapering the medication. They may also be done to make sure that we are not missing certain undetected seizures. Otherwise, routine follow-up EEGs are usually not required during treatment.

Blood tests Blood tests frequently performed in the evaluation of seizures include blood chemistries (electrolytes, renal function tests, glucose, calcium, and liver function tests) and a complete blood count. Usually, these can be accomplished from a single blood drawing.

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41

Other tests Depending on the individual child’s presentation, other tests may occasionally need to be done such as:

•

a lumbar puncture (“spinal tap”) looking for infection in or around the brain

• •

detailed metabolic tests such as amino and organic acids chromosome and other blood tests.

What if the tests are all normal? Many parents are somewhat frustrated when we tell them that all of the tests come back normal. They want to know why their child had this event! Fortunately, though, we usually don’t find an obvious cause for childhood onset seizures (unlike adult onset seizures). That is good news. Having a normal MRI certainly beats the alternative.

Chapter 6

T O T R E AT OR N O T, A N D W HE N T O S T OP?

The decision to treat with anticonvulsants: What do you need to know? Each year in the US, between 25,000 and 40,000 children will have their first “unprovoked” seizure—that is, a seizure not secondary to a cause such as fever, infection, or trauma (Hirtz et al. 2003). In order to help doctors and families reach appropriate decisions for these children, the American Academy of Neurology in conjunction with the Child Neurology Society reviewed the literature to produce a practice parameter on the treatment of a first, unprovoked seizure (Hirtz et al. 2003). Presented below are some of the questions and best available answers found in that review of tonic-clonic or partial seizures. The answers to these questions can help parents and their doctors reach an informed decision regarding how best to care for the child.

What potential problems could result from a second seizure? In order to decide whether or not to treat a child with anticonvulsants, it is reasonable to ask, “What could happen if my child had another seizure?” Studies with children show that even prolonged seizures are unlikely to induce their own damage (above and beyond whatever precipitated the seizure in the first place). Some animal studies identified in the practice parameter show that prolonged or recurrent seizures may in some situations cause brain cell damage and increase the risk of epilepsy. The practice parameter concludes “the relevance of data from these animal models to seizures in humans is unclear.” The parameter also states “serious injury from a seizure in a child is a rare event, usually from a fall with loss of consciousness.” They recommend simple accident precautions such as those discussed in Chapter 4. 42

TO TREAT OR NOT, AND WHEN TO STOP?

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Finally, the authors also point out that sudden unexpected death in children with epilepsy is “very uncommon” (Hirtz et al. 2003, p.168). A study by Camfield et al. (2002) showed that children with childhood onset seizures have the same risk of death as children without any significant neurological handicap.

After a first unprovoked seizure, how likely is a single recurrence? Multiple studies reach multiple different conclusions about the risk of a seizure recurrence by one year after the first seizure, with estimates ranging from 14 percent to 65 percent. Freeman et al. (2002, p.173) place the risk of a first major seizure being followed by another to be around 30 percent. A normal EEG and MRI would be expected to put a child at the lower end of this range. The majority of these recurrences occurred within the first year or two of the first seizure. Note that subtle seizures such as absence spells are rarely detected after the first one. Thus, subtle seizures are typically already recurrent when first diagnosed, and typically require treatment.

After a first unprovoked seizure, how likely are multiple recurrences? In children followed on average for more than ten years:

• • • •

46 percent had at least one or more recurrences 19 percent had at least four or more recurrences 10 percent had at least ten or more recurrences “few” of the children were considered “intractable.” (Berg et al. 2001).

What are the risk factors of having a recurrence? Not surprisingly, children with an identifiable underlying brain disorder (such as an old injury) are at a higher risk of recurrence, as are children with an abnormal EEG. For those children with a first idiopathic seizure, the recurrence risk is 30–50 percent at two-year follow up. For children with a first seizure due to a pre-existing brain problem, the risk is typically greater than 50 percent.

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What are the chances of having a prolonged seizure? If a child’s first seizure lasts more than 30 minutes (“status epilepticus”), his or her risk for the recurrence of any length seizure is not higher than for children with a brief first seizure. However, children who present in status epilepticus are at much higher risk for any possible future recurrences to also be prolonged. In fact, the authors of the practice parameter concluded that the risk of having a future episode of status epilepticus “is limited largely to children whose first seizure was prolonged” (Hirtz et al. 2003, p.169).

Does anticonvulsant treatment after a first seizure actually work? The practice parameter concludes that the use of anticonvulsants after a first seizure does reduce the risk of recurrence—but, the size of that effect varies between studies, and the data from pediatric studies is not strong. Available data also provides good evidence that waiting to start treatment until after the second seizure does not reduce the chance of obtaining a one to two-year reprise from the seizures.

To treat or not to treat: Individualizing the decision So, how do we put this all together? The decision to treat children with one or more seizures with anticonvulsants is made jointly by family members and their doctors. Factors in the decision include the:

• • • • • •

type of seizures (including their duration and focality) number and frequency of seizures type of epilepsy syndrome (see Part 2 of this book) age of the child family “comfort” levels EEG and MRI findings.

In general, most neurologists do not suggest anticonvulsants to children after a first, generalized, brief seizure in the setting of a normal EEG and MRI. However, the care for each child must be individualized, taking into account the risks of seizures vs. the risks of medication (including medical, behavioral, and psychosocial aspects of drug treatment). See Part 2 for treatment decisions for individual epilepsy syndromes.

TO TREAT OR NOT, AND WHEN TO STOP?

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Stopping the anticonvulsants: The facts Once the medications have been started, when can you stop them? Typically, children are treated with anticonvulsants until they have gone two consecutive years without a seizure. As always, individualization is key. Each time that the child has a breakthrough seizure while on medication, the “clock” is reset to two more years of treatment. At the time that tapering is considered, the EEG is repeated, and consideration with the family is given to tapering off the anticonvulsant over a several month period. (Sudden discontinuation of certain anticonvulsants might trigger an exacerbation of severe seizures.) Risk factors for a relapse after discontinuing anticonvulsants include: difficulty obtaining seizure control initially, the presence of partial seizures, the first seizure starting after 12 years of age, neurological or developmental abnormalities in the child, and an abnormal EEG at time of discontinuation. Freeman et al. (2002, p.176) cite the following success rates when medication is tapered after having completed a two-year seizure free period:

•

Neurologically typical children with idiopathic seizures and essentially normal EEG have a 90–95 percent chance of staying seizure free.

•

Children with seizures related to an old injury (even if the EEG is moderately abnormal) have a 40–60 percent chance of staying seizure free.

•

Children with severely abnormal EEGs at the end of treatment have a poor chance of remaining seizure free. Examples of children in this category include those with Juvenile Myoclonic Epilepsy or Lennox–Gastaut syndrome.

If there are relapses, half will occur within the first year of discontinuation of medication, and almost all relapses occur within the first two years of discontinuation (Freeman et al. 2002, p.179). If the child relapses after a first attempt at tapering the anticonvulsants, we might try it again in a few more years.

Stopping the anticonvulsants: The emotional aspects Understandably, parents and children often have mixed feelings about tapering the anticonvulsants. On the one hand, it is an exciting prospect to get this problem behind us. On the other hand, we’ve become secure about the seizures now that the child is doing so well, and are afraid of what might happen once the medication is slowly withdrawn. To address these concerns, consider the following advice. Is the child seizure free because he or she has outgrown them, or is he or she seizure free only because of the medication? Ultimately there is only one way to know whether your child needs the medication or not—and that is to see how he or

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she does off it. Although there are exceptions, at some point we really do have to see how most children will do off medications. After all, when your child is 105 years old, do you want his wife to ask, “Honey, why do you take that medication? You haven’t had a seizure for the last 95 years!” Some parents ask, “But couldn’t he stay on the medication just another year?” Well, it’s your child, but it probably doesn’t make sense. It just prolongs the worries until next year. You will still ultimately have to go through the same period of anxiety. There is no getting around it. Additionally, the lifestyle of a child only becomes more risky and less supervised as the child gets older—such as driving and (for girls) pregnancy. It is better to see if a child still needs medication well in advance of such events. In particular, it is less disruptive if a teen can be off medication for at least a year before reaching driving age. (Pregnancy and driving issues are discussed in Chapter 10.) Also, remember that the chances of being hurt by a relapse is very low—your child has already had one or more seizures, and she’s doing fine now, isn’t she? What better evidence could you ask for? For a period of time during and after the taper, we resume strict adherence to the same types of accident precautions as when we first started having seizures. (See Chapter 4 on Accident Precautions.) Discuss this with your doctor. Although the decision to come off of medication can be anxiety producing, we have to remember that this is the good point that we’ve been waiting for. A decision is called for, but let’s not confuse the need to make a decision with the existence of a bad problem. We are deciding between two good options: a normal life on medication (we already know that’s the case if you are at this point), or, it is hoped, a normal life off medication. Of course, it’s easy for us neurologists to give calm advice about someone else’s child.

Chapter 7

T R E AT M E N T W I T H M E D I C AT I O N S 1

Choice of anticonvulsants In the previous chapter, we discussed when to treat. In this chapter, we discuss what anticonvulsant medications can be used to treat. To facilitate use of this information in multiple countries, we will refer to the medications by their generic name followed by their US trademark name in parenthesis. For example: carbamazepine (Tegretol). Non-medical treatments, which are usually reserved for patients not satisfactorily controlled with medications, are discussed in the next chapter. An appropriate anticonvulsant is chosen on the basis of several factors, including safety, side effects, and efficacy for the type of epilepsy being treated. As demonstrated in Table 7.1, certain anticonvulsants work better for particular types of seizures.

Anticonvulsants for partial seizures Let’s take an overview look at Table 7.1. On the top part are the partial seizures (simple partial and complex partial). Note that from a therapeutic point of view, both simple and complex partial seizures are treated with the same medications. 1

Like other information in this book, this information is evolving and expected to change rapidly. It does not constitute medical advice, nor does it replace the medical advice given by your doctor. This medication summary is by no means meant to be all-inclusive, nor can it necessarily keep up to date! Some of the common uses described in this chapter are not yet US Food and Drug Administration (FDA) approved, including the types of seizures being treated, and the age of the child for whom the medications may be prescribed. Prescribing practices may vary by country. Current and more detailed information about individual anticonvulsant medications can be found in the manufacturer’s package insert and at many Internet sites.

47

Table 7.1 Summary of anticonvulsants. Not all uses are FDA approved, and data are subject to change

Partial

Seizure Type

Older Anticonvulsants (often used first because of extensive experience) carbamazepine (Tegretol) oxcarbazepine* (Trileptal) phenytoin (Dilantin) phenobarbital

valproic acid (Depakote) (Depakane)

Complex partial

üüüü

Tonic/clonic

üüüü

ethosuximide (Zarontin)

New Anticonvulsants

benzodiazepines (Klonopin) (Ativan) (Tranxene)

gabapentin (Neurontin)

üüü

üü

üüü

üüü

üüü

üüü

üüüü

üüüü

üü

üüü

üüü

üüü

Avoid in primarily generalized, see below

üüü

May cause absence status

üüü

May worsen

üüü

May worsen

lamotrigine (Lamictal)

topiramate (Topamax)

levetiracetam (Keppra)

zonisamide (Zonegran)

vigabatrin (Sabril) (not approved in USA)

Simple partial

Not approved

Generalized

tiagabine (Gabitril)

Absence

phenytoin carbamazepine (and ?oxcarb) may worsen

üüüü

Minor motor (myoclonic and atonic)

carbamazepine (and ?oxcarb) may worsen

üüüü

üüüü

üüüü

üüü

üü

üüüü

May worsen

üü

*Oxcarbazepine is a fairly new anticonvulsant, but listed here next to carbamazepine since they are very similar. Key: Mildly effective ü up to Very effective üüüü

üüü

üüü üüü Avoid, see below

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You can see that oxcarbazepine (Trileptal), carbamazepine (Tegretol), phenytoin (Dilantin), and phenobarbital are targeted mainly at the partial seizures, but additionally work well for generalized tonic-clonic seizures (see precautions below). Note that oxcarbazepine (Trileptal) is actually one of the “new” anticonvulsants, but is listed in the chart next to carbamazepine (Tegretol) because of the similarity of the two medications. When possible, oxcarbazepine or carbamazepine are usually chosen first in order to avoid the hyperactivity frequently seen with phenobarbital, and the gum hypertrophy and facial hair growth seen with phenytoin. However, phenobarbital is frequently used first in children less than a few years of age. Also, since oxcarbazepine and carbamazepine are not available in intravenous forms, sometimes phenytoin is used when emergency control of seizures is required. Later, the phenytoin may (or may not) be more leisurely switched over to one of the other anticonvulsants. Valproic acid (Depakene) and divalproex sodium (Depakote) are also effective for partial seizures. Note that throughout this text, we will use the terms “valproic acid,” “valproate” and “divalproex sodium” interchangeably, even though they are actually slightly different forms of the same chemical. The newer anticonvulsants such as levetiracetam (Keppra), gabapentin (Neurontin), topiramate (Topamax), tiagabine (Gabitril), and lamotrigine (Lamictal) are generally currently indicated as add-on therapy for partial seizures in patients who are already taking another anticonvulsant. Although there are differences in side effects, there seems to be no clear difference in efficacy between the old anticonvulsants and the new ones—nor are any of the new anticonvulsant clearly better than the other new ones (Panayiotopoulos 2002, p.206). Details about these medications—including the particular age of approval, as well as the suspected (but not yet FDA approved) type of seizures that they control—are described below, and in published reviews (Brown and Holmes 2004; French et al. 2004; Pellock, Morton and Watemberg 1998). Some of the common practice uses of the anticonvulsants that are discussed here are not yet US Food and Drug Administration (FDA) approved.

Anticonvulsants for generalized seizures Referring again to Table 7.1, we see that the generalized seizures are listed on the bottom part of the chart. Valproic acid (Depakene, Depakote) works particularly well on all generalized seizures, including primarily generalized tonic-clonic, absence, atonic, and myoclonic seizures. Valproate appears effective with partial seizures as well. Use of valproate may be tempered by the presence of risk factors for valproate-induced hepatic failure, which include young patient age, neurological impairment, and concomitant use of other anticonvulsants.

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Lamotrigine (Lamictal) clearly helps tonic-clonic seizures, but may sometimes exacerbate myoclonic seizures. Ethosuximide (Zarontin) is generally limited to the treatment of absence seizures. Levetiracetam (Keppra) is a promising medication for a broad spectrum of idiopathic generalized seizures. Topiramate (Topamax) also has a broad range of activity. The benzodiazepine medications such as clonazepam (Klonopin) and lorazepam (Ativan) are also useful particularly for syndromes that include absence or myoclonic seizures. Oxcarbazepine (Trileptal), carbamazepine (Tegretol), phenytoin (Dilantin), and phenobarbital can also be used to control primary generalized tonic-clonic seizures, with the following caveats:

•

carbamazepine (Tegretol), and thus presumably oxcarbazepine (Trileptal), can worsen other types of generalized seizures, but can sometimes be added on to help treat the convulsive seizures if other medications are controlling the absence and myoclonic seizures

•

phenytoin (Dilantin) may have a negative effect on absence seizures.

Note that certain drugs are relatively contraindicated in the treatment of primary generalized tonic-clonic seizures, as they may precipitate even prolonged absence seizures in predisposed patients. Such medications include tiagabine (Gabitril) and vigabatrin (Sabril).

Will the anticonvulsants change my child’s behavior or learning? Most anticonvulsants cause little if any intellectual impairment, especially when taken at typical doses. In fact, group studies have shown slight or no improvement in intellect after medications have been discontinued. Hidden within these group statistics, though, there certainly may be individual children who do have significant changes in cognitive function from the anticonvulsants (Devinsky 2002, p.225). Therefore, any change in the child’s behavior or intellect should be discussed with your doctor. Certain drugs, such as phenobarbital and the benzodiazepines (such as Klonopin or Ativan) are well known to cause sedation, moodiness, irritability, or hyperactivity in some of the children. The cognitive slowing seen with topiramate (Topamax) can be minimized by going “slow and low,” i.e. titrating the medication slowly and using the lowest dose possible. (See the individual description of each medication below.)

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Monitoring anticonvulsants by using clinical symptoms Patients on most anticonvulsants should be watched for the following clinical symptoms. Individual anticonvulsants, of course, can have their own particular set of side effects. Promptly report any concerns to your doctor.

•

Toxic high blood levels might cause sleepiness, confusion, vomiting, unsteady gait, tremor, and even double vision. In other words, the child might look “drunk.”

•

Liver inflammation may present with vomiting, abdominal pain, jaundice, malaise, fatigue, facial swelling, weakness, or loss of appetite.

•

Suppression of blood products that are normally made by the bone marrow may present with frequent or unusual infections, unusual fevers, appearing pale or anemic, easy bleeding or bruising, or clusters of tiny, flat red spots in the skin called “petechiae.”

•

Any skin reaction should be reported immediately to the doctor. In particular, hives, swelling of the face, breathing problems; or inflammation of the eyes, mouth, genitalia, or rectum can represent a severe (even life-threatening) reaction called “Stevens–Johnson Syndrome”—and should be immediately brought to medical attention. Fever and swollen lymph nodes may herald the onset of the syndrome. Such potential symptoms of an allergic reaction occur classically within a few weeks after starting a medication, but can occur months later (or even possibly at any time).

Monitoring anticonvulsants by using laboratory tests Different doctors may follow different procedures for monitoring a patient who is taking anticonvulsants. Thus, laboratory testing on any of these anticonvulsants needs to be individualized. In general, though, blood tests may be done as a baseline before starting the medication, then a few weeks into treatment, then monthly for several months, and then every six months. These tests can be drawn from a single needle stick and then typically sent for:

•

Complete blood count with differential (“CBC with diff”). The CBC tests for any suppression of the blood products. It measures the total number of white blood cells (WBCs) that fight infection, the hemoglobin/hematocrit that checks for anemia, and the platelet count that determines if there are enough platelets to help the blood clot properly. The “differential” measures the percentage of different kinds of white blood cells that comprise the total WBC.

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•

Liver function tests (LFTs) examine the levels of a series of enzymes from the liver such as the SGOT (a.k.a. “AST”) and SGPT (a.k.a. “ALT”). These enzymes normally leak slightly into the bloodstream. If the liver is inflamed, though, there will be an increased amount of these enzymes in the blood.

•

Drug levels in the blood can be obtained for most of the anticonvulsants. “Normal” drug levels are given as a range. For example, normal phenobarbital levels are from 15 to 35 mcg/ml. The lower end of the therapeutic range (15 for phenobarbital) is the drug concentration in the blood where many patients will begin to get a therapeutic response to the medication. As the level goes up, the efficacy of the medication typically goes up as well. However, the side effects tend to increase, also. When the blood level exceeds the suggested upper number of the therapeutic range (35 for phenobarbital), many patients will begin to show “toxicity” or side effects. Note that many patients will have a beneficial effect even when their levels are “below therapeutic,” some patients will have side effects even when their levels are below the upper range of normal, and some patients will not appear toxic even when their levels are above the upper range of normal.

•

Drug levels fluctuate in between doses. In general, it is most useful to obtain “trough levels,” which are those obtained at the low point just before the next dose. Levels obtained shortly after the child swallows the medication are hard to interpret. We don’t know if the level represents a trough (if the medication hasn’t been absorbed yet), or if it represents a “peak” (if the medication was just fully absorbed). Get trough levels, when possible. This is typically done before the morning dose, or sometimes just before the afternoon dose.

•

Other blood tests such as electrolyte levels may be ordered depending on the medication.

•

Urine tests—looking for signs of kidney stones—are followed for some medications such as topiramate (Topamax) or zonisamide (Zonegram). Clinical symptoms of kidney stones might include blood in the urine, flank pain, or pain with urination.

Titrating the anticonvulsant dosage In common practice, a preliminary target daily dosage is chosen based on the child’s weight. A low daily drug dose is used initially, and gradually built up to that target over a period of days or weeks. The dosage may then be adjusted based

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53

on the resultant drug levels, the effectiveness of the seizure control, and the presence of any side effects. The drug levels are statistical guides. The proper anticonvulsant level is the one that controls the seizures without significant side effects.

What about using generic medications? While generic medications may make sense in other settings, they should probably best be avoided in the setting of epilepsy. It is not so much a question about the quality of the medication in the generic formulations. Rather, it is that the formulation from each generic company varies in bioavailability and rate of release—not just from the brand name, but from each other as well. In fact, the FDA only requires that generics be within 20 percent (either up or down) of the same amount of medication released as the equivalent brand name preparation. That means that if your pharmacy switches to a different generic formulation, there could be as much as a 40 percent difference in the amount of medication that the patient receives (if a generic on the low end of availability is switched to a generic on the high end of availability). This can lead to unpredictable changes in drug levels and seizure control. Also, some generics release the medication at different rates into the bloodstream, sometimes leading to side effects such as sleepiness or stomach upset. In an area as unpredictable as seizure control, who needs all of these additional variables? If cost is an issue, try to get a health plan with a prescription benefit. Your doctor, local Epilepsy Foundation, friends, or pharmacist may have advice regarding obtaining medication at lower prices. Also, see the chapter on Further Reading for websites related to obtaining medication at reduced cost.

What if I may have missed a dose? If you are sure that you missed a dose, then make it up over the remainder of the day. If you are not sure if you missed it—or if the child may have vomited it—then you might hedge your bet by making up half of the typical dose. That way, you won’t be particularly far from the correct dose, whether the child actually missed it or not.

Other general comments about anticonvulsant usage A number of other general principles apply to the usage of most of the anticonvulsants:

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•

Check with your doctor or pharmacist for any drug interactions or any other problems with the use of any other medication in a child with seizures. In general, it is safe to use acetaminophen (Tylenol), ibuprofen (Motrin, Advil), amoxicillin, other penicillins, and cephalosporins (such as Ceclor). Erythromycin based antibiotics, though, may react unfavorably with a number of the anticonvulsants, such as carbamazepine (Tegretol)—causing dangerously altered drug levels. Cold remedies which contain medications like pseudophedrine seem to be of less risk than antihistamines (such as diphenhydramine) at inducing seizures (Devinsky 2002, p.67).

•

Take the medication regularly! Missed doses can allow seizures to break through. If you realize that a dose was missed earlier that day, then make it up over the next 24 hours.

•

Anticonvulsants can often alter the metabolism of each other. Although statistical predictions can be made, the only way to really monitor the effect of their interaction is to check the blood levels.

•

Medications are typically dosed at intervals appropriate to the drug’s “half-life.” The half-life of a drug is the number of hours that it takes the body to cut the blood level in half. Drugs with a short half-life of six hours need to be dosed four times a day. Drugs with a long half-life of 12 hours can be dosed just twice a day. It takes five half-lives for a drug to reach its new steady-state blood level. For example, phenytoin (Dilantin) has a half-life of 12 hours. It will take 5 x 12 hours = 60 hours (two to three days) to reach a new level after a dosage change. In contrast, phenobarbital has an exceptionally long half-life of three days! Thus, a dosage change of phenobarbital will take 5 x 3 days = 15 days to reach its new drug level. As a rule of thumb, it takes a week or two before we know if a dosage change is effective and/or tolerated.

•

Any sedating effects of a medication can be minimized by:

° dividing the total daily dosage into small, frequent doses ° taking a larger fraction of the total 24-hour dosage at bedtime, and ° taking the medication after a meal to slow its rate of absorption.

•

Special care must be taken when using anticonvulsants in females of childbearing age. Many of the anticonvulsants have the potential to cause birth defects in an embryo exposed to the medication. Valproic acid (Depakote) is at a particular risk to cause fetal spinal cord defects. The supplement folic acid can lessen (but not eliminate) this problem. It is often given preventatively to girls who can conceive children—even those

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55

who don’t plan to! All plans for childbirth need to be discussed well in advance. (See Chapter 10 on teen issues.)

•

Certain anticonvulsants may interfere with the effectiveness of birth control pills.

•

Can anyone guarantee you that these medications are perfectly safe? Can anyone guarantee you that no new side effects will ever be discovered? No and no. The question, though, is not “Are the medications without risk?” Rather, the important question is, “Are the risks of medication less than the medical and psychosocial risks of untreated seizures?”

What supplements may be needed? Vitamin supplements are sometimes useful to counteract certain possible side effects of the anticonvulsants:

•

Folic acid may help reduce the incidence of fetal defects occurring when mothers take certain anticonvulsants while pregnant. This is most important when taking valproic acid (Depakote), but also applies to other antiseizure medications. Most females on anticonvulsants of childbearing age should be taking two mg of folic acid/day. Certain patients should be taking four mg/day, such as patients taking high doses of valproic acid (more than 1000 mg/day) or with a family history of spinal cord defects. Consult your doctor.

•

Calcium and vitamin D supplementation should be considered in patients at risk for osteoporosis (thinning of the bones). A bone X-ray or endocrinology consult can be obtained for patients who have taken certain anticonvulsants for more than several years, such as carbamazepine, phenobarbital, primidone, phenytoin, or valproic acid. Data are lacking on the effect of the newer anticonvulsants on bone marrow density.

•

Selenium and zinc may help reduce hair loss associated with valproic acid.

•

Carnitine may possibly help reduce some other side effects of valproic acid.

•

Vitamin B6 (pyridoxine) supplements can rarely be required in the treatment of rare, persistent seizure disorders seen mainly in newborns or infants.

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About the individual anticonvulsants Below is a very brief summary of the individual anticonvulsants as they are commonly named and used in the US. Not all of these uses are US FDA approved either in terms of the type of seizure, their use alone, or the age of the patient being treated. This information is not meant to be all-inclusive! (See disclaimer at the beginning of this book and chapter.) Current and more detailed information about individual anticonvulsant medications can be found in the package insert and at many Internet sites. For example, www.drugs.com has patient summary information sheets, drug interaction checkers, and links to the detailed information contained in drug company package inserts. The pharmaceutical companies keep information from their perspective on the newer anticonvulsants at web sites typically named www.[insert US brand name].com. Up until 1990, there were six major available anticonvulsants: carbamazepine, ethosuximide, phenobarbital, phenytoin, primidone, and valproic acid. In the search for better control with fewer side effects and fewer drug interactions, another seven major anticonvulsants have been released in the US. Listed in order of their FDA approval, these are: gabapentin, lamotrigine, topiramate, levetiracetam, oxcarbazepine, tiagabine, and zonisamide. In general, these new medications have fewer drug interactions, are better tolerated, and have equivalent efficacy to the older anticonvulsants. Some may also be safer, and may need less laboratory monitoring (French et al. 2004). However, their possible advantages need to be balanced against their lack of experience and long-term follow-up (and their increased cost). Many of the new anticonvulsants have not yet obtained FDA approval for use as initial therapy for patients with new-onset seizures. It’s a classic dilemma: do you use the tried and true medication, or do you use the fancy new one that seems to be better but has less clinical experience? As a rule of thumb, especially in children, I prefer to start with a medication that has a long safety record—unless compelled by failure or lack of tolerance of the older ones, or some important feature/benefit of a new medication.

The “older” anticonvulsants carbamazepine (Tegretol, Tegretol XR and Carbatrol) Carbamazepine has been one of the leading anticonvulsants used in children. It controls partial seizures in more than 70 percent of patients (Panayiotopoulos 2002, p.206). Carbamazepine treats partial seizures and generalized tonic-clonic seizures, but might actually worsen other types of generalized seizures such as myoclonic spells. Oxcarbazepine (see Trileptal below) likely has the same spectrum of activity.

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Carbamazepine is usually quite well tolerated. When started, it may be somewhat sedating. To allow the child to adjust to the metabolism of carbamazepine, it should be started at a low dose and built to a target dosage over about two weeks. The sedation is more common in the liquid and chewable tablet forms, which have rapid peaks of absorption compared to the regular tablets or long-lasting preparations. Otherwise, there are few effects on thinking or learning. It may actually help improve a child’s mood and behavior. If a problem, peak levels and stomach upset can be minimized by splitting the total daily dose into multiple small doses (three or four) separated throughout the day. Long-lasting preparations such as Tegretol XR and Carbatrol can reduce the dosing schedule to just twice a day. This medication can commonly cause a mild—but usually clinically insignificant—suppression of the white blood cell (WBC) count. Only very rarely will the bone marrow suppression be severe or involve all bone marrow products such as the red blood cells, white blood cells, and platelets (a condition called “aplastic anemia”). Irreversible liver failure is also possible but very rare. Allergic reactions can occur with some frequency. They are usually mild but occasionally very severe. Carbamazepine is rarely associated with a low serum sodium level due to the inappropriate retention of water. Carbamazepine may cause a slightly higher risk of fetal spinal cord defects than other anticonvulsants (except valproic acid which has a very high risk). Carbamazepine seems sometimes to raise cholesterol levels and atherosclerotic risk factors. Toxic high levels produce the typical “drunk-like” effect of sedation, vomiting, headache, and unsteadiness. Blurred or double vision can also occur with high levels of carbamazepine. Grapefruit juice and erythromycin based antibiotics can cause a dangerous increase in drug levels. There are numerous other possible drug interactions, so always check with your doctor and pharmacist.

phenytoin a.k.a. diphenylhydantoin (Dilantin) Phenytoin (Dilantin) has been extensively used for more than half a century. It is effective for partial seizures and generalized tonic-clonic seizures. Since it is not sedating, phenytoin can be used to “load” patients, i.e. patients can be brought to full therapeutic levels quickly. A patient can achieve therapeutic levels while still in the emergency room via the intravenous route, or within a day or so by the oral route. This means that it is often used in the emergency room to rapidly treat severe seizures. (A safer intravenous form of phenytoin called fosphenytoin (Cerebyx) is available, which has less chance of causing cardiac arrhythmias or low blood pressure.) If phenytoin is started because of its quick onset, sometimes it will be continued orally for long-term seizure control.

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However, phenytoin has its problems when used for prolonged periods, and is often switched over to another anticonvulsant: oral absorption is often very erratic in young children, making it difficult to maintain therapeutic levels in this age range. Additionally, after prolonged use, phenytoin can produce coarse facial features, facial hair, and puffy gums. The gum problems can be minimized with excellent oral hygiene and frequent dental visits. Otherwise, phenytoin shares many of the same side effects as carbamazepine, including allergic skin reactions and rare bone marrow or liver problems. Long-term use (more than several years) can also lead to deficient bone mineralization called osteoporosis, which can be treated with calcium and vitamin D, if needed.

phenobarbital Phenobarbital is one of the oldest anticonvulsants. It has a similar spectrum of activity to carbamazepine and phenytoin, i.e. partial seizures and generalized tonic-clonic seizures. It is not implicated in worsening absence or myoclonic seizures. Phenobarbital may not be as effective as phenytoin and carbamazepine for the treatment of partial seizures. Children can be loaded intravenously with phenobarbital, making it useful for emergency room treatment of severe seizures. However, rapid loading with phenobarbital can cause serious sedation, respiratory suppression, and low blood pressure. When used orally, the major problem with phenobarbital is sedation. However, about a third of older children will experience hyperactivity or irritability with phenobarbital. All of these issues are less prominent with infants. These behavioral changes limit the use of this medication, despite the reliable drug levels that come from its reliable absorption and metabolism. Besides these behavioral problems, phenobarbital can cause allergic skin reactions, and very rarely liver problems. Phenobarbital has a very long half-life (about two to three days), making it possible to dose only once or twice a day. Often, the larger part of the total 24-hour dose (or the entire daily dose) is given at bedtime, in order to minimize daytime sedation. Animal studies raise concerns about possible negative effects of phenobarbital on the developing nervous system. In decades of clinical experience, this has not had a discernable long-term effect in children. Phenobarbital (like carbamazepine) can sometimes raise cholesterol and atherosclerotic risk factors. Possible side effects like these are part of why we try to use medications only when necessary.

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primidone (Mysoline) Think of primidone (Mysoline) as “phenobarbital plus.” The anticonvulsant effect of primidone comes from its own anticonvulsant activity, as well the anticonvulsant effect of one of its metabolites—which happens to be phenobarbital. Hence, primidone may be useful when phenobarbital almost achieves adequate control, but the child needs a little more medication. In common practice, given all of the new anticonvulsants, primidone is not commonly started any more. valproic acid (Depakene) and divalproex (Depakote) Valproic acid (Depakene) and divalproex (Depakote) are variations of the same chemical. In this text, we refer to them interchangeably. In typical practice, Depakote is used for its smoother and longer course of action—unless the child requires a liquid preparation, which only exists for Depakene. (Depakene does come in tablet form, as well.) In addition to the regular Depakote tablet form, Depakote comes as sprinkle capsules that can be swallowed whole or opened and sprinkled on food, and as Depakote ER (“Extended Release”) tablets. Since all of the medication in the ER tablets is not quite available biologically to the patient, the daily dosage of Depakote ER tablets runs 8–20 percent higher than when other forms are used. Valproic acid is a very effective anticonvulsant with a very broad spectrum of activity. It does not raise cholesterol levels. Originally hailed as an anticonvulsant for all types of generalized seizures (including tonic-clonic, absence, and myoclonic), it has since been recognized to be very effective for partial seizures as well. Thus, valproic acid is particularly useful for patients who have a mixture of seizure types. So why aren’t all patients with seizures being treated with valproic acid? Although usually well tolerated, there are significant possible side effects. (See above for possible effects on the fetus.) Common possible side effects include:

• • •

weight gain, which often becomes problematic

•

mild abdominal discomfort (which is minimized by taking the medication with meals, use of a long-acting preparation, or by taking the shorter-acting preparations in smaller, more frequent doses).

tremor brittle hair (which is usually not a significant problem, and can be reduced with selenium and zinc vitamin supplements).

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Rare but serious possible side effects include:

•

Bone marrow suppression. The most common type is an isolated suppression of the platelet count, which can result in easy bleeding, bruising, or petechiae. (See section above on clinical monitoring of anticonvulsants.) The platelet suppression is usually dose related and reversible. More generalized or irreversible bone marrow suppression is quite unusual with valproic acid.

•

Liver dysfunction. The risk factors for liver failure from valproic acid include:

° Age: child age less than two years old is the greatest risk, and more than ten years old is the least risk. ° Use of additional anticonvulsants. ° Neurological impairment, especially for those whose history is consistent with “metabolic disorders.” Metabolic disorders might be considered for children with unexplained developmental delay, low muscle tone, a history of protein intolerance, or a family history of sudden unexplained childhood death. Emergency symptoms of metabolic disease include spells of severe vomiting; significant lethargy; unusual odors to the breath, skin, or urine; or unexplained rapid breathing. The risk of liver failure with all these three risk factors is greatest; the risk of liver failure for a normal child more than ten years old on just valproic acid approaches zero. The symptoms of liver failure are discussed in the above section on the clinical monitoring of anticonvulsants.

•

Pancreatitis. This is an unusual, but potentially very serious, possible side effect. Clinical symptoms of pancreatitis include abdominal pain, nausea, vomiting, or loss of appetite. These symptoms should be reported immediately to your doctor.

•

Polycystic ovaries and polycystic ovarian syndrome possibly occur at a higher rate in women taking valproic acid. Symptoms include increased skin hair, masculinization, acne, weight gain, and fertility and menstrual problems.

benzodiazepines The benzodiazepines are a class of medications of which the most well-known is diazepam (Valium). These medications are used for both anxiety and seizures. The ones most commonly used in the US for seizures are:

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• • • •

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lorazepam (Ativan) clonazepam (Klonopin) clorazepate (Tranxene) diazepam (Valium and rectal Diastat).

These medications actually have a wide spectrum of activity, but they are rarely used alone because (1) they have sedating and possible cognitive slowing effects; and (2) patients build up a tolerance to them after a few months or so, requiring the dosage to be increased, or the medication to be changed to a different benzodiazepine. Thus, their primary role is as an add-on medication for the kinds of seizures that they control well but standard anticonvulsants do not: for myoclonic, atonic, absence, and atypical absence seizures. A rectal gel form of diazepam is marketed in the US under the brand name Diastat. Diastat is discussed in the section on prolonged seizures (“status epilepticus” on p.23). This class of medication is actually fairly safe. It is a “what-you-see-iswhat-you-get” type of medicine. If it’s working, the child is on enough. If the child is sleepy, he or she is on too much. High doses can cause respiratory suppression, especially if given intravenously, or if given rapidly to a patient who is not used to it. It is very unlikely to cause an allergic reaction, bone marrow or liver problem. Drug levels are not clinically useful, and blood tests are not typically followed with these medications unless there is some special symptom. There can be additive sedation when these medications are used with other sedating drugs. Always check for drug interactions with your doctor and pharmacist. The combination of valproic acid and benzodiazepines has been reported to cause prolonged absence seizures. When used for seizures, patients build up a physical but not emotional dependency on the medications. As a practical matter, that simply means that this class of medication cannot be stopped suddenly, for concern that sudden discontinuation could cause severe seizures or “hangover” withdrawal symptoms.

The “newer” anticonvulsants In general, the “newer” anticonvulsants are first approved in the US as “add-on” therapy for older children and adults who are already taking a traditional anticonvulsant. Those restrictions are largely based on a research artifact wherein it was felt unethical to do a randomized study where half of the patients are placed on a known effective drug, and the other half on an as-yet-unproven experimental drug. Thus, the research compromise was to see whether or not a new drug offered additional benefit when “added on” to the previously used medication. The age

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restriction comes from the limited number of children who were studied during the original trials. Fortunately, recent US laws will now require that new medications be fully tested (and thus be potentially approvable) in children as well as adults. The newer anticonvulsants tend to be significantly more expensive than the older ones. There is always concern over the relative lack of experience and long-term follow-up with any new medication. These concerns need to be balanced against the promise of important new safety and tolerability features.

oxcarbazepine (Trileptal) Oxcarbazepine (Trileptal) is currently FDA approved to be used by itself or in conjunction with other anticonvulsants for the treatment of partial seizures in patients four years of age or older. It is possible that it might worsen some types of generalized seizures such as myoclonic spells. Although it is a somewhat new anticonvulsant, it is quite similar to carbamazepine (Tegretol). Oxcarbazepine has rapidly become a commonly used anticonvulsant. It is usually quite well tolerated, and seems to cause less sedation, less liver inflammation, and less bone marrow problems than the similar drug carbamazepine (Tegretol) that it is often replacing. The most common side effects are sedation, dizziness, unsteadiness, vomiting, stomach upset, visual symptoms, and tremor. On occasion, it can cause a low sodium level in the blood. Additionally, there have been recent warnings about severe skin allergic reactions (“Stevens–Johnson syndrome”) and multi-organ failure. This medication may also reduce the effectiveness of birth control pills. When following blood tests, it may be worthwhile to get a comprehensive chemistry panel (including liver function tests and sodium level) rather than testing the chemistries solely for liver function tests. levetiracetam (Keppra) We are not sure how levetiracetam (Keppra) works—it seems to have its own novel mechanism of action—but it seems to work very effectively and safely. It is FDA approved for patients four years and older as add-on therapy for the treatment of partial onset seizures. Also, it appears to have a quite promising broad spectrum of activity above and beyond the partial seizures for which it is approved—including generalized seizures of probably all types. The most common side effects are sedation, weakness, dizziness, and headache. Behavioral problems can sometimes be quite significant—ranging from apathy to agitation and hostility—and may sometimes necessitate cessation of the medication. Otherwise, it is expected to have a welcome lack of serious organ damage. Drug interactions are minimal. It may take two weeks to see the benefit of the medication.

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gabapentin (Neurontin) Gabapentin (Neurontin) is US FDA approved for add-on therapy for partial seizures (with or without secondary generalization) in patients who are three years of age or older. Despite its many enticing safety features, many neurologists seem to remain unconvinced that gabapentin is as effective as other anticonvulsants. It may turn out to be an excellent choice for use in benign childhood epilepsy with centrotemporal spikes. Gabapentin is a fairly safe anticonvulsant. It is a “what-you-see-is-whatyou-get” type of medicine. If it’s working, the child is on enough. If the child is sleepy or dizzy, he or she is on too much. Behavioral problems can sometimes occur with gabapentin. Although drug levels can be done if desired to get an idea of how much more medication the child might need, other blood tests may not need to be routinely monitored. It also has the appealing feature of not interacting with the levels of most other medications. topiramate (Topamax) Topiramate (Topamax) appears to have a very broad spectrum of activity, including partial and all types of generalized seizures. It is currently FDA approved for initial use alone in patients ten years of age or older who have partial seizures or primary generalized tonic-clonic seizures. The FDA approved age range is extended, though, when used for the add-on control of partial and primarily generalized tonic-clonic seizures in patients two years of age or older, and for seizure types associated with Lennox–Gastaut syndrome. Topamax appears to be a fairly effective anticonvulsant. It has developed somewhat of a reputation though, for its ability to cause patients to be sleepy, think slower, and have language problems. These effects seem to be minimized by a slow titration to a lower dose. Children are also prone to loss of appetite and weight loss, attention and memory problems, and behavioral changes such as aggression or anxiety. Rarely, eye pain or visual disturbance can be signs of acute glaucoma and should be brought immediately to a doctor’s attention. Patients are at some risk for an inability to produce adequate amounts of sweat in hot weather (a condition called anhydrosis), and may subsequently develop a dangerously high body temperature. Topiramate can cause abnormal sensory symptoms. It may also cause kidney stones, which might be detected with routine urine analysis. Given the concerns about kidney stones, and the trouble that some kids have with sweating, patients need to stay well hydrated. Topiramate can also occasionally cause serious disturbances in the blood chemistries, such as a low bicarbonate level. These need to be monitored periodically with a full blood chemistry panel—typically done in conjunction with a drug level, complete blood count, and urine analysis.

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lamotrigine (Lamictal) When lamotrigine (Lamictal) was first introduced, neurologists felt that it was going to be used often and for a broad spectrum of childhood seizures. However, the occurrence of potentially severe rashes has tempered its use to be more confined to its current FDA approved uses:

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add-on therapy for children two years or older with partial seizures

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monotherapy (treatment with just one anticonvulsant) for adults with partial seizures who are converting from other anticonvulsants.

add-on therapy for children two years or older with the generalized seizures (tonic-clonic, atonic, and myoclonic) of Lennox–Gastaut syndrome

There are many important drug interactions with lamotrigine. Anticonvulsants that tend to slow liver metabolism (such as valproate) greatly raise the lamotrigine level. This explains the increased risk of Stevens–Johnson syndrome from the combination of lamotrigine and valproate. In contrast, lamotrigine levels are reduced in the presence of anticonvulsants that have revved up the liver, such as carbamazepine, phenytoin, and phenobarbital. Lamotrigine usually does not conversely alter the drug levels of the other anticonvulsants themselves. However, the combination of lamotrigine and carbamazepine can result in visual symptoms, possibly due to elevated carbamazepine metabolites that are not measured with normal drug level tests. The major worrisome possible side effect of lamotrigine is a rash in 10 percent of patients, which can progress to the severe condition called Stevens–Johnson syndrome (see section above on monitoring anticonvulsants with clinical symptoms, pp.42–43). According to the manufacturer’s package insert, lamotrigine should ordinarily be stopped at the first sign of a rash unless there is another explanation for the rash. Risk factors for Stevens–Johnson from lamotrigine include patient age being less than 16 years old; and apparently also, the simultaneous use of valproate, or the rapid upward titration of the lamotrigine as it is being introduced. Stevens–Johnson occurs in 8 out of 1000 children less than 16 years of age who are also taking other anticonvulsants. A study showed that one child died of the severe allergic reaction out of 1983 children taking lamotrigine in combination with other anticonvulsants (Lamictal package insert, 2005). Lamotrigine may worsen myoclonic seizures in some patients. Lamotrigine is otherwise usually fairly well tolerated.

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tiagabine (Gabitril) Tiagabine (Gabitril) has a narrow spectrum of activity. It is FDA approved for use in the US as add-on therapy for partial seizures in patients 12 years of age and over. It has few serious side effects, but like most of the anticonvulsants can be associated with dizziness, fatigue or generalized weakness, slowed thinking, behavioral changes such as anxiety or depression, tremor, or abdominal pain. A total of 13 percent of patients had to drop out of the studies due to such side effects (vs. 5 percent of patients on placebo). No abnormalities were found in the standard blood tests (Schachter 2001). The therapeutic range for this medication has not been set. According to the package insert, “prescribers should be aware of the possibility of long-term ophthalmologic effects.” This concern may help explain why tiagabine has lagged in popularity. Tiagabine levels in the bloodstream can be affected by the use of other anticonvulsants, although tiagabine does not significantly alter the level of other drugs. Tiagabine has been used “off label” (meaning not yet FDA approved) for other problems such as pain, muscle tone, or psychiatric disorders. Counter-intuitively, such uses of tiagabine have been reported to be associated with new-onset seizures (including prolonged seizures). Tiagabine should be tapered off slowly in order to avoid the precipitation of withdrawal seizures. zonisamide (Zonegran) Zonisamide (Zonegran) is FDA approved for use as an add-on medication for adults with partial seizures. There may be severe allergic skin reactions, and significant reactions in patients who are allergic to sulfonamide drugs. Loss of the ability to sweat can cause significant hyperthermia (high temperature) and even heat stroke. Patients should avoid excessive heat and stay well hydrated. Several percent of patients have kidney stones. felbamate (Felbatol) Felbamate (Felbatol) will only be started now under exceptional circumstances because of the high risk of bone marrow failure and liver failure. vigabatrin (Sabril) Vigabatrin (Sabril) is not approved in the US because of a high risk of visual field deficits (loss of sight in certain areas of vision). This may be particularly hard to detect in uncooperative children. In certain cases, though, vigabatrin is particularly useful.

Chapter 8

T R E AT M E N T OP T I O N S O T H E R T H A N M E D I C AT I O N

Introduction Anticonvulsant medications are usually considered the first line of treatment for seizures. (See Chapter 7.) Of course, part of the first treatment plan also includes “healthy living.” This means plentiful and regular sleep, good eating habits, and the avoidance of alcohol and other recreational drugs. Most of the time, these treatments will be successful. However, if the child has not tolerated and/or achieved adequate seizure control during trials of at least two anticonvulsants, then it gets increasingly unlikely that another anticonvulsant will work completely. Thus, after two or three unsuccessful attempts with medication, other approaches may be considered. In this chapter, we briefly introduce the major non-pharmacological treatments, which include: the ketogenic diet, the modified Atkins diet, the vagal nerve stimulator (VNS), and epilepsy surgery.

Ketogenic diet It has been known for hundreds of years that fasting has helped to reduce seizures in some patients with epilepsy. The formal ketogenic diet (literally meaning a diet “giving rise to ketones”) was initiated in the 1920s, but largely grew out of favor as the simpler anticonvulsant therapies advanced. However, after the successful use of the diet by a boy named Charlie in 1994, interest resurfaced. (See www.charliefoundation.org). So, what is the ketogenic diet? The child is allowed to eat a diet consisting of a large ratio of fat compared to low protein and very reduced carbohydrate. This creates a metabolic state called “ketosis,” which seems to help seizures. We still do

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not know why it works. A typical meal consists of a very small portion of meat, fish or chicken; another almost as small portion of fruit; and the rest as a combination of butter, mayonnaise, and heavy cream. Not surprisingly, this is a difficult diet to enforce in children who are physically able to reach other food on their own. One “stolen” cookie can cause a loss of seizure control. Hidden sugar often is found in unexpected places such as in medication preparations and toothpaste. The diet is initiated under the care of a neurologist and dietician experienced in its use. The ketogenic diet must be carefully supervised! Typically, this would include a several-day hospitalization during which the child fasts in order to bring about the ketotic state more rapidly. The child needs to be carefully monitored for low blood sugar. During the hospitalization, the family is taught how to use the diet. Just because this treatment does not involve medication does not mean that it is without risks! There is nothing “natural” about this diet. We can expect poor weight gain and constipation. There may also be other difficulties such as growth problems and the development of kidney stones. Cholesterol, triglyceride, and lipid levels can be significantly altered, but can often be addressed with alterations of the diet. Vitamin supplementation will be required. Also, an escape of seizure control can occur if the diet is even slightly broken. Overall, though, if the ketogenic diet is discontinued, it is usually because it did not work, not because of side effects. The diet is clearly not for everyone with intractable epilepsy. Children with ready access to sneaking other food can be difficult candidates, and many children just won’t take the food. Patients with certain rare metabolic diseases can be worsened by the diet. What can we expect from the diet? Seizure control may improve within a few days to weeks. By six months, half of the patients will have a 50 percent or greater reduction in seizures. Of the children with such a response, half again will have a greater than 90 percent response. Complete seizure control is achieved by about 10 percent of children on the diet (Kosoff 2005, p.13). The diet seems to help many kinds of seizures, but is particularly useful for children with atonic seizures. On the other hand, complex partial seizures may not significantly improve. Placebo-controlled trials are underway. After two years, the ketogenic diet is often gradually broken in order to see if the child still needs it. It can be restarted if needed, although not all patients regain the seizure control. When effective, another big additional effect of the diet is that the child may be able to gradually come off medications that may have been causing side effects such as sedation.

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Modified Atkins diet There has been recent interest and ongoing research into the “modified Atkins diet.” Compared to the traditional ketogenic diet, the modified Atkins diet allows unrestricted calories and water, more protein and carbohydrate, and less fat. It does not require weighing of the food, allows eating in restaurants, and is started without hospitalization. Research is underway to study the effectiveness of the modified Atkins diet.

Vagal nerve stimulator (VNS) The vagal nerve stimulator (VNS) is a small device surgically implanted under the skin just below the patient’s collarbone. Thin wires from the device are carefully brought into the neck and wrapped around the vagal nerve. Periodic electrical impulses from the VNS onto the vagal nerve seem to desynchronize seizure activity in the brain, although no one really knows how the procedure works. Adjustments to the VNS settings are made with a computer via a magnetic wand. The magnetic wand may sometimes even be used to abort an actual seizure. The VNS is approved in the US for patients aged 12 and older, but has been used in young children as well. The procedure is usually well tolerated, but there are always risks to any surgical procedure that need to be discussed with your doctors. The device may cause hoarseness or coughing. Depending on the type of study, there seems to be an overall 25–40 percent reduction of seizures with the VNS—roughly the same improvement seen with most of the new anticonvulsants (Kosoff 2005, p.15). Although VNS is not expected to provide complete seizure control, it may reduce the number of seizures while simultaneously allowing a reduction of the patient’s medications. Improvement may takes months, or even years. Some patients seem to become more alert and less depressed with the use of the vagal nerve stimulator.

Epilepsy surgery When standard medical and other treatments have failed, some patients are excellent candidates for “epilepsy surgery” on their brain. This is rarely needed! Detailed EEG and imaging studies (see Chapter 5, Medical Evaluation of Seizures) allow the epilepsy center team to pinpoint the exact location for surgical excision. Other surgical options include a larger area of resection, such as removing part or all of one brain hemisphere. For such procedures to be effective, it must be shown that the seizures reliably originate from specific brain parts, and

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that these areas are in operable locations. Rarely, a “corpus callosotomy” might be performed to sever the “highway” which connects the two sides of the brain. This prevents the spread of the seizure to the other side of the brain, and seems particularly useful in the setting of frequent atonic (drop attack) seizures. See Freeman et al. (2002) for more details.

What about “homeopathic” treatments? Other than using vitamin B6 as treatment for a possible rare cause of recalcitrant neonatal/infant seizures, nutritional supplements with vitamins, amino acids, and herbs have not yet been proven to have any beneficial effect on seizures (Devinsky 2002, pp.66–70). Just because these substances exist “naturally” somewhere on our planet does not mean that taking them in high doses is “natural.” Objectively, it is difficult to see the appeal of “homeopathic remedies” that have never been adequately tested (if at all) for either efficacy or safety. (Supplements with vitamins to help minimize side effects of the anticonvulsants are discussed in the previous chapter, Treatment with Medication.)

Chapter 9

F O R KI D S T O RE A D W I T H T H E I R PA R E N T S : W H AT A R E S E I Z U R E S, A N Y WAY ?

A note to the parents Some parents are afraid to talk to their kids about this stuff.

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Parents are afraid that the child might get scared if they talk about it. They shouldn’t be. Children already know that something special is going on. Who knows what weird ideas might float through a kid’s head if he or she isn’t being directly told the right information? And think how much worse people feel when they have no one to whom they can ask questions.

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Parents are afraid that the child won’t understand everything. They shouldn’t be. Whatever children can’t begin to understand won’t bother them—they won’t even notice. If something does bother your child, then make sure that he or she knows that he or she can always ask questions.

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Parents are afraid that they don’t know how to explain seizures to their child. That’s an easy one. Parent and kids can all read this chapter together. Parents can also get materials for kids from the Epilepsy Foundation of America at www.epilepsyfoundation.org.

Kids, let’s get started! Kids with seizures often ask, “What is a seizure, anyway? Am I normal? Am I going to be OK? It’s my life. I want to know.” The first way to find out about your own seizures is to ask your parents and your doctor.

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You can also read about seizures, and here’s what you need to know. If you don’t understand something—or if something upsets you—then ask about it!

What is a seizure? A typical brain like yours has about 10 billion (10,000,000,000) special cells called nerve cells or “neurons.” These nerve cells are all bunched up together, and “talk” with each other using electricity. Certain chemicals in your brain help keep all of this electrical activity from getting out of control. In a seizure, a “short circuit” occurs, which makes the nerve cells keep firing. Since these brain cells control your muscles, then the muscles keep firing. This stiffening or jerking of your muscles is what everyone sees and calls a seizure. Sometimes, your body might just go into a staring spell, but it’s due to the same kind of short circuit.

What is “epilepsy?” “Epilepsy” is just a fancy doctor word for “more than one seizure.” Don’t worry: it’s not contagious, and it has nothing to do with how smart you are. It just means that the person has had more than one seizure.

Do lots of people have seizures? Yes! About one out of ten people will experience a seizure at some point during their life. About 1 out of 150 people will have more than one seizure, i.e. have “epilepsy.” Since you probably have met many more than 150 people in your life, that probably means you already know other people who also have seizures—you just don’t know it because they’re normal. So, you have plenty of company. If you go to a major league baseball stadium, there are probably 300 people in the stadium who have epilepsy. And you have plenty of good company, too. For example, Joan of Arc, Ludwig van Beethoven, Michelangelo, and Julius Caesar all seemed to have had seizures.

Are there different kinds of seizures? Yes. Some kids have a warning before the seizure starts. That’s called an “aura.” The aura can be a funny movement of one part of your body or face, a funny feeling in your stomach, a tingling feeling, funny vision or speech, or just about anything your brain is capable of doing. It’s basically just the actual beginning of a seizure.

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During an aura, the part of your brain that is not having the seizure is watching the part of your brain that is having it. These are called “partial seizures,” meaning that they start in only part of the brain. If you have a special way that your seizure starts, make sure to tell your parents and doctors. That way, they can best help control your seizures. Some kids have no warning before the seizure. Perhaps the aura (warning) was so brief that it couldn’t be noticed. Or, perhaps, the seizure is the type that begins by immediately involving the whole brain. If the whole brain is having a short circuit, then there isn’t any part of the brain awake enough to keep you properly alert. These are called “generalized” seizures, since they have generalized to the whole brain. During the generalized part of a seizure, we are not aware of what is happening to us. People around us may say that we were staring off into space like a zombie, or they may say that we fell to the ground and were totally “out of it” and shaking. Sometimes, the seizure is just a quick jerk of our arms, and that’s it. If you are holding something, it might suddenly drop from your hand. This type of seizure often happens in the morning. If you have this type of “myoclonic” seizure, tell your parents and your doctor! Other things that can happen during a seizure:

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Sometimes, we might bite our tongue. It may hurt a little, but usually isn’t serious.

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If we’ve drunk some liquids recently, then our bladder may cause us to “wet ourselves.”

After a seizure, we may be sleepy or feel bad for a brief time.

What causes the seizures? Do you want to see your parents get frustrated? Watch them when the doctor tells them that they usually can’t find the cause of your seizures. Usually, in kids, seizures are just due to the chemicals in their brain having electrical short circuits. Sometimes it runs in families, but usually not. Only rarely would a child’s seizures be due to something “bad.” Don’t worry. Your doctor has probably already checked.

Will I need tests? The doctor may have ordered an electroencephalogram (an “EEG”) or a Magnetic Resonance Imaging scan (an “MRI”), or blood tests. During an EEG, thin wires are pasted onto your head, and the electrical activity of your brainwaves is recorded. It is perfectly painless. Usually we want kids to get a

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good night’s sleep—especially if they have a seizure condition. However, sometimes the night before an actual EEG, the doctor may ask that you stay up really late. (That’s the good part. The bad part is that you may also be asked to wake up early.) For some types of seizures, taking an EEG when you are tired helps make the EEG more accurate. An MRI is another painless test. (See Figure 9.1.) Magnet and radio waves are used to take a photograph of the inside of your head. There are a lot of knocking sounds. It’s noisy but pretty interesting. It’s kind of like a space ride at the amusement park. All you have to do is stay still. Ask the people who take the pictures if you can see them.

Figure 9.1 An MRI machine and the pictures that it takes

Will the seizure hurt me? It is very unlikely that a seizure will hurt you! You know, no one can ever guarantee you things in life; but fortunately, it turns out that you are very unlikely to be hurt by a seizure. We don’t expect it to cause you any kind of brain damage. After all, you’ve already had a seizure and you’re OK, aren’t you? What better evidence could we offer? Now, that’s not to say that a seizure is exactly good for you, either! Many kids find it embarrassing (not that they need to be embarrassed), and you might have an accident during a seizure. So, it is important to regularly take any medication that your doctor might give you. (Of course I’d say that—I’m a doctor writing this!)

Can I still play video games? We want your life to be as wonderful as possible. For many kids, that includes playing video games. There is no evidence that playing video games causes epilepsy. However, some people think that the flashing lights of video games might rarely

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trigger a seizure in someone who already has a seizure disorder. Especially if the flashing strobe lights during your EEG triggered seizure spikes (ask your doctor), then you might be at a higher risk for a seizure while playing or watching video games. But the EEGs of only 3 out of 100 kids show such a reaction to the flashing lights—not very common. If anyone is worried, your parents should watch you while playing, and you should report any weird feelings while playing the video game. Don’t play for too long a time or when you are really tired. It may be a good idea to lessen the experience of flashing lights during a video game: choose games with less flashing activity, use a handheld game machine (which flashes less than a full screen), use a small screen and stay as far away from the screen as possible, and turn up the lights in the room to make the TV screen flashes less noticeable.

Are there any special things I can do to help myself ? Yes! I’m glad that you asked! Most importantly, be actively involved in your own seizure care. Take your medicine! Ask questions. Tell your parents and doctors about any problems or fears that you may have. It’s your life. You can get more information from books and online. Start at the Epilepsy Foundation of America’s website at www.epilepsyfoundation.org. They have information for kids and teens. Dr. Eric Chudler’s Neuroscience for Kids has seizure information at http://faculty.washington.edu/chudler/epi.html. You might also want to read the following chapter about teens and seizures with your parents. In addition, there are a few things you can do to keep yourself safe in case you have another seizure. Your doctor may have somewhat different suggestions.

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If you have a warning that a seizure is coming (called an “aura,” remember?), then call for help and get yourself into a safe position if possible (such as lying down or sitting).

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No climbing higher than about six feet (the height of an adult).

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Use common sense.

No riding your bike around cars for a while. Always wear a bike helmet (whether you have seizures or not). An adult always needs to supervise carefully when you are near or in water, including bathtubs.

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Other than that, we try to let you live a totally normal life! You can play almost all sports, have sleepover dates, and all that good stuff.

Will I be OK? We expect only good things for you. Most kids outgrow their seizures, and can stop taking any anti-seizure medication after a few years. Your doctor will discuss this with your family. People with seizures usually live totally normal lives. They get married, have kids, have jobs, and even have dogs if they want. A few jobs—like being a jet pilot—are probably not good ideas.

So, am I normal? Yes! If you still don’t believe it, go back and read this chapter again. Most (but not all) kids outgrow their seizures after a few years. Good luck. And don’t forget to keep asking questions.

Chapter 10

C H A P T E R ON (A N D FO R ) TEENS WITH EPILEPSY Marissa A. Broadley and Martin L. Kutscher

As if being a teenager isn’t hard enough, now you’ve got this seizure thing to deal with also. More questions about your life are probably not just what you needed! So, to find some answers about seizures, start by reading the basic material in the previous chapter for kids. Then read this chapter, which deals with issues of particular interest to teens. Or, read whatever parts of this book that you want! Dr. Devinsky’s book (2002)—see the Further Reading section—is geared more to adults with epilepsy, and covers in more detail some of the “grown up” issues such as the workplace and family planning.

Will I be able to drive? Driving is probably the most exciting privilege that a person gains as a teenager. However, along with most privileges comes responsibility. People with seizure disorders have more responsibility and regulations to follow when it comes to driving. Your doctor should be able to tell you about local driving regulations related to people with seizures. In the US, you can find information about your own state’s driving laws at the website of the Epilepsy Foundation of America at www.epilepsyfoundation.org.

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How long do I have to be seizure free before I can start driving? If you have had a seizure, the law in most states will require a period of time during which you are seizure free—to give reasonable evidence that your seizures are under satisfactory control. This observation period will be required whether you are taking anticonvulsants or not, and even if you

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have only had one recent seizure in your life. Discuss this with your doctor.

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What happens to my driving privileges if there is a change in my medication, or if I am ready to taper off of the medication? You will probably have to suspend driving when you are switching or stopping medications. For how long? Well, most seizure recurrences occur within three to six months after tapering the medication (Freeman et al. 2002, p.354). Many doctors typically have patients wait a year after tapering their medications before they can drive. Your doctor will help you to understand the laws in your area, and you should discuss any issues or questions about your particular situation with your doctor. The law recognizes that epilepsy puts the driver at some risk for an accident. However this risk is no greater than for people with cardiovascular disease or diabetes, and far less than the risk for people driving under the influence of alcohol (Freeman et al. 2002, p.352). The law and the doctors try their best to balance your right to drive vs. the need to protect both you and other people on the road. Ultimately, though, there are no guarantees. That’s life.

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Am I obligated to inform my state’s Department of Motor Vehicles? Each state has its own set of laws as to who is supposed to contact authorities about seizures or other spells which impair consciousness. Make sure you know the information for your state. See www.epilepsyfoundation.org. Discuss these issues with your doctor, and play it straight. Usually, the law’s restrictions will be no more restrictive than what you and your doctor would have worked out, anyway. Should an accident occur, at least you have been driving legally.

What should I do if I can’t drive? If you are unable to drive, there are some options to help you compensate for this limitation—as the inability to drive is often a social and economic hardship. Of course, if you live in a city with good public transportation, you are set! Otherwise, you can have friends and family members give you rides, or use available public transportation such as buses. When choosing a college, apartment, or job, try to choose locations where driving is not required. And if you can drive… BE RESPONSIBLE!!! This is the only way to lower any risk of having a seizure and maintain your driver’s license.

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1.

Get enough sleep!!!

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Do not use alcohol or recreational drugs!

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Take your medication as your doctor recommends—do not miss doses!

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Do not drive if you “feel” like you may have a seizure.

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Make sure all of the paper work for the department of motor vehicles is done correctly and in a timely fashion.

Bottom line: it’s lousy that you can’t drive during certain periods. But it’s the law; and if you think about it, it’s the only safe thing to do—for others and yourself.

Should I tell my friends and dates about my seizures? If your seizures are infrequent, you can initially hold off on telling most friends and dates about your condition. However, once relationships get more comfortable or serious, you probably should tell the people. A great benefit to this is that they will know what to do if you have a seizure. If you are uncomfortable telling friends, then only tell those who really need to know. Remember that, if they reject you because you have epilepsy, those friends are not very good friends at all. If you do decide to tell friends, make sure they have the right information. You should include: What is epilepsy? How do they know if you are having a seizure? What should they do if you have a seizure? The answers to all of these questions are found in other chapters of this book. In fact, you should probably read those chapters for yourself as well. Remember: your attitude towards your own seizures is likely to affect your friends’ attitude as well. You have lots of traits. Epilepsy does not define you!

What about pregnancy? If sex should become even a possibility in a relationship, it is important that partners know about the seizure disorder. It is also absolutely imperative that you talk with your doctors about it. Although some issues do involve males with epilepsy, most issues regarding sexual activity and epilepsy are related to females. Women with seizures need to plan their pregnancies well in advance of actually becoming pregnant! There are multiple issues to address:

•

Can anticonvulsants affect birth control medications? If you are sexually active, you want to make sure that the most effective contraception is used. Some anticonvulsants alter the effectiveness of the

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birth control pill and the morning after pill. Talk to your doctors—in advance!

•

Can pregnancy affect my seizure control? Pregnancy seems equally likely to worsen, to improve, or to make no change in seizure control. Certainly, there are women for whom pregnancy makes the seizures worse, though. This can be partly due to alteration of anticonvulsant metabolism (and thus blood levels) during pregnancy, and drug levels will need to be followed very closely.

•

If I have a seizure while pregnant, is that dangerous? The seizure could cause an accident for the woman, and prolonged seizures in the mother might affect the fetus. There is little evidence, though, that a fetus is hurt by a brief seizure of the mother.

•

If a parent has epilepsy, does that increase the risk of having an abnormal infant? For a mother who never had seizures, the risk of a developmental abnormality in her infant is about 2–3 percent. If the mother has (or had) epilepsy, the risk doubles to just 4–8 percent. If the father, instead, has epilepsy, the increased risk of a developmental abnormality is even less (Freeman et al. 2002, p.357).

•

Do anticonvulsants increase the risk of having an abnormal infant? Whereas some studies show no further increased risk (other than the small risk discussed above that comes just from having a parent with epilepsy), others show the risk to be from 7 to 17 percent of infants having a developmental problem if the mother was on anticonvulsants (Freeman et al. 2002, pp.358). The most obvious way to avoid anticonvulsant-induced fetal anomalies is to avoid being on anticonvulsants during pregnancy. This takes planning! Well in advance of a planned pregnancy, it may be advisable to undertake a trial to see if the mother still needs anticonvulsants. Anticonvulsant medication should not be used during pregnancy unless truly needed.

•

What about folic acid? Certain types of anticonvulsants are of particular risk for causing problems such as fetal spinal cord abnormalities. Folic acid seems to help reduce (but not eliminate) the risk of such fetal defects from the anticonvulsant medication. Most of the damage is done during the fetus’ exposure to the anticonvulsant in the first few weeks of gestation, even before the woman has missed her period. To play its role, women must be taking folic acid before they know that they are pregnant. Depending on which anticonvulsant you are taking, your doctor may advise all women of childbearing age who are taking anticonvulsants to take folic acid. Note that anticonvulsants typically do not have any significant negative effects on the sperm or eggs before an actual pregnancy.

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•

If one of the parents has seizures, will my child have seizures? The risk of epilepsy in an infant born to either parent with epilepsy is about double that for an infant whose parents do not have seizures—but even so, at least 96 percent of the offspring will not have to deal with epilepsy! This is the same risk for siblings of a child with seizures: if one child in a family has idiopathic epilepsy, then the other siblings also have a 96 percent chance of remaining seizure free (Freeman et al. 2002, pp.360–362). Certain epilepsies, such as Juvenile Myoclonic Epilepsy, are at a higher risk of being passed genetically.

•

What if a girl misses her period? She should discuss this with her doctor immediately. If the missed period is from pregnancy, then it will need to be carefully addressed. Irregular menses can be part of the polycystic ovary syndrome, especially if the woman is taking valproic acid (Depakote). See Chapter 7 on medication treatments.

•

Can I be a good mother? Of course!

Will I be able to get a job and have a career? Getting a job Will you be able to get a job? Yes, of course! Some teenagers have summer and after-school jobs. Some want the extra money for fun things and others need to work in order to pay for necessities. If you should decide to work along with going to school, be sure it does not interfere with your sleep. Sleep is essential for people with seizures. Also, do not stress yourself too much—a high level of physiologic stress is another risk factor for a seizure. Balancing school and extracurricular activities is hard enough on any teenager. Adding part-time employment is even more difficult. Careers As you get into your late teens, you may be thinking about what you want to do with your life. Well, for the most part, you should not have a problem doing what you want to do. Here are some careers you probably cannot have: 1.

Commercial airline pilot. (Hey, they are strict. You have to have perfect vision for that, too!)

2.

Livery driver. (This means you can not have a job in which you drive vehicles for the purpose of the transportation of people, i.e. buses, trains, cabs, planes, etc.).

3.

Typically, you cannot join the armed forces. This includes but is not limited to the navy, army, marines, and air force.

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Other than that, you should not be too limited. Use common sense, but don’t let epilepsy get in the way of your dreams. You should be able to be what you want to be.

What are my rights? You have the right to be treated with dignity, respect, and appropriate accommodations. You are protected in the US under the Americans with Disabilities Act. See information on discrimination during employment at www.eeoc.gov, the website of the US Equal Employment Opportunity Commission.

Off to college! If you are thinking about a career, you may also be thinking about college. College life involves a huge change of lifestyle, as you will probably be moving away from home to live in a dormitory. Now, it’s up to you to take care of yourself! This is a time when you will experience new things and become very independent. Your parents will no longer be able to make sure that you take your medicine every day, or that you don’t stay out all night partying. Here are some things you should do when you go to college. 1.

Someone should know about your seizures. You should probably tell your roommate, but at least tell your RA (resident assistant) or RD (resident director). Both the RA and the RD usually live in the same building as you.

2.

Do not party all night or pull all-nighters. Plan in advance for tests and papers. You need your sleep!

3.

Make sure you take your medications. A weekly pillbox may help you remember to take your medication—and whether or not you have already taken a dose.

4.

Do not experiment with recreational drugs. This is not negotiable.

5.

Do not drink alcohol. You are probably too young to drink it anyway!

6.

Try not to be alone for prolonged periods. Depending on how well the seizures are controlled, and on the types of seizures you have, this may be very important. If you must be alone, do not do anything that could be potentially dangerous if you should have a seizure.

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7.

Take care in or around water. Showers are safer than bathtubs, which can be dangerous when unsupervised. Someone who knows about your seizures and can call the lifeguard should be watching you while swimming.

8.

No climbing heights, in case you should fall.

9.

Discuss driving with your doctor.

Drugs and alcohol There is no question about it: using “recreational” drugs is risky and stupid—whether or not you have seizures—but especially if you do. Many illicit drugs can precipitate severe seizures or other problems, and can interact with your seizure medications. Regarding alcohol, binge drinking is dangerous and idiotic, and even more dangerous to people with seizures! Devinsky (2002, p.212) points out that one or two alcoholic drinks on occasion should not cause a significant change in anticonvulsant blood levels or in seizure control. (More than one or two occasional alcoholic drinks can clearly change some people’s drug metabolism.) However, he points out that unsupervised drinking of alcohol is very dangerous if you have a seizure disorder. There are several reasons. There may be an additive sedative effect during the combination of anticonvulsants and alcohol. Also, one or two occasional drinks can lead to more, and that is particularly dangerous! You may become so sleepy that you miss your medication, or may go on to more significant alcohol or substance abuse problems—conditions which can clearly increase your risk of having a seizure. Honestly, it’s better to just stay away from alcohol altogether. Besides, you’re probably below legal drinking age.

What about smoking? Are you crazy? We’ve been so concerned about the small risks of seizures and anticonvulsants, and now you want to smoke? Now you want to join the half million people a year that die from cigarette-related diseases? Although cigarettes do not clearly exacerbate seizures, the risk of having a seizure and thus burning down your house or apartment is greater for people with epilepsy. Your rights to smoke do not compare to other people’s rights not to be damaged by a cigarette-related fire occurring during a seizure. Now, you’re playing with other people’s lives as well as your own—and not even for any worthwhile benefit to anyone. If you don’t smoke, don’t start. If you do smoke, get help and STOP!

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Compliance with medication Teenagers are notorious for thinking, “It can’t happen to me!” Such denial is a normal and typical feeling of adolescents, who may need that attitude as they face the prospect of embarking out into the world on their own. However, in reality, it can happen to you! Take your medicine as prescribed! If you have concerns about taking the medication, discuss it with your parents and doctor. You might ask to talk to the doctor alone. But don’t just stop taking your medication. Sudden discontinuation of anticonvulsants can precipitate unusually severe seizures, and opens you to the risks of seizure recurrence (such as hurting yourself or others while driving). If there is any “enemy” in this whole area, it is the seizure disorder itself—not the medication. The medication is not the cause of the problem; it is on your side! Oddly enough, the quickest way to get off medication is to take it regularly. How so? Well, every time you skip your medication and have a breakthrough seizure, the clock usually gets reset to taking the anticonvulsants for another two years. So, the fastest way to get off the meds is to stay seizure free by keeping taking the meds regularly!

Who supervises taking the medication? As teenagers become adults, they need to assume “ownership” for the care of their own problems. Taking your anticonvulsants responsibly on your own is a way to take ownership of this issue. Your parents may not think that you are ready, yet, to reliably take your medication on your own. A good compromise is a seven-day pillbox that the teen can fill up on his or her own each week. The parents can—and should, at least initially—still double check the box to be sure that the medicine is being taken as directed.

Conclusion So, let’s face it. Some parts about having seizures simply stink. It’s not fair. But to put it very simply, nobody ever promised that life is completely fair. Realizing this simple fact is part of becoming an adult. Everybody has something in his or her life that “isn’t fair.” Some kids are born into a life of poverty and starvation, some are born in countries without freedom, some have parents who don’t love them, some have…well, you get the idea. Everyone has their problems; and you know what? Everyone makes it through them. You will, too. There is simply no other choice. It’s your life. Make the most of it!

Part 2

INDIVIDUAL EPILEPSY SYNDROMES

Chapter 11

I N T RO D U C T I O N TO THE EPILEPSIES

As we discussed in Chapter 1 (Overview of Seizures), there are two ways to classify information related to epilepsy: 1.

classification by the type of seizure event

2.

classification by the type of epilepsy syndromes that the seizure events are part of.

In Part 1, we discussed the classification of seizures—the individual events caused by brain short circuits. We covered the seizure types shown in Table 11.1.

Table 11.1 A simplified classification of seizure types Generalized seizures

Focal seizures

(that start initially throughout the whole brain)

(that start initially in part of one hemisphere)

• Absence seizures

• Simple partial seizures (consciousness remains normal during the seizure)

• Tonic-clonic seizures

• Complex partial seizures (consciousness is altered during the seizure)

• Myoclonic seizures

• Focal seizures that then secondarily generalize

• Atonic seizures

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In Part 2, we will discuss the epilepsy syndromes—syndromes marked by varying combinations of the seizure types above, the age of the patient, the EEG findings, the response to treatment, and the ultimate prognosis. Sorting out all of the possible epilepsy syndromes can be confusing. In fact, the classification of the epilepsy syndromes is still in the midst of a several-year revision by the International League Against Epilepsy (ILAE). In the proposed ILAE scheme, the epilepsies are first divided into those marked by either generalized or focal seizures. The focal seizures are further subdivided into:

• • •

idiopathic (doctors do not yet know why the seizures occur) familial (doctors have found a chromosomal abnormality) symptomatic (doctors have found a structural or other cause).

This text presents a simplified classification of the most common epilepsy syndromes, while remaining consistent with both the old and currently proposed classification schemes. In this classification by epilepsy syndrome, we will individually discuss the syndromes shown in Table 11.2. The full ILAE proposed classification has a host of additional syndromes (see www.ilae-epilepsy.org). Tables 11.3 and 11.4 show how the two systems inter-relate. Note that an individual type of seizure can be a building block in many kinds of epilepsy syndromes. For example, absence seizure events can be part of the epilepsy syndromes of childhood absence epilepsy (“petit mal”), juvenile absence epilepsy, juvenile myoclonic epilepsy, and Lennox–Gastaut epilepsy syndrome. In each of the following chapters, we will discuss a single epilepsy syndrome, starting with the focal epilepsies. Each chapter will utilize the format: 1.

brief, typical case study

2.

introduction and epidemiology of the syndrome

3.

evaluation, including typical EEG and MRI findings

4.

treatment

5.

expected outcome.

Table 11.2 A simplified classification of the major epilepsies Generalized epilepsies

Partial (also known as “focal” or “local”) epilepsies

Other syndromes

• Absence epilepsy (formerly called

1.Idiopathic focal epilepsies

• Febrile seizures • Neonatal seizures (not discussed in

“petit mal” epilepsy):

° Childhood ° Juvenile • Juvenile myoclonic epilepsy • Epilepsy with generalized

• Benign childhood epilepsy with centrotemporal spikes (a.k.a. benign rolandic epilepsy)

this text)

• Benign childhood occipital epilepsy: ° early onset (Panayiotopoulos type) ° late onset (Gastaut type)

tonic-clonic seizures only

• Infantile spasms1 • Lennox–Gastaut syndrome1 2. Symptomatic focal epilepsies

• Temporal lobe epilepsies: ° Mesial temporal lobe epilepsies – due to hippocampal sclerosis – due to other causes ° Lateral temporal lobe epilepsies • Frontal lobe epilepsies • Parietal lobe epilepsies • Occipital lobe epilepsies 1

Some classification systems would put syndromes such as infantile spasms and Lennox–Gastaut syndrome into a separate category of “Epileptic Encephalopathies,” which are epilepsy syndromes in which the seizures may contribute to progressive dysfunction.

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Table 11.3 Relationship between generalized seizures and generalized epilepsies Generalized (start both hemispheres) Type of seizure

Type of epilepsy syndrome Childhood and juvenile absence epilepsy (EEG: 3-per-second spike/wave)

Juvenile myoclonic epilepsy

Absence

++

+

Tonic/Clonic

10% risk

+

Epilepsy with tonic-clonic seizures only

Infantile spasms (EEG: hypsarhythmia)

Lennox– Gastaut (EEG: slow spike/ wave)

+ ++

+

Atonic

++

Myoclonic

++

++

+

Key: + common ++ very common (a defining feature)

Table 11.4 Relationship between partial seizures and partial epilepsies Partial (start in part of one hemisphere) Type of seizure

Type of epilepsy syndrome Idiopathic focal epilepsy syndromes

Partial simple

Benign childhood epilepsy with centrotemporal spikes (a.k.a. benign rolandic epilepsy)

Childhood occipital epilepsies:

+

+

Partial complex Partial seizures with secondary generalization Key: + common

+

Early onset Late onset

Symptomatic focal epilepsy syndromes Temporal lobe epilepsies: Mesial temporal Lateral temporal

Frontal lobe epilepsies Parietal lobe epilepsies Occipital lobe epilepsies +

+

+

+

+

+

+

Chapter 12

I D I O PAT H I C F O C A L E P I L E P S I E S : BENIGN CHILDHOOD EPILEPSY W I T H C E N T ROT E M P O R A L S P I K E S (BCECTS) Marissa A. Broadley and Martin L. Kutscher

Bill was eight years old when he snuggled with his family into a single hotel room while traveling. Shortly before dawn, a knocking sound awoke the parents. They discovered Bill, still in bed, jerking his whole body, but his left side more strongly than his right. He was drooling, but seemed to be breathing well. After a minute, the shaking slowed and then stopped. The parents tried to wake Bill up, but it took him a few minutes to become fully alert. The doctors asked if this had ever happened before. His brother, Artie (who shares the bedroom at home with Bill) piped in that he had heard Bill do something like that during his sleep a few months ago. Artie thought it was just a bad dream, and never mentioned it. When asked, Bill remembered a spell two weeks ago when the right side of his mouth seemed to freeze up for a minute. Bill thought it was a little odd, but since it had happened a few times before, he never mentioned it, either. Bill’s MRI was normal. His first EEG—done only while awake—was normal. His neurologist ordered a sleep deprived EEG, which showed seizure spikes coming from the center of the brain—sometimes from the left, and sometimes from the right. Since there had been multiple seizures already, the parents and doctors decided to treat with Trileptal. After two years of successful treatment, the EEG was repeated. It still showed some spikes, but the medicine was successfully weaned off. Life remains normal.

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What is benign childhood epilepsy with centrotemporal spikes (a.k.a. benign rolandic epilepsy)? Benign childhood epilepsy with centrotemporal spikes (BCECTS) is also referred to as benign rolandic epilepsy (BRE). The syndrome is fairly common, and accounts for 15–20 percent of all childhood epilepsies. This type of epilepsy is typically only seen in children, usually between 3 and 13 years of age, and particularly between 5 and 10 years of age (Beaussart 1972). BCECTS is more common in boys than girls. Typically, children with BCECTS are intellectually and otherwise normal. The syndrome is considered idiopathic, meaning that there is no known cause of the epilepsy. However, there is frequently a genetic component. A sibling has about a 15 percent chance of also developing seizures (Heijbel, Blom and Rasmuson 1975).

What types of seizures occur with BCECTS? Half of the time, the seizure occurs when the child is sleeping, although many seizures also occur upon awakening or even at any time (Luders et al. 1987). Therefore, the child is usually in the safety of his or her home when the seizures occur. If it occurs while he or she is awake, the child is usually conscious during the seizure and can recount it. The seizure typically affects one side of the child’s body (i.e. it is a focal seizure). In particular, the child may have abnormal movement or sensation in the mouth, with drooling and trouble talking. He or she may start twitching on one side of the mouth. Sometimes, the twitching may spread to the arms and legs. This could continue into a secondarily generalized tonic-clonic seizure, in which the child will lose consciousness. Seizures are usually not very frequent. Ten percent of the children will have only the first seizure. However, in 20 percent of children with BCECTS, the seizures can be frequent—rarely up to several times per day (Dalla Bernardina, Sgro and Fejerman 2002). How did this type of epilepsy get its name? Benign is part of the name because this type of epilepsy:

• • • •

is almost always outgrown and may not require treatment is not due to any underlying structural brain problem may frequently recur, but would only very, very rarely cause any harm, and is usually not detrimental to the child’s intelligence.

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Rolandic refers to the area of the brain where these seizures typically start: the “rolandic” strip. This area is also known as the “motor strip,” and is located in the central and temporal parts of the brain. This explains why this syndrome has dual names—rolandic and centrotemporal. Epilepsy simply refers to recurrent seizures.

How is BCECTS diagnosed? BCECTS should be suspected when seizures:

• • •

occur in a healthy, preschool or school age child occur typically during sleep or shortly after awakening, and often include a focal component—particularly involving the child’s mouth.

This history usually gives strong clues to the diagnosis. Although most BCECTS seizures are nocturnal, they could happen during daytime hours as well. If there is nausea or vomiting, one should consider the alternate diagnosis of early onset benign childhood occipital epilepsy (COE) (see Chapter 13). Note that nausea, vomiting, headache, and sleepiness can suggest raised pressure in the head. An EEG (electroencephalogram) is done to confirm the diagnosis. The EEG of people with BCECTS shows centrotemporal spikes (increased brain activity in the central and temporal regions of the brain). These spikes are much more prominent during sleep; and in about one third of children, the spikes occur only during sleep (Lombroso 1967). Thus, the EEG should be done with sleep deprivation or sedation, so that a sample of sleep is captured on the EEG. Frequently, the awake EEG will be perfectly normal, and yet the sleeping EEG will be studded with spikes. The MRI would be expected to be normal, but has at times detected important lesions (Shevell et al. 1996).

What is the treatment? Many times, no treatment is needed. According to Engel and Fejerman (2003), drug therapy is only necessary in about 30 percent of cases, and can usually be withheld after just the first seizure. However, if the seizures rapidly become recurrent and/or bothersome, start at a young age, or have a strong focality to them, medication may be needed. This type of epilepsy responds very well to medications. If a child needs to be placed on medication, it is usually wise to wait until the child is seizure-free for about two years before discontinuing it. During this period, the child may have

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outgrown his or her seizures. A recent study has suggested that a one-year treatment period might be sufficient (Braathen et al. 1996). Many antiepileptic medications can be used to treat these types of epilepsy, including oxcarbazepine (Trileptal) and carbamazepine (Tegretol). Although very effective for these types of seizures, medications do have potential negative side effects. The risk/benefit ratio of treatment needs to be considered when deciding whether or not to treat the seizures with medication.

What can we expect for the future in this syndrome? BCECTS usually has an excellent long-prognosis. Seizures usually resolve before the child reaches the age of 16 (Beaussart 1972). Only very rarely would there be a relapse of seizures in adulthood. Prolonged seizures (status epilepticus) are quite uncommon. Cognition has been typically felt to remain normal. However, a condition called “not-so-benign rolandic epilepsy” is also being discussed, which is associated with behavioral, reading, and writing difficulties (Papavasilliou et al. 2005). What are the risks for family members? If one child has BCECTS, then:

• •

15 percent of his/her siblings will also have seizures of BCECTS

•

10 percent will have a history of BCECTS in one of the parents (Heijbel et al. 1975).

another 19 percent will just have the spikes on EEG, but not have actual seizures

Chapter 13

I D I O PAT H I C F O C A L E P I L E P S I E S : C H I L D H O O D O C C I P I TA L EPILEPSIES (COE)

It’s the third time that it has happened, now. My four-year-old daughter, Jill, went to sleep just fine. I heard her vomiting in her bed, and came to find her staring off unresponsively to the side. She seemed pale, was breathing rapidly, and had wet herself. Her doctor though it might just have been a stomach upset, but was suspicious of a seizure. The first EEG was normal, but the second EEG done during sleep showed seizure spikes coming from the back of the brain. Thankfully, the MRI was normal! My neurologist explained that children are rarely hurt by such seizures, and that the spells usually stop after a few years. He also explained that this early onset form of childhood occipital epilepsy is very different from the later onset form, where kids see bright colored circles during the day.

Introduction In this chapter, we will discuss two distinct forms of idiopathic focal epilepsy that are notable for a predominance of seizure spikes coming from the occipital region. (The occipital lobe of the brain is in the back part of the brain, and primarily controls visual functions.) These two varieties of idiopathic childhood occipital epilepsy (COE) are referred to as:

•

early onset benign COE (also called Panayiotopoulos type), which is typically marked by night-time spells of vomiting and staring

•

late onset COE (also called Gastaut type), which is typically marked by daytime spells of seeing the illusion of colored circles.

COE is sometimes referred to as CEOP (childhood epilepsy with occipital paroxysms).

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Benign early onset COE (Panayiotopoulos type) Who gets benign early onset COE? According to Panayiotopoulos (2002, p.95), early onset COE occurs in as many as 2–3 per 1000 of all otherwise typical boys and girls. Most seizures begin in the age range of three to six years old, but can start anywhere between 1 and 14 years of age. This syndrome accounts for as many as 6 percent of cases of childhood epilepsy patients. Even so, Panayiotopoulos points out that most epilepsy textbooks do not even mention the syndrome. Some of the children previously may have had febrile seizures, but usually no one else in the family has a similar seizure disorder. There is probably some overlap of this syndrome with BCECTS (a.k.a. benign rolandic epilepsy). What types of seizures occur with benign early onset COE? Characteristics of the seizures include:

• •

typical occurrence at night (two-thirds of the time)

• •

eyes and head usually turn to one side, or the child stares straight ahead

typical onset with nausea and/or vomiting (which does not occur with BCECTS) other disturbances of the autonomic nervous system, such as:

° ° ° ° °

a change in the child’s skin color (pale, flushed, or blue) a change in respiration, pulse, or temperature a change in pupil size (large or small) loss of urine or stool control increased saliva

•

visual hallucinations, facial spasms, and speech arrest can occasionally occur

•

subsequent altered level of consciousness may occur (confusion or unresponsive)

•

subsequent focal clonic seizure (often of half of the body) or full body clonic seizure activity may follow

•

sometimes, the child may suddenly become limp and unresponsive—either with or without additional symptoms as above

CHILDHOOD OCCIPITAL EPILEPSIES

•

97

partial seizures frequently last for prolonged periods (status epilepticus) but there are no recorded cases of neurological sequelae of such seizures in benign early onset COE (Panayiotopoulos 2002, p.97).

How is early onset benign COE diagnosed? Benign COE is suggested by seizures fitting the above pattern. The presence of nausea/vomiting should help rule out BCECTS. Migraines, syncope, gastroenteritis, and encephalitis can be confused with the syndrome. The EEG is abnormal in 90 percent of cases. Similar to BCECTS, the EEG is most strikingly abnormal when obtained during sleep. The stereotypic epileptic spikes of COE look like those of BCECTS. However, in BCECTS, the spikes occur in the Rolandic area (central and temporal brain regions); whereas in COE, the spikes most often occur in the occipital area and/or other parts of the brain towards the back of the head. In COE, the spikes only occur when the child’s eyes are closed. Note that only half of the children who have occipital spikes on their EEG actually have clinical seizures, and that occipital spikes can be seen in other types of epilepsy other than COE (Brown and Holmes 2004, p.87). By definition, the MRI should be normal in idiopathic cases. However, an MRI should be done to look for structural problems causing seizures that might mimic COE. What is the treatment of early onset COE? A total of 27 percent of patients will have only one seizure; 47 percent will have two to five seizures, and only 5 percent will have more than 10 seizures (Panayiotopoulos 2002, p.102). Panayiotopoulos does not recommend anticonvulsant prophylaxis for a child with one or brief seizures, although many doctors might treat after the second seizure. Carbamazepine (Tegretol) is the most commonly used. Lengthy seizures are a medical emergency; rectal diazepam (Diastat) may play a role in such cases. What can we expect for the future in early onset benign COE? Benign early onset COE is a strikingly benign condition. It would be reportable for a child to be injured by such a seizure. Further, most of the children stop having seizures only one to two years after onset. Even those children who go on to have more persistent seizures will stop having them by age 16.

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Late onset COE (Gastaut type) Who gets late onset COE? The mean age when late onset COE (Gastaut type) starts to occur is eight years old, although they can start anywhere between 3 and 15 years of age. This is a relatively rare form of idiopathic benign focal childhood epilepsy (Panayiotopoulos 2002, p.103). What are the features of the seizures in late onset COE? Seizures in late onset COE are much simpler than in the early onset type. Basically, the seizures consist of visual symptoms. Typically, the child “sees” small circles of multiple colors. Over the course of the seizure—which typically lasts for seconds up to three minutes in duration—these colored circles multiply, become larger, and seem to move in a horizontal manner. Rarely, the visual illusions may be more complex, result in blindness for a few minutes, or be followed by convulsive movements. Sometimes, there are other symptoms related to the visual system, such as movement of the eyeballs, eye fluttering, or eye pain. The child’s mental state remains normal during the visual part of the seizures. In half of the patients with late onset COE, the visual disturbance is followed by a severe, unilateral, throbbing headache. The additional presence of nausea in some patients adds to the confusion of these spells with migraines. However, compared to migraines, the visual disturbances of occipital epilepsy tend to be briefer (typically lasting seconds up to a few minutes), more frequent (daily rather than episodically), more colorful (rather than dull or black and white), more circular (rather than linear), and sometimes associated with eye deviation to the side. How is late onset COE diagnosed? In addition to the above symptoms, the EEG often shows seizure spikes coming typically from the occipital part of the brain. These spikes may sometimes only be found when the EEG is done during sleep, and sometimes are never seen even during repeated EEGs. The MRI should be normal in idiopathic late onset COE, but needs to be performed to rule out structural lesions—since symptomatic occipital seizures can present in the same fashion. What is the treatment and prognosis of late onset COE? Since the seizures of late onset COE tend to be quite frequent (unlike the other benign childhood epilepsies), Panayiotopoulos considers treatment as “probably

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mandatory.” Carbamazepine (Tegretol) has been used most commonly, and is remarkably effective more than 90 percent of the time. More than half of the children will outgrow these seizures within two to four years (Panayiotopoulos 2002, p.105).

Chapter 14

S Y M P T O M AT I C FO C A L EPILEPSIES: THE TEMPORAL LOBE EPILEPSIES Martin L. Kutscher with Zachary Gottlieb

Weeks after his thirteenth birthday, John complained of a twisting feeling in his stomach before he went unconscious. While unconscious, his face jerked. Afterwards, Johnny described that he was afraid as he fell into a dream-like state. Johnny’s first seizures had been as a two-year-old, when he had a few seizures during episodes of a high fever. Since then, he had been a normal, healthy boy. John’s doctor ordered an MRI, which showed a small scar in the middle part of the temporal lobe of John’s brain. The EEG showed spikes originating in the front and middle of John’s temporal lobe. The doctor diagnosed mesial temporal lobe epilepsy, and suggested treatment with Trileptal. John has been seizure free for three years.

Introduction This chapter covers two types of partial epilepsy that originate in the temporal lobe of the brain. 1.

Mesial temporal lobe epilepsy (MTLE), in which the aura is defined by stomach upset and jaw/mouth movements. This is often associated with a scar called “hippocampal sclerosis.”

2.

Lateral temporal lobe epilepsy (LTLE), which is often accompanied by hallucinations, illusions, and body rotation.

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Mesial temporal lobe epilepsy (MTLE) Who gets it? Many people who develop temporal lope epilepsy have previously had febrile seizures as young children. However, there is debate as to whether the febrile seizures themselves actually cause a scar in the brain (that later causes temporal lobe seizures), or whether there was an abnormality there all along—which showed up first as seizures triggered by fever as a young child, and showed up subsequently as temporal lobe seizures. Where does it get its name? The name mesial temporal lobe epilepsy (MTLE) is purely geographical in nature. Mesial means “middle,” and the temporal lobe is the part of the brain (near the ear) where the seizure originates. Hippocampal sclerosis describes scarring in that area of the brain. The hippocampus is a part of the brain that controls emotion and memory storage, and “sclerosis” means a scarring of tissue. Although this damage is often caused by developments in infancy (or before), temporal lobe seizures do not usually appear until late childhood or early adolescence. What are the features of the seizures in MTLE? Characteristic symptoms of the seizures include a prominent aura of fear, panic, or stomach upset/awkward stomach feelings (butterflies, turning). There are typically “automatic movements” such as lip smacking and jaw movements, hand picking/rubbing, eye fluttering, or hyperventilation. Sometimes there are visual illusions. The seizure usually results in altered consciousness, even though it may begin with full awareness of the situation. Occasionally, the whole episode occurs during sleep. The average seizure lasts two to three minutes. Fortunately, only a few patients have prolonged seizures (status epilepticus). Some patients will cough, spit, have language problems, or be confused immediately after the seizure. Since the events begin in one part of the brain, and typically alter the patient’s level of consciousness, they are classified as “partial complex” seizures. How is it diagnosed? MTLE should be considered if there is prominent pre-seizure stomach upset or other aura as described above, especially if there is a previous history of childhood febrile seizures. The EEG will sometimes show no abnormality or small breaks in symmetrical waves. Most patients with this type of epilepsy will have spikes and slow or sharp waves originating from the front part of the temporal lobe.

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An MRI will show “hippocampal sclerosis” (damage to the hippocampus) in 87 percent of patients with temporal lobe epilepsy (Ko and Sahai-Srivastava 2005). This may have been caused by infection, birth complications, vein deformation, or debatably febrile seizures.

What are the differences between lateral (neocortical) temporal lobe epilepsy and mesial temporal lobe epilepsy? The biggest differences between these two similar types of seizures are the aura and the EEG. The aura of lateral temporal lobe epilepsy (LTLE) is marked by more hallucination and often body or head rotation. In addition, facial jerks are often present. In contrast, an upset stomach is suggestive of MTLE. The EEG further differentiates between the two forms of epilepsy: mesial temporal lobe seizures originate in the middle (inside) part of the temporal lobe, while lateral temporal lobe epilepsy originates on the lateral (outer) sides of the lobe.

What are the differences between temporal lobe epilepsy and absence seizures? The differences between these two types of seizures are easy to identify—in theory. While both lead to altered levels of consciousness, the periods before and after the seizure help differentiate the two. An absence spell is a primarily generalized seizure, and consists of an immediate lapse of consciousness without an aura or other warning. It lasts for about 10–30 seconds, and the child quickly snaps back immediately into a conscious state. However, a TLE seizure has a defined aura and lasts for two to three minutes. In addition, a child with TLE usually has post-ictal confusion. During an EEG, someone with absence seizures will show 3-per-second spikes and waves from the whole brain. A patient with TLE will show focal spikes originating in the temporal lobe.

What is the treatment of TLE? Standard anticonvulsant medications are used to treat TLE. Some of the traditional drugs used for such focal epilepsy are carbamazepine (Tegretol), phenytoin (Dilantin), phenobarbital, or even valproate (Depakote). However, some newer drugs can be as effective with better tolerance, including oxcarbazepine (Trileptal), topiramate (Topamax), lamotrigine (Lamictal), levetiracetam (Keppra), or zonisamide (Zonegran).

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Approximately 47–60 percent of TLE patients can control their seizures with the first drug they are prescribed (Kim, Park and Lee 1999). Unfortunately, 40 percent of patients continue to have seizures even after trials of three different antiepileptic drugs (Ko and Sahai-Srivastava 2005).

Are there alternative treatments for TLE? When medications do not produce satisfactory control, the vagal nerve stimulator (VNS) might be used. The size of a hockey puck, the actual device is placed under the skin in the left side of the patient’s chest. It contains a wire that is wrapped around the vagus nerve in the neck. Electric stimulation can help decrease seizure frequency by 25–28 percent (Ko and Sahai-Srivastava 2005). Side effects can be local pain, a hoarse voice, coughing, trouble swallowing, and trouble breathing during intensive exercise. If there is damage on only one side of the hippocampus, surgery may be a way to cure the epilepsy. Of those patients who are chosen for such surgery, 70–80 percent are seizure free two years after surgery (Foldvary, Nashold and Mascha 2000). See Chapter 8, Treatment Options other than Medication.

Chapter 15

S Y M P T O M AT I C FO C A L E P I L E P S I E S : OT H E R T Y P E S

Introduction In the previous chapter, we examined epilepsy syndromes that originated from problems in the temporal lobes of the brain. Here, we will cover the symptomatic focal onset epilepsies that originate from other parts of the brain such as the frontal lobes, parietal lobes, or occipital lobes. These syndromes are presumed to be a symptom of some underlying structural problem—and are thus called symptomatic focal seizures. (The idiopathic focal seizures—seizures that begin in one part of the brain due to as yet unknown reasons—have been covered in Chapter 13, on early and late onset childhood occipital epilepsy and Chapter 12, on benign childhood epilepsy with centrotemporal spikes.)

Frontal lobe epilepsies Second only to the frequency of temporal lobe seizures, frontal lobe epilepsies are the next most common type of symptomatic focal seizures, accounting for about 20–30 percent of patients entered into the registries of potential surgical cases (Foldvary 2001, p.469). The frontal lobes are huge, and control a large variety of brain functions. They are also intimately connected to other lobes of the brain. Thus, there is a very large spectrum of seizure types seen in seizures emanating from the frontal lobes. Seizures can be simple partial, complex partial, tonic-clonic, tonic, atonic, and myoclonic. Frontal lobe seizures often occur in clusters and may be prolonged (“status epilepticus”). Unlike temporal lobe seizures, frontal lobe seizures tend to start quickly, last briefly, and end quickly with little post-ictal state.

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The particular characteristics vary with the exact site of origin of the seizure within the frontal lobe. For further details, see Foldvary (2001) and Brown and Holmes (2004).

•

Motor area seizures begin in one part of the “motor strip”—the part of the brain in the frontal lobe that controls muscle (“motor”) movements on the other side of the body. Thus, motor area seizures cause tonic or clonic movements starting on one side of the body. The seizure may slowly spread along the surface of the brain, causing the seizure to slowly involve additional parts of the body. For example, the seizure may start on one side of the face or arm, and then spread to the leg. This is referred to as a Jacksonian march. As long as this spread has been confined to one hemisphere of the brain, the child may remain alert and later be able to recall that part of the spell. If the seizure then jumps over to also involve the second hemisphere, the child will lose awareness, and the observer will now see the whole body involved. This pattern is referred to as a partial seizure with secondary generalization. Note that sometimes this spread of the seizure occurs so quickly that no one is aware that it actually started in one part of the body. A post-ictal weakness of just one part of the body is common in motor area seizures. If there is involvement of the part of the motor strip that controls movement of the mouth, there may be interference with the production of speech. Sometimes, the seizure of one small part of the brain may be prolonged, causing a lengthy but very localized seizure called epilepsy partialis continua.

•

Supplemental sensory-motor area (SSMA) seizures may begin with an aura of unusual sensation or muscle tightness. Then, there is a brief (lasting 10–40 seconds) episode of an asymmetric body stiffening, which is possibly accompanied by abnormal speech, turning of the head, and facial contortion. Some jerking (“clonic”) movements may follow. The patient typically remains alert throughout the spell, and there are minimal post-ictal changes. The seizures tend to be very consistent from one spell to the other (a feature called being “stereotypical”), occur mainly in sleep, and tend to occur in clusters. They are often difficult to control medically (Browne and Holmes 2004, p.46).

•

Orbitofrontal and cingulate gyrus seizures resemble mesial temporal lobe seizures (see Chapter 14 on temporal lobe epilepsies) because these areas have such close interconnections with the temporal lobes.

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•

Dorsolateral frontal lobe seizures may have a wide variety of auras followed by focal stiffening or jerking activity, along with cessation of activity and possibly speech problems.

•

Anterior frontopolar seizures can have focal and generalized tonic and clonic seizures following falling, forced thinking or loss of contact with the environment, and diversion of head and eyes to the side.

•

Opercular area seizures are not well described in the literature, but seem to involve unusual movements of the mouth.

Parietal lobe epilepsy The parietal lobes are located behind the frontal lobes, and are involved primarily with the processing of sensation (in contrast to the frontal lobes which are involved primarily with muscle movements). Not surprisingly, then, parietal lobe seizures are notable primarily for the presence of abnormal sensations on the opposite side of the body. This may include numbness, pins and needles, pain, burning; and disturbances of smell, vision, or body image. Additional symptoms such as tonic-clonic movements suggest spread to the frontal lobes. Confused and repetitive movements, or strong emotions, suggest spread to the temporal lobes (Foldvary 2001, p.472).

Occipital lobe epilepsy The occipital lobe is primarily involved in the processing of vision. Occipital lobe epilepsy accounts for less than 10 percent of the symptomatic focal epilepsies (Foldvary 2001, p.472). The seizures can include either areas of bright or colored lights, or areas of black visual loss. As in late onset childhood occipital epilepsy, there may be diversion of the head and/or eyes to the side, eye blinking or eye shaking.

What do tests show in the symptomatic focal epilepsies? The EEG findings are highly variable and often misleading. The EEG can show spikes or brainwave slowing in the area as would be expected, but may show spikes in areas not well related to the actual focus of the seizure, or may be normal. Particularly in frontal lobe seizures, the EEG may sometimes be normal even during a seizure! It is important to search for focal lesions such as tumors, vascular malformations, old scars from birth or head trauma, or encephalitis. The MRI will usually do

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a good job at detecting these lesions. However, the very common conditions of mesial temporal sclerosis and other abnormal areas of brain development often remain undetectable by current imaging techniques.

What are the treatments of the symptomatic focal epilepsies? The treatment options of the symptomatic focal epilepsies are discussed in Chapter 7 on medication and Chapter 8 on other treatment options.

What can we expect for the future with symptomatic focal epilepsies? Overall, anticonvulsant treatment produces good control in 60–70 percent of patients with symptomatic focal epilepsy (Annegers, Hauser and Elveback 1979). The children who do best are those whose seizures are brought easily under control and who have a normal EEG and MRI. Risk factors for poor seizure control include finding an identifiable cause, an abnormal MRI or EEG, abnormal neurological status of the child, and difficulty controlling the seizures (i.e. the seizures are frequent or require multiple anticonvulsants). According to Foldvary (2001, p.473), evaluation for possible epilepsy surgery should be considered for patients with identifiable structural findings on MRI, and those whose seizures persist despite one to two years of anticonvulsant trials.

Chapter 16

THE GENERALIZED EPILEPSIES: C H I L D H O O D A N D JU V E N I L E ABSENCE EPILEPSY Martin L. Kutscher with Eric Kutscher

Melanie is a six-year-old girl. Her mother thought that she would “trance out” every once in a while, but did not make anything of it until the babysitter mentioned it, also. Watching more carefully, her mom noticed that even in the middle of an activity, Melanie would freeze, stare straight ahead, blink, stop all speech, and have mild facial jerks. Now that everyone was watching, they could see that Melanie was having dozens of seizures each day, even though Melanie herself did not seem to be aware that she was having them. The family went to a doctor who asked her to hyperventilate while in the office. This provoked a typical staring spell. The doctor recommended an EEG, which showed 3-per-second spike-and-wave discharges, even when she wasn’t appearing to have any seizures. Her seizures stopped after she started taking the anticonvulsant ethosuximide. After two years of good seizure control, her medication was slowly tapered, and she continues to do well.

What is childhood absence epilepsy (CAE)? Childhood absence epilepsy (CAE) used to be called “petit mal epilepsy,” because it is marked by “small” absence seizures rather than “large” tonic-clonic seizures (“grand mal”). However, as we have seen, there are many kinds of “small” seizures—such as absence, myoclonic, atonic, and partial seizures. Thus, the name “petit mal” is not specific enough, and has been abandoned.

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By definition, childhood absence epilepsy is composed almost exclusively of absence seizures. These absence spells are brief (up to 20 seconds) and frequent (about ten or even many more each day). Since they are primarily generalized seizures, there is no aura. During the seizure, the child abruptly becomes frozen and unresponsive. She is unaware that she is having the spell, and is unaware of what is happening around her. The child’s eyes usually stare straight ahead, her eyes may flutter, and her eyes might twitch slightly. Afterwards, the child immediately returns to her previous activity, unaware that anything happened. Some observant children, though, will notice in retrospect that a few seconds seem to have been spliced out of their life. For example, the child may notice that the class is now “suddenly” on a different math problem. “Atypical absence seizures” may vary from this description. Absence seizures can sometimes be confused with partial complex seizures since a trance-like state is prominent in both types. The two types of seizures can be distinguished by the criteria in Table 1.2 in Chapter 1, Overview of Seizures. Absence seizures can also be confused with daydreaming or ADHD (attention deficit hyperactivity disorder). Observation of the features in Table 16.1 should help the physician sort out the two conditions. Sometimes, the distinction can be difficult, even with the aid of an electroencephalogram.

Table 16.1 Distinguishing inattention from seizures Staring off in seizures

Staring off with inattention

Occurs any time, including in the middle of an activity, such as while talking or eating.

Occurs only during “down time,” such as while bored or watching TV.

Touching or loudly calling to the child does NOT end the spell.

The spell stops when the child is called loudly or touched.

There may be associated symptoms such as eye fluttering, lip smacking, or body twitching.

There are no such associated symptoms.

From Kutscher (2005).

Childhood absence epilepsy occurs in children ages two to ten years old, but is most common from ages five to seven years. Most studies show that two-thirds of children with this type of epilepsy are girls (Panayiotopoulos 2002, p.132). When the seizures start at an older age, the syndrome is referred to as “juvenile absence epilepsy” (see below). Of all patients with epilepsy, only 2 percent to 8 percent of patients can be classified as having childhood absence epilepsy. There is a 10

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percent chance that siblings of CAE children will have some of the same symptoms (Berkovic and Benbadis 2001, pp.485–486). Sometimes, children with childhood absence epilepsy will also develop an occasional tonic-clonic seizure. Additionally, 10 percent of patients with childhood absence epilepsy have a previous history of febrile convulsions (Berkovic and Benbadis 2001, p.486). However, when other types of seizures prominently accompany absence seizures, the classification of childhood absence epilepsy should not be used. These kids are typically otherwise neurologically normal.

How is CAE diagnosed? The criteria for diagnosis are:

• •

a healthy two to ten-year-old child with normal neurological development

•

an EEG classically showing 3-per-second spike-wave discharges occurring in a generalized fashion throughout the brain. Seizures are likely to be provoked by hyperventilation during the EEG recording. It may take several minutes of deliberate hyperventilation to bring out a spell

•

an MRI would be expected to be normal.

absence staring spells throughout the day. Although absence seizures can be stimulated by hyperventilation, they do not usually occur during the heavy breathing that results from sports activities

What is the treatment for CAE? Since the seizures of childhood absence epilepsy have usually been recurrent by the time of diagnosis, anticonvulsant treatment is recommended. Most doctors would start to suppress the seizures with either ethosuximide (Zarontin) or valproic acid (Depakene, Depakote). These medications usually control the seizures in more than 80 percent of children (Panayiotopoulos 2002, p.132). Ethosuximide may be arguably safer to use, especially in young children. Note that ethosuximide only treats absence seizures, and does not protect against tonic-clonic seizures. When absence and tonic-clonic seizures are both occurring, then valproic acid alone could treat both types of seizures. If needed, valproate and ethosuximide can be used together. Other medications that may be effective when needed are the benzodiazepines (such as Klonopin), lamotrigine (Lamictal), levetiracetam (Keppra), and topiramate (Topamax). Certain anticonvulsants are

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contraindicated in absence seizures, such as carbamazepine (Tegretol), tiagabine (Gabatril), vigabatrin (Sabril), and phenytoin (Dilantin) (Berkovic and Benbadis 2001, pp.487–489). Most children are treated with anticonvulsants for a seizure-free period of two years, although each case is individualized. A repeat EEG can help guide the decision. We also like to repeat the EEG after the medication is stopped, in order to be sure that we are not missing a return of subtle seizures.

What can we expect for the future of children with CAE? When strictly defined as above, childhood absence epilepsy has an excellent prognosis for remission by age 12, and the absence seizures almost never persist into adulthood. Remission rates range in different studies from 80 percent to 95 percent. Some authors place the risk of an occasional, easily controlled convulsive tonic-clonic seizure at 40 percent (Berkovic and Benbadis 2001, pp.486–487). Other authors write that fewer than 10 percent of patients will develop a single or occasional convulsive seizure later in life (Panayiotopoulos 2002, p.132). The difference probably comes from how the studies define childhood absence epilepsy. Some patients also seem to evolve into the syndrome of juvenile myoclonic epilepsy (see Chapter 17 on juvenile myoclonic epilepsy). Careful education of the child, parent, family, and teachers can help minimize the social adjustment problems that some children experience despite the typically benign neurological outcome.

What is the difference between childhood absence epilepsy and juvenile absence epilepsy? Juvenile absence epilepsy (JAE) has only recently been clearly separated from childhood absence epilepsy. In JAE, onset is typically between 10 and 17 years of age. It may be under-diagnosed if the child is first brought to attention for convulsive seizures, which occur in 80 percent of JAE patients (Berkovic and Benbadis 2001, p.488). The take-home lesson: whenever a patient is being evaluated for one type of seizure, a historical evaluation for other seizure types should be undertaken as well. In addition to later age of onset compared to CAE, the absence seizures of JAE tend to be much less frequent—perhaps just one to three per day. Also, the absence seizures in JAE tend to impair consciousness less completely. Myoclonic seizures may also occur in JAE, but are less prominent than in juvenile myoclonic epilepsy.

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The EEG in juvenile absence epilepsy shows generalized spikes and waves induced by hyperventilation, but is less stereotyped than in childhood absence epilepsy. Valproic acid (Depakote) is a common anticonvulsant of choice in this disorder, which is typically marked by multiple seizure types. Avoidance of alcohol and good sleep habits are also important. The long-term prognosis is not yet clearly delineated.

Chapter 17

THE GENERALIZED EPILEPSIES: J U V E N I L E M YOC L O N I C EPILEPSY (JME) Marissa A. Broadley

When she was 14 years old, Emily had her first generalized tonic-clonic seizure. In the emergency room, she was started on Trileptal. However, after her second tonic-clonic seizure, she was referred to a neurologist who asked her if her arms ever jerked in the morning. Emily said that shortly after waking up, her arms might fly up and she would drop things. Her mom was shocked that Emily had never mentioned these spells—but Emily explained that she had forgotten about them because they had been going on for a while now, and no one ever asked her about them. The neurologist suspected a condition called juvenile myoclonic epilepsy. Her medication was switched to Depakote, and both the morning myoclonic spells and the tonic-clonic spells resolved. She remains on medication and is doing well.

What is juvenile myoclonic epilepsy? The name “juvenile myoclonic epilepsy” (JME) tells us a lot about this syndrome:

• • •

“juvenile” means it is often diagnosed in adolescence “myoclonic” means it is characterized by myoclonic jerks “epilepsy” means recurrent seizures.

Juvenile myoclonic epilepsy is a type of benign idiopathic generalized epilepsy, i.e. there are recurrent seizures of unknown cause that involve the whole brain.

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Who has this type of epilepsy? Approximately one person out of 1000–2000 people has this disorder, which occurs in otherwise normal people. The syndrome usually starts during adolescence, and there is a slight female predominance. JME is a type of epilepsy that is inherited, but the exact pattern of genetic inheritance of JME still remains unclear. JME accounts for about 10 percent of all epilepsies (Serratosa 2001, p.492). However, there are probably more cases of JME that go misdiagnosed or ignored. As always, it is essential that myoclonic seizures be investigated while evaluating absence and/or tonic/clonic seizures. Healthcare professionals should also consider JME when a patient complains of excessive nervousness, twitches or clumsiness. What types of seizures occur with juvenile myoclonic epilepsy? Several types of seizures can occur in JME: myoclonic, generalized tonic-clonic, or occasionally absence. All patients with JME experience myoclonic seizures. Each myoclonic jerk is a seizure. The jerks are bilateral, quick startle-like movements that most often involve the arms and shoulders. If the myoclonic jerk is violent enough, the patient may drop things or fall to the floor. Importantly, there is no observable loss of consciousness during these seizures. Often, the children have become accustomed to these spells and never mention them to their parents! Therefore, whenever any seizure disorder is being considered, it is imperative to ask about myoclonic spells (and absence spells as well). Some patients with JME also experience generalized tonic-clonic and absence seizures. In JME, the tonic-clonic seizures are often preceded by myoclonic jerks. This is often helpful as it acts as a warning to the patient. People with JME most often have seizures upon awakening—either after a night’s sleep or an afternoon nap.

How is juvenile myoclonic epilepsy diagnosed? As always, the child’s history is essential in making the diagnosis. The history might be clearly morning-time myoclonic jerks, or may present as excessive nervousness or clumsiness. An EEG is generally performed to confirm the diagnosis of JME. The EEG typically shows generalized 4–6 Hz polyspikes and polyspike-wave complexes. However, a patient may have JME and have a normal EEG. CT scans and MRI are generally normal.

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What is the treatment? There are several antiepileptic drugs that can be used with JME. They include:

• • • • •

valproate (Depakote, Depakene) clonazepam (Klonopin) and other benzodiazepines topiramate (Topamax) levetiracetam (Keppra) lamotrigine (Lamictal) but it might worsen myoclonic seizures.

Most patients with JME have seizure control on just once anticonvulsant. The typical drug of choice to treat JME is sodium valproate (Depakote). Eighty-five to ninety percent of patients with JME gain seizure control on valproate alone (Renganathan and Delanty 2003, p.79). However, it is unlikely that a patient with JME will ever be able to be seizure-free without medication. Patients with JME should avoid precipitating factors of seizures such as:

• • • •

flickering lights sleep deprivation alcohol/drug use excessive stress.

What can we expect for the future? It is important that people with JME get properly diagnosed because appropriate treatment can lead to a normal and productive life. As above, the seizures can usually be well controlled. However, most patients with JME will continue to require anticonvulsant treatment throughout their lives. This may pose particular problems for women as they approach childbearing years.

Chapter 18

THE GENERALIZED EPILEPSIES: I D I O PAT H I C E P I L E P S Y WITH GENERALIZED T O N I C - C L O N I C S E I Z U R E S O N LY

What is “idiopathic epilepsy with generalized tonic-clonic seizures only?” “Idiopathic epilepsy with generalized tonic-clonic seizures only” is a rather poorly defined entity. The name is longer than our understanding is deep. By definition, the syndrome is distinguished by the occurrence solely of generalized tonic-clonic seizures. Most likely, though, the syndrome represents one end of the spectrum of the idiopathic seizures, wherein the generalized tonic-clonic seizures occur far in excess of the other idiopathic seizures (absence, myoclonic, atonic, etc.), the latter group of which may either not be occurring at all, occurring infrequently, or not being detected. Presumably, this syndrome includes the previous category called “epilepsy with generalized tonic-clonic seizures on awakening,” which is better documented in the literature and forms the basis of the following description. The syndrome starts typically in the teens, but can begin anywhere from young childhood to mid-adulthood. Estimates for the frequency of this condition range from 0.9 percent of idiopathic generalized epilepsies to as high as 15 percent. Some patients have a family history of seizures (Panayiotopoulos 2002, pp.145–146). The seizures typically (but not always) occur within several hours of awakening—regardless of awakening in the morning or from a nap. Additional precipitants include alcohol and other physiological stresses such as poor sleep, change in times of sleep (such as switching work hours), or fatigue.

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How is the syndrome diagnosed? In addition to the typical history as above, the EEG shows generalized spike disturbances in more than half of the patients. The EEG should capture periods of sleep and awakening. Flashing lights from a strobe during the EEG may trigger a response. The MRI would be expected to be normal.

How is the syndrome treated? Appropriate lifestyle choices can help reduce the seizure triggers, such as the avoidance of alcohol and the maintenance of consistent and plentiful sleep. Anticonvulsants targeted at the generalized seizures can help achieve seizure control. These medications would typically start with valproic acid (Depakote). Lamotrigine (Lamictal) is also effective but might exacerbate myoclonic seizures. It would appear that levetiracetam (Keppra) and topiramate (Topamax) should also be effective for a broad spectrum of seizures. Phenytoin (Dilantin) and phenobarbital may help if only tonic-clonic seizures are present. Note that ethosuximide (Zarontin) is only effective for absence seizures. Certain drugs may be contraindicated in this syndrome. These include carbamazepine (Tegretol), which might only be used in this setting as an add-on treatment to first-line drugs. Vigabatrin (Sabril)—which is not approved for use in the US—and tiagabine (Gabitril) can provoke prolonged absence seizures.

What can we expect for the future? Like other idiopathic generalized epilepsies that begin in the teen years, this syndrome will probably persist throughout adulthood. Untreated, the seizures tend to increase as the patient gets older. More than 80 percent of patients will have a recurrence of seizures if their medication is stopped (Janz 1994). With appropriate lifestyle and medication treatment, though, the seizures can usually be brought under control.

Chapter 19

THE GENERALIZED EPILEPSIES: I N FA N T I L E S PA S M S ( W E S T S Y N D RO M E )

Jill was an eight-month-old baby who had been developing normally. A few days ago, Jill’s mom was puzzled by a few unusual startles occurring shortly after Jill awoke in the morning. Mom tried changing Jill’s formula. Yesterday, just after a nap, Jill had a startle for no reason, another startle five seconds later, and two more over the next minute. She looked fine otherwise, but her mother called the pediatrician. He had her come right to the office. Jill’s exam was normal, but the history was worrisome to the doctor. She was referred immediately to a pediatric neurologist, who arranged right away for an EEG and some other tests. The EEG showed a particular pattern called “hypsarrhythmia.” The other tests were normal, but the neurologist hospitalized the baby to begin an aggressive treatment with steroid shots. Several weeks later, the spells had stopped and the EEG had returned to normal. The steroids were slowly weaned, and the family returned to normalcy.

John had also developed normally until he was five months old, when he suffered from an overwhelming case of meningitis that left him severely neurologically impaired. When he turned eight months of age, his mother began to note clusters of spells where he would suddenly thrust himself backwards momentarily. The EEG also showed hypsarrhythmia, and the CT scan showed extensive old brain damage. The steroid shots slowed the seizures, but the EEG never returned to normal. John remained neurologically impaired. When he turned two years old, he developed tonic-clonic seizures as well, which were incompletely controlled with ongoing anticonvulsants.

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What are infantile spasms, and who has them? The term “infantile spasms” refers to an epilepsy syndrome seen in infants typically 4–24 months of age, who develop myoclonic seizures along with a very abnormal EEG pattern called hypsarrhythmia. This form of epilepsy was first described by Dr. W.J. West as far back as 1841, and is sometimes referred to as “West syndrome.” Half of the children with infantile spasms have seizure onset between four and six months of age, and 90 percent of the children will have onset before 12 months of age (Kellaway et al. 1979). Infantile spasms are rare in the first few months of life. Sixty percent of the children with infantile spasms are boys. Unless a specific genetic cause is found, only rarely would multiple family members have the condition. The incidence of infantile spasms is about 3–5/10,000 births (Panayiotopoulos 2002, p.57). The spasms are usually the first sign of the condition, but may be preceded by several weeks of neurological regression, such as the infant’s losing interest in his or her surroundings. Alternately, a neurological regression may begin after the seizures start. Each individual jerk is a seizure. The seizure jerks typically occur in clusters over a several-minute period. They are commonly associated with awakening, or occur while the baby is about to fall asleep. They occur much less commonly during actual sleep. Several such clusters usually occur each day. The seizure itself can have several forms:

•

A myoclonic startle which can be distinguished from a typical baby startle as per Table 19.1. The startles are very quick.

•

A forward flexion of the muscles that is known as a “flexor spasm.” These spasms are slightly more prolonged than the myoclonic startles. They start with a quick flexion movement followed by a few seconds of stiffening.

•

A backward extension of the muscles that is known as an “extensor spasm.”

•

A loss of muscle tone momentarily, as if the strings on a marionette were briefly cut, referred to as an “atonic” seizure.

•

A prolonged stiffening of the extremities that can last a few seconds.

Table 19.1 will help differentiate the seizures of infantile spasms from normal “Moro” startle responses. Any startle response that is becoming more prominent as the baby gets older is grounds for suspicion. The distinction of infantile spasms from other events can be difficult and should be promptly addressed by a qualified doctor.

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Table 19.1 Distinguishing a normal baby “Moro” startle response from a myoclonic seizure Normal “Moro” startle response What is the age of the infant at Maximal frequency at birth, onset? and then get less frequent

Myoclonic seizure Rare at birth, and then get more frequent

Stop by 4–5 months of age

Begin from several months of age to 2 years

Are they induced by stimulus such as loud noise, fright, or being lowered backwards?

Yes

Not typically

Are they repetitive?

No. One Moro per stimulus

Yes. Tend to occur in clusters over several minutes

What causes infantile spasms? Children with infantile spasms fall into one of two groups: cryptogenic (due to a hidden cause), or symptomatic (due to some readily identifiable underlying neurological condition).

Cryptogenic group Like Jill (in the case study above), some of the children have been developing normally and have normal examinations and medical evaluations. Such children are considered to fall into the “cryptogenic” or “idiopathic” group—which simply means that the reason for the seizures in these otherwise typical children is currently “hidden” or unknown. Symptomatic or “remote” group Other children, like John (above), have the seizures as a result of some clear neurological impairment, and are considered to fall into the “symptomatic” group. In this group, the seizures are a symptom of some other ongoing neurological problem. There are a multitude of neurological conditions that might cause symptomatic infantile spasms. These conditions can occur prenatally, around the time of birth, or during infancy. A sampling of these conditions include:

•

any severe neurological “insult” to the baby’s brain, such as severe birth difficulties, meningitis, encephalitis, shock, near drowning, or cardiac arrest

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• •

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congenital malformations of the brain, including Aicardi syndrome neurocutaneous disorders (disorders of the skin and nervous system). These include:

° tuberous sclerosis (a syndrome of dark and light skin spots, developmental delay, seizures, and growths in the brain) ° neurofibromatosis (a syndrome of coffee-colored “café-au-lait” spots on the skin, and growths throughout the nervous system) ° Sturge–Weber syndrome (a syndrome of port-wine colored birthmarks around the eye and abnormal vessels in the brain) ° incontinentia pigmenti (a syndrome of skin blisters which leave streaks of skin discoloration).

•

metabolic disorders such as abnormalities with amino acids (e.g. PKU), organic acids, or mitochondrial function

• •

chromosomal disorders such as Down’s syndrome degenerative neurological disorders.

What conditions can be confused with infantile spasms? There is a long list of conditions that can be confused with infantile spasms. These include:

• • •

normal baby startles (see discussion above)

• •

high muscle tone of the infant (“spasticity”) causing arching

•

Benign Myoclonus of Early Infancy, marked by myoclonic jerks where the EEG remains normal during and in between the jerks. The myoclonus is not related to entering or coming out of sleep, and the child remains neurologically normal

• •

other kinds of seizures

colic or abdominal pain stiffening and turning the head to the side due to vomiting/gastro-esophageal reflux (“heartburn”) which is referred to as Sandifer’s syndrome Benign Neonatal Sleep Myoclonus (just brief startles only during early sleep with a normal EEG)

myoclonus that is a secondary effect of certain tumors.

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How are infantile spasms diagnosed? Infantile spasms are first suspected by the history of spasms as described above. Any suspicion of such activity should be discussed immediately with your doctor. After examining the infant, the next step is typically an EEG. The typical EEG finding of infantile spasms type of epilepsy is called “hypsarrhythmia.” There are three basic criteria for diagnosing hypsarrhythmia on the EEG: 1.

Seizure spikes are multifocal, i.e. they come from varying, multiple parts of the brain.

2.

After a burst of spikes, the brainwaves temporarily flatten out—a pattern called “burst-suppression.”

3.

There is chaotic disorganization of the typical background brain activity.

As we’ll see below, it appears important that the evaluation for possible infantile spasms be done quickly—for long-term prognostic reasons. Sometimes, though, the EEG findings may lag behind the seizures that are seen by the caregivers. Thus, if an initial EEG (done shortly after the spells start) is normal, then a repeat EEG may need to be done a bit later. Your neurologist will decide with you upon an appropriate medical work-up. Besides careful examination of the skin and an EEG, a typical work-up might include a CT scan (better for detecting the calcifications of tuberous sclerosis); MRI scan (usually more sensitive than a CT scan for other problems); routine blood studies; studies for amino acids, organic acids, and other metabolic abnormalities; chromosomes; ophthalmology examination; viral titers; and possibly lumbar puncture (“spinal tap”). Sometimes, a PET scan can pick up particularly active foci of seizures.

What is the treatment? The best treatment of infantile spasms is still being actively debated. In a survey of United States doctors (comprised primarily of child neurologists), 88 percent reported the use of a steroid injection called ACTH (adrenocorticotropin hormone) as the leading therapy. In a Japanese survey, the vitamin pyridoxine, valproic acid, and ACTH were used in that order. In a survey of pediatric neurologists in the UK, vigabatrin was the initial treatment used (Mackay et al. 2004). The American Academy of Neurology, in conjunction with the Child Neurology Society, reviewed the literature of 159 articles. Trying to apply strict scientific standards, they produced a practice parameter with the following conclusions:

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Short-term effectiveness of treatments

•

ACTH steroid injections are “probably effective” for the short-term treatment of infantile spasms and for improving the hypsarrhythmia on the EEG.

° Most of the patients who responded did so within two weeks of treatment. ° No good data could provide the optimal dose or length of treatment. ° ACTH steroid injections were more effective than oral steroids. In fact, oral steroids could not be clearly shown to help.

•

Vigabatrin is “possibly effective” for the short-term treatment of infantile spasms, including “possibly effective” for those children with tuberous sclerosis.

° “Serious concerns” about retinal toxicity raise the need for careful ophthalmologic screening in children, but precise recommendations based on the literature could not be made. Vigabatrin has not been approved by the FDA for use in the United States.

•

Other therapies had either insufficient or inadequate data to demonstrate effectiveness in the treatment of infantile spasms. These unproven treatments include:

° ° ° ° ° °

valproic acid nitrazepam pyridoxine zonisamide topiramate novel therapies such as the ketogenic diet or IVIG (intravenous immune globulin) ° combinations of these unproven treatments (Mackay et al. 2004).

Long-term effectiveness of treatments Given the conflicting and insufficient nature of the data, the practice parameter states that:

•

No recommendations can be made on the use of steroids, vigabatrin, or pyridoxine regarding their effect on the long-term outcome of the child’s neurological development of long-term seizures.

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•

The parameter committee is unable to conclude that early treatment affects the long-term outlook for children with infantile spasms (Mackay et al. 2004).

Since there are some studies that do show a significant improvement in the long-term developmental outlook with early treatment—especially for those children in the cryptogenic group (Lombroso 1983; Matsumoto et al. 1981; Riikonen 1982)—many doctors (including myself) try to start treatment of infantile spasms as soon as possible, i.e. ideally well within a month of their onset. Discuss all such issues with your doctors.

Side effects of ACTH treatment ACTH is a hormone made by the brain’s pituitary gland that signals the adrenal glands to make their own steroid hormones. Theories abound, but it is still not clear why giving ACTH seems to work better than giving oral steroids. To most US doctors, at least, ACTH injections are the best hope for a child with infantile spasms—particularly those in the cryptogenic group. The ACTH injections, though, come with their own set of problems. Some of these include:

• • •

the child has to receive daily intramuscular injections for weeks or longer the parents (or a visiting nurse) must give daily intramuscular injections the child typically may be hospitalized at the start of therapy to watch for side effects and to train the parents.

In addition, there may be:

•

hypertension, which may be severe and require anti-hypertensive medications

• • • • • • • • •

irritability weight gain and ravenous appetite brain shrinkage (temporary) heart muscle hypertrophy electrolyte disturbances in the blood that are monitored with blood tests stomach bleeding that is monitored with testing the stool for blood raised blood sugar that is monitored by blood and urine tests reduced resistance to infection occasional deaths, most often from overwhelming infection.

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What can we expect for the future in this syndrome? As we can see above, the data regarding effectiveness of long-term treatment for infantile spasms is insufficient. Thus, the prognosis for these children is difficult to predict. That being understood, we can at least state that:

•

In the symptomatic group, the overwhelming proportion of children will remain neurologically delayed—often, quite significantly so. This makes sense. No matter how you treat the seizures, the underlying causative brain damage will still remain. Even if the seizures themselves do come under control, many of the children will have a relapse. Almost a third of these children will go on to develop another type of epilepsy called Lennox–Gastaut syndrome, which is discussed in the next chapter.

•

In the cryptogenic group, the prognosis is much, much better. According to some studies, about 40 percent of such infants remain intellectually normal at long-term follow-up (Matsumoto et al. 1981; Riikonen 1982). As above, it is possible that particularly in this subgroup of cryptogenic patients, a delay in initiation of treatment may be associated with worse outcome.

Chapter 20

THE GENERALIZED EPILEPSIES: L E N N OX – G A S TAUT S Y N D RO M E

Let’s pick up John’s story from Chapter 19 on infantile spasms. Remember that John had been developing normally until a severe case of meningitis at five months of age left him very neurologically impaired. At eight months, he was diagnosed as having infantile spasms on the basis of his extensor spasms of the body and an EEG showing hypsarrhythmia. ACTH steroid shots brought the spasms under control, but John remained very neurologically delayed. At age two, he had just started to walk, but said no words. Around that time, he started having “drop attacks,” where he would suddenly lose all muscle tone and collapse momentarily. No one knew what to make of these falls at first. Maybe, he was just losing his balance? Then, John had a full blown tonic-clonic seizure. It was time for another EEG. His neurologist said that the EEG now showed a “slow spike and wave” pattern. Putting all of the pieces together, his doctor diagnosed a new epilepsy syndrome called “Lennox–Gastaut.” One anticonvulsant was tried, but it only controlled the tonic-clonic seizures. Then John started having some staring “absence” spells. A broad-spectrum anticonvulsant was tried with significant improvement. Through the years, though, his medications have been frequently readjusted—often in combination. Although John still gets an occasional seizure, his parents, therapists, and special education teachers have become more comfortable when they do occur.

What is “Lennox–Gastaut” syndrome, and who has it? Lennox–Gastaut syndrome is an epilepsy syndrome marked by a triad:

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1.

A mixture of seizure types, including tonic, absence, and/or atonic seizures.

2.

Mental retardation or regression in the child.

3.

An EEG showing a “slow spike and wave” pattern.

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Children usually develop the syndrome between two and eight years of age, much more often in boys than in girls. Neurologically impaired children usually develop the syndrome at the earlier part of this age range. About one-third of the children with infantile spasms later develop Lennox–Gastaut (Gastaut et al. 1966). The syndrome occurs in about 0.3 per 1000 children (Brown and Holmes 2004, p.91). Although relatively rare, the persistence of the syndrome makes Lennox–Gastaut represent a significant proportion of children with ongoing epilepsy. Let’s look at each of the features of the triad comprising Lennox–Gastaut.

The types of seizures in Lennox–Gastaut The seizures of Lennox–Gastaut tend to be quite repetitive, and can be of several types:

•

Tonic seizures are among the most prominent. These stiffening seizures tend to be relatively brief, and last just about ten seconds on average. However, they can often be shorter, or commonly last up to one minute, or last more than 20 minutes (“status epilepticus”). Often, they occur during sleep, and may even go unnoticed. During the day, the seizures may throw the child off balance and cause a fall. The child may stare and open his or her eyes widely during the spell. Sometimes, the spells are triggered by a stimulus such as a sound or movement.

•

Atypical absence seizures occur frequently in Lennox–Gastaut as well. These “atypical” absence staring seizures are not as well defined in their onset/offset as typical absence spells. They may last much longer than the usual ten seconds of typical absence, and may be followed by some confusion after the spell. During an atypical absence, the child might still be able to function in a sort of dazed, fugue-like state for even prolonged periods of time. There are commonly some quick muscle twitches or abnormal muscle posturing during the atypical absences. Prolonged video-EEG studies show that parents often underestimate the frequency of atypical absence seizures.

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•

Atonic seizures (also known as “drop attacks”) may occur as well. These are instantaneous, momentary losses of muscle tone. They may affect just the child’s head, which falls forward on the neck. Dangerous falls can occur when they affect the entire body. Atonic seizures can be difficult to distinguish clinically from myoclonic seizures.

•

Other seizure types include tonic-clonic, partial seizures, and occasionally myoclonic seizures.

The neurological impairment seen in Lennox–Gastaut Similar to the situation with infantile spasms, children with Lennox–Gastaut can be categorized into two groups: “cryptogenic” (the child has no known previous neurological impairment or disorder) or “symptomatic” (the child already has a neurological disorder). Although some 20–40 percent of children with Lennox–Gastaut come from the cryptogenic group (Glauser and Morita 2002), neurological problems almost always develop as the syndrome progresses. The mental deterioration may show itself as apathy or excessive insistence on continuing an activity, slowing of visual motor skills, or memory problems. They are more likely to be severe when the child comes from the symptomatic group, has a younger age of onset (before 12–24 months of age), has a previous history of infantile spasms, and has more frequent seizures (Glauser and Morita 2002). In the younger children, there may be severe slowing or regression of intellectual progress. Older children may have less pronounced developmental problems. In addition, there may often be psychiatric problems. Younger children may show hyperactivity, aggression, unstable moods, or social behavior problems. Older children may even develop psychosis (Dulac and Engel 2003). EEG findings in Lennox–Gastaut The typical EEG findings in Lennox–Gastaut include:

• •

a disorganized and abnormally slow background of brainwaves a “slow spike and wave” pattern. “Spike and wave” refers to a quick, sharp needle-like spike in the EEG followed immediately by a long dome shaped wave. “Slow” refers to the repetition of these spike/wave complexes occurring at a rate of only 1.5 to 2.5 cycles/second (vs. the typical rate of 3 cycles/second seen in classic absence seizures). These spikes become more frequent during the sleeping parts of the EEG

LENNOX–GASTAUT SYNDROME

•

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other EEG findings can include spike and waves at 3 cycles/second, polyspikes (a rapid succession of needle-like spike waves), or polyspikes and waves.

What causes Lennox–Gastaut syndrome? Although between 20 and 40 percent of children with Lennox–Gastaut fall into the cryptogenic group, the majority of patients fall into the symptomatic group. The possible etiologies are similar to those for infantile spasms. Some of them include:

•

prenatal (before birth), perinatal (around the time of birth), or postnatal brain injury, such as severe lack of oxygen or brain infection

•

congenital malformations of the brain, where the brain cells do not correctly migrate to the correct places in the brain

•

neurocutaneous disorders, which are the disorders of the skin and nervous system mentioned in the chapter on infantile spasms

•

metabolic disorders, which are deficiencies in the ability to enzymatically process chemicals in the body; such as abnormalities with the metabolism of amino acids, organic acids, or mitochondrial function

• •

chromosomal disorders degenerative neurological disorders.

How is Lennox–Gastaut syndrome evaluated medically? The medical work-up may typically include:

•

a careful history, physical, and neurological examination, including looking for abnormal skin findings

•

an EEG, preferably during both the asleep and awake states, showing the findings above

•

a prolonged video EEG might be considered, to help identify seizures that are being missed clinically by caregivers. The video EEG may also be useful to let caregivers identify what unusual behaviors are—or are not—actual seizures

•

an MRI scan

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•

a CT scan might also be done to look for brain calcifications that can be missed on the MRI

•

PET or SPECT scans may be indicated if epilepsy surgery is being considered

•

other work-up might include chromosomes and metabolic studies (including amino and organic acids).

What is the treatment? The medical treatment of Lennox–Gastaut is difficult and often frustrating. Many children’s seizures manage to continually escape control. Brown and Holmes (2004) and Dulac and Engel (2003) offer the following comments about treatment. (Read more about the individual anticonvulsants in Chapter 7, on medications.)

•

valproic acid (Depakote) has the advantage of treating multiple types of seizures with one medication. Clinical experience has often been quite positive, although the medication may lose its effectiveness over time. Behaviorally, the medication is usually well tolerated, but the risk of bone marrow and/or liver damage exists especially in young children who are neurologically impaired and taking multiple anticonvulsants.

•

topiramate (Topamax) treats a broad spectrum of seizure types, but can be associated with cognitive slowing, kidney stones, heat and metabolic disturbances. It rarely controls the seizures of Lennox–Gastaut completely by itself.

•

lamotrigine (Lamictal) is another broad spectrum anticonvulsant, which, again, rarely controls all Lennox–Gastaut seizures as the only therapy. Severe skin reactions are more common in children less than 16 years old, and especially if they are also taking valproic acid (which significantly raises the lamotrigine levels). In the US, Lamictal is indicated for use in combination with other medications for the generalized seizures of Lennox–Gastaut syndrome in patients two years of age or older.

•

benzodiazepines such as clonazepam (Klonopin) also cover a large spectrum of seizures, and are medically quite safe when taken in appropriate doses. However, they are frequently sedating. Patients often build up a tolerance to these medications, requiring frequent dosage changes.

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•

phenytoin (Dilantin) is good for the tonic seizures, but not the absence or atonic spells.

•

felbamate (Felbatol) is effective for a large number of seizure types, but is only rarely used now (as a last resort) because of a high incidence of bone marrow and liver failure.

•

Overall, one drug is unlikely to completely control all seizure types. Unfortunately, even in combination, the anticonvulsants are still not likely to provide complete control.

•

The ketogenic diet is discussed in Chapter 8, on other treatments. Use of the ketogenic diet in Lennox–Gastaut patients seems to follow a “one-third” rule:

° One-third (to one-half) of patients have an excellent response (marked or complete seizure control). ° One-third of patients have a partial/incomplete response. ° One-third of patients have no response.

What can we expect for the future in this syndrome? Unfortunately, children with Lennox–Gastaut rarely do well from either an intellectual or a seizure control perspective (Brown and Holmes 2004; Dulac and Engel 2003). Even children from the cryptogenic group rarely have a preserved development. Intellectual status tends to deteriorate as the patient gets older. Only a few children go into seizure remission. Although the absence, atonic, and myoclonic seizures may improve as the child gets older, the tonic seizures tend to worsen, and partial seizures may start to occur.

Chapter 21

S E I Z U R E S N O T RE QU I R I N G T H E D I AG N O S I S OF E P I L E P S Y: FEBRILE SEIZURES

Billy was eight months old when he had his first febrile seizure. He had a stuffy nose for two days, but was otherwise okay until his mother heard a strange, raspy, breathing sound coming from the crib. She found Billy having a generalized, tonic-clonic seizure. His mom was sure that Billy was going to die. Thankfully, after a minute, the seizure stopped. Only then did anyone realize that the baby felt really hot. By the time the ambulance arrived, Billy was starting to cry. In the emergency room, he was still fussy, and his temperature was 104 degrees Fahrenheit (40 degrees Celsius). The doctor felt that he needed a spinal tap, which fortunately turned out to be normal. Billy had another spell when he was 15 months old, but has otherwise done well.

What are febrile seizures, and who has them? Febrile seizures are seizures that are triggered by a child’s rapid rise in fever. Febrile seizures are the most common form of childhood seizures, occurring in 3–4 percent of all children. That is a lot of children. Features of a “typical febrile seizure” include:

• •

relatively brief (90 percent of febrile seizures are less than 15 minutes long)

• •

occur only once per 24-hour period

generalized (85–95 percent of febrile seizures involve both sides of the body equally) usually occur within 24 hours of onset of high fever—typically greater than 39 degrees Centigrade (102.2 degrees Fahrenheit)

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•

the child’s average age for the onset of seizures is 24 months. Febrile seizures are rare for children under six months old or over five to six years old

•

there is no history of afebrile seizures in the child.

By definition, a “complex febrile seizure” has one or more of the following features:

• • •

lasts more than 15 minutes has a focal onset in one part of the brain, or occurs more than once within a 24-hour period.

Note that the term “febrile seizures” should ordinarily not be used in children who have afebrile seizures as well. In children who have afebrile seizures, the fever is likely to be the trigger—not the sole cause—of that seizure. This is because physiological stress (such as illness and/or fever) is a common precipitant for people who already have epilepsy. When the seizure occurs during a fever, but does not meet the criteria above, it is probably best to refer to the spell as a “seizure with fever,” rather than as a “febrile seizure.” This reminds us that we do not really have a particular diagnosis yet.

How are febrile seizures diagnosed? Febrile seizures are diagnosed by a history of seizures caused by fever as described above. Initial evaluation of the child with a seizure with fever usually begins in the emergency room. Simultaneously, the child needs to be evaluated for the cause of the fever and the cause of the seizure. Essentially by definition, any EEG, CT/MRI, or blood studies that are done should be normal.

History, physical, and neurological exam Obviously, the evaluation begins with a history and physical. Based on those findings, the doctors will evaluate the need for any of the following additional studies. Lumbar puncture (“spinal tap”) When a child presents with a seizure and fever, it is essential to consider meningitis or encephalitis as possible causes. This may require a procedure called a lumbar puncture. By the time you are reading this, such an individualized decision has likely already been made.

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Blood tests The American Academy of Pediatrics (AAP 1996) suggests that routine blood tests are not indicated for simple febrile seizures unless required for evaluation of the fever itself, or something else in the particular patient’s history and physical exam. Practically speaking, blood tests are commonly performed. From a single blood sample, a series of blood tests can be done. These may include:

•

a complete blood count (CBC), looking in particular for infection or anemia

•

a chemistry panel, including electrolyte salts, calcium, and glucose levels (particularly if there has been vomiting or diarrhea)

•

a blood culture, which is a sample of blood drawn sterilely that is observed over several days for the growth of bacteria.

Other tests to determine the cause of fever, such as a chest X-ray, may also be ordered if needed.

EEG An EEG is usually not recommended in a neurologically healthy child with a first typical, simple febrile seizure. (AAP 1996). The decision needs to be individualized, but an EEG should be strongly considered if:

• • •

the seizures with fever are complex or otherwise atypical the child has an abnormal developmental history or exam, or there are multiple febrile seizures.

CT scan A CT scan is not usually performed for children with typical febrile seizures. Complex febrile seizures, and atypical seizures with fever may likely require a CT scan. This would often be done when the child is taken to the emergency room. MRI scan Again, this is not usually performed for children with typical, simple febrile seizures. Just like an EEG, the decision to obtain an MRI needs to be individualized, but strongly considered if:

• • •

the seizures with fever are complex or otherwise atypical the child has an abnormal developmental history or exam, or there are multiple febrile seizures.

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My child has had a febrile seizure. What can I expect for the future? In order to be prepared, and in order to help make rational decisions regarding any possible treatments, you will need answers to the following questions:

Is my child likely to be damaged by a febrile seizure? Dr. John Freeman, from the Department of Pediatric Neurology at Johns Hopkins Medical School, wrote: “There is no increased risk of dying, no increased risk of injury, no increased risk of brain damage and no evidence of an increased risk of learning disorders” from one or even many febrile seizures (Freeman 1992). A study of the long-term intellectual and behavioral outcome of children with simple or complex febrile seizures had no epidemiological difference when compared with children without febrile seizures (Verity et al. 1998). Although some controversy exists, there is even a very low risk of damage even for children with prolonged idiopathic febrile seizures (i.e. not due to some other cause such as meningitis) (Maytal et al. 1989). Personally, I feel that it is hard for doctors to absolutely guarantee much of anything. Let’s just say that it is really unlikely for a child to be hurt from a febrile seizure. What are the chances that my child will have more febrile seizures? Overall, a child has a one in three chance of having additional febrile seizures. The earlier the age of the first febrile seizure, then the more likely that there will be recurrences. However, as we saw above, even if the febrile seizures do recur, they would be very unlikely to cause any harm. Specifically, children with:

•

onset of febrile seizures before one year of age have a 50 percent chance of recurrence

•

onset of febrile seizures from one to three years of age have a 25 percent chance of recurrence

•

onset of febrile seizures over five years of age have a 10 percent chance of recurrence (Nelson et al. 1978).

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Besides age of onset, Berg (1996) identified additional factors that can help predict the recurrence of febrile seizures:

• • • •

low fever, less than 102 degrees Fahrenheit brief duration of fever (less than one hour) preceding the seizure age of child less than 18 months family history of febrile seizures.

With none of the above risk factors (low risk), only 14 percent of children will have a recurrence. With three or four risk factors (high risk), 64 percent of children will have a recurrence of febrile seizures.

What are the chances my child will develop recurrent afebrile seizures (epilepsy)? It turns out that the overwhelming majority of children with febrile seizures DO NOT develop epilepsy (recurrent afebrile seizures). Table 21.1 summarizes data from a study of some 2000 children with febrile seizures (Nelson et al. 1978).

Table 21.1 Relative risks of developing epilepsy for a child with febrile seizures History of the child

Risk of later developing epilepsy

Never had a febrile seizures (control)

0.5%

Had febrile seizures (1 or more) with no risk factors

0.9%

Had febrile seizures with one risk factor below:

• abnormal neurological status • complex febrile seizures • family history of afebrile seizures

2.3%

Had febrile seizures with two risk factors above

9.6%

1.4% 3.6%

Note that these are excellent odds! Even if a child has had multiple typical febrile seizures, only 0.9 percent of such children will develop epilepsy; i.e. the child has a greater than 99 percent chance of NOT developing epilepsy. This risk is not higher—practically speaking—than for control children who never had febrile seizures (who have a 0.5 percent risk of developing epilepsy). There has been controversy—still unsettled—as to whether or not febrile seizures can sometimes lead to a small scar in the temporal lobes called mesial

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temporal sclerosis. Knudsen et al. (1996) found that none of 300 children with febrile seizures followed for 12 years developed temporal lobe epilepsy due to hippocampal sclerosis. Kuzniecky et al. (1996), however, reports that 57 percent of their patients referred for temporal lobectomy had a childhood history of “complex febrile seizures.” It is not clear whether the febrile seizures caused the scar, or rather, were the first sign of it.

What are our treatment options? There are really two questions to be asked: (1) What are the treatments against more febrile seizures? and (2) What are the treatments against possible future afebrile seizures (epilepsy)? Let’s answer these questions individually.

What are the treatments against more febrile seizures? Certain common-sense precautions can be taken, such as watching the child closely during times of illness and/or fever. Although it is commonly suggested to try to keep the fever down with antipyretics, evidence actually showing the usefulness of this advice is lacking. Appropriate use of fever medications should be discussed with your child’s doctor. There are two common treatment options for the actual seizures: rectal diazepam given at the time of seizures, and daily preventative use of phenobarbital. Even though many children will have a recurrence of their febrile seizures, keep in mind that they are unlikely to be harmed by them. Rectal diazepam can be useful to abort the occasional occurrence of a prolonged seizure. Diazepam is the generic name for Valium. A rectal gel form of diazepam in pre-measured, plastic-tipped plungers is marketed in the US as Diastat. Typically, if a seizure lasts more than several minutes, then the caregiver delivers one of the plastic Diastat plungers into the child’s rectum. No needle is involved! This is typically quite effective in preventing the seizure from becoming prolonged. The most common side effect of rectal diazepam is sedation (which can be a source of confusion when evaluating the child with fever). The risk of respiratory suppression from Diastat seems to be minimized by the rectal route. Many families are comforted by the presence of a medication in the house that could be used to abort a febrile seizure—even though they will most likely never need it. Other families want nothing to do with such medicine. Daily oral phenobarbital does reduce the risk of having more febrile seizures while a child is taking it. The question is, “Is it worth it?” For most kids/families, the answer is “No.” The side effects of phenobarbital include frequent hyperactivity/irritability in the child, occasional sleepiness, unlikely allergic reactions, and the

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(at least, theoretical) possibility of long-term issues in the child’s developing brain. Since a child is unlikely to be hurt from a febrile seizure, then it is not usually felt to be worth the side effects of phenobarbital. On occasion, you and your doctor may decide to use daily phenobarbital. Indications for such use might include particularly severe febrile seizures, particularly frequent febrile seizures, or extreme parental anxiety. The other medication that works to prevent febrile seizures is valproic acid (Depakene). However, the use of valproic acid in children of this age has significantly more potential side effects than phenobarbital, and is rarely recommended for febrile seizures. Note that attempting to start oral phenobarbital at the first sign of a fever or seizure is NOT effective—it takes much too long for adequate doses to get into the child’s bloodstream.

What treatments make sense to help prevent future epilepsy? The chances that a child with febrile seizures will later develop epilepsy are really quite low (see Table 21.1). Thus, it usually does not make sense to treat large numbers of children for years with phenobarbital just so that we can be preventatively treating seizures that might develop later in very small minority.

So, what is the prognosis for children with febrile seizures? The short answer: the prognosis for children with febrile seizures is great! Recapping the above information:

• • • • •

Febrile seizures are very common. Febrile seizures are very unlikely to hurt the child. Febrile seizures may recur, but resolve by five to six years of age. Febrile seizures are quite unlikely to be a harbinger of epilepsy. Most children do not require any specific treatment, although rectal diazepam might be kept available.

Considering that you recently thought that your child might be dying, the above facts are pretty good!

Chapter 22

T H E HI S T O RY O F E P I L E P S Y: A TIMELINE Eric Kutscher

2000 BCE

Babylonian textbook of medicine is written. It contains information about epilepsy and the different types of seizures we know of today. The textbook says that epilepsy is a spiritual disorder that requires spiritual treatment.

400 BCE

Hippocrates writes the first book on epilepsy saying people with epilepsy are not possessed but simply have a brain disorder. He suggests a physical treatment rather than a spiritual one. His ideas are disputed, and for the next 2000 years, seizures are largely considered a supernatural disorder—causing widespread prejudice. Nevertheless, successful people with epilepsy apparently included Julius Caesar, Peter the Great, Pope Pius IX, and Fedor Dostoevsky.

1494

In a book on witch-hunting, Malleus Malefucarum reports that seizures are a sign of witchcraft.

1773

George Washington’s daughter, Patsy, dies from epilepsy.

1857

The first drug recognized to treat epilepsy, bromide, is introduced.

1857

A hospital in London is created for people with epilepsy and those who are paralyzed.

1859

The modern era of epilepsy begins. Epilepsy is no longer considered a spiritual problem, but now a neurological disorder. This is due to three scientists (Jackson, Reynolds, and Gowers) recognizing that epilepsy is a disorder of brain tissue. They also notice that seizures can alter consciousness and other functions.

1873

Hughlings Jackson proposes a theory that seizures are due to electrochemical discharges in the brain. He goes further and explains that different types of seizures may be due to the location in the brain where they begin.

1874

David Ferrier (London), Gustav Fritsch (Germany), and Eduard Hitzig (Germany) discover that electricity can stimulate the brain.

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1904

William Spralting becomes the first North American doctor specializing in epilepsy, called an “epileptologist.”

1909

The International League Against Epilepsy (ILAE) is formed. It includes professionals from 60 countries.

1912

Phenobarbital, an antiepilepsy drug, is created and is still used.

1920

The ketogenic diet is formally discovered. It is based on the ancient observation that epilepsy may improve when patients severely reduce the amount of carbohydrates they consume.

1929

Hans Berger invents the electroencephalogram (EEG) in Germany. This machine allows doctors to track the electrical current of seizures.

1938

Phenytoin (Dilantin) is discovered. It is still used today.

1953

Carbamazepine (Tegretol) is manufactured. It is still considered a first choice to treat patients with partial and tonic-clonic seizures.

1958

Ethosuximide (Zarontin) is introduced, and remains a drug of choice for absence seizures.

1961

The International Bureau for Epilepsy is formed for non-professional members.

1963

Sodium valproate (Depakote) is accidentally discovered when it was used as a solvent for other chemicals being tested as anticonvulsants.

1968

The Epilepsy Foundation of America is formed.

1970

The US Veterans Administration establishes epilepsy centers.

1990

Americans with Disabilities Act of 1990 is passed. This act prohibits any discrimination against the rights of people with epilepsy in the US to marry and have children.

1990s

After an almost 30-year hiatus, a flurry of new anticonvulsants are approved in the US: Felbatol, Lamictal, Topamax, Gabitril, Keppra, and Trileptal.

1997

The International Bureau for Epilepsy and the International League Against Epilepsy work with the World Health Organization to form the Global Campaign Against Epilepsy.

2000

The conference “Curing Epilepsy: The Promise and the Challenge” is held. It is considered a milestone to reflect how far the concept of epilepsy has come.

2001

Seventy percent of the fifty million people in the world with epilepsy still do not have appropriate treatment, either because epilepsy is not considered a medical disorder in the area, or because of lack of availability of medical resources.

Note: Information taken from World Health Organization (2001) and www.epilepsy.com/epilepsy/history.html (accessed 2005).

REFERENCES

AAP (1996) “Practice Parameter: The neurodiagnostic evaluation of the child with a first simple febrile seizure.” Pediatrics 97, 5, 769–775. Annegers, J.F., Hauser, W.A. and Elveback, L.R. (1979) “Remission of seizures and relapse in patients with epilepsy.” Epilepsia 20, 729–737. Beaussart, M. (1972) “Benign epilepsy of children with rolandic (centrotemporal) paroxysmal foci.” Epilepsia 13, 795–811. Berg, A.T. (1996) “Complex febrile seizures.” Epilepsia 37, 2, 126–133. Berg, A.T., Shinnar, S., Levy, S.R. et al. (2001) “Early development of intractable epilepsy in children: a prospective study.” Neurology 56, 1445–1452. Berkovic, S.F. and Benbadis, S. (2001) “Childhood and juvenile absence epilepsies.” In E. Wyllie (ed) The Treatment of Epilepsy: Principles and Practice (3rd edition). Philadelphia, PA: Lippincott Williams and Wilkins. Braathen, G., Andersson, T., Gylje, H. et al. (1996) “Comparison between one and three years of treatment in uncomplicated childhood epilepsy: a prospective study. I. Outcome in different seizure types.” Epilepsia 37, 9, 822–832. Brown, T.R. and Holmes, G.L. (2004) Handbook of Epilepsy (3rd edition). Philadelphia, PA: Lippincott Williams and Wilkins. Camfield, C.S., Camfield, P.R., and Vengelers, P.J. (2002) “Death in children with epilepsy: a population-based study.” Lancet 315, 1891–1895. Dalla Bernadina, B., Sgro, V. and Fejerman, N. (2002) “Epilepsy with centrotemporal spikes and related syndromes.” In J. Roger, M. Bureau, Ch. Dravet et al. (eds) Epileptic Syndromes in Infancy, Childhood and Adolescence (3rd edition). Eastleigh, UK: John Libbey, pp.81–202. Devinsky, O. (2002) Epilepsy: Patient and Family Guide (2nd edition). Philadelphia, PA: F.A. Davis Company. Dulac, O. and Engel, J. (2003) “Lennox–Gastaut Syndrome” at www.epilepsy.org. Engel, J. and Fejerman, N. (2003) “Benign epilepsy of childhood with centrotemporal spikes.” www.epilepsy.org. Epilepsy Foundation of America (2005) www.epilepsyfoundation.org. Foldvary, N. (2001) “Symptomatic focal epilepsies.” In E. Wyllie (ed) The Treatment of Epilepsy: Principles and Practice (3rd edition). Philadelphia, PA: Lippincott Williams and Wilkins, pp.467–474. Foldvary, N., Nashold, B. and Mascha, E. (2000) “Seizure outcome after temporal lobectomy for temporal lobe epilepsy: a Kaplan–Meier survival analysis.” Neurology 54, 3, 630–634. Freeman, J. (1992) “Decision making and the child with febrile seizures.” PREP 13, 8.

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Freeman, J.M., Vining, E.P. and Pillas, D.J. (2002) Seizures and Epilepsy in Childood: A Guide for Parents (3rd edition). Baltimore, MD: Johns Hopkins University Press. French, J., Kanner, A., Bautista, J. et al. (2004) “Efficacy and tolerability of the new antiepileptic drugs I: Treatment of new onset epilepsy.” Neurology 62, 8, 1252–1260. French, J.A., Williamson, P.D., Thadani, V.M. et al. (1989) “Characteristics of medial temporal lobe epilepsy, I: Results of history and physical examination.” Annals of Neurology 25, 82–87. Gastaut, H., Roger, R., Soulayrol, R. et al. (1966) “Childhood epileptic encephalopathy with diffuse slow spike-waves (otherwise known as ‘Petit mal variant’) or Lennox syndrome.” Epilepsia 7, 2, 139–179. Glauser, T.A. and Morita, D.A. (2002) www.emedicine.com/NEURO/topic186.htm. Heijbel, J., Blom, S. and Rasmuson, M. (1975) “Benign epilepsy of childhood with centrotemporal EEG foci: a genetic study.” Epilepsia 16, 285–293. Hirtz, D., Berg, A., Bettis, D. et al. (2003) “Practice parameter: Treatment of the child with a first unprovoked seizure. Report of the Quality Standards Subcommittee of the American Academy of Neurology and the Practice Committee of the Child Neurology Society.” Neurology 60, 166–175. Janz, D. (1994) “Pitfalls in the diagnosis of grand mal on awakening.” In P. Wolf (ed) Epileptic Seizures and Syndromes. London: John Libbey and Company, pp.213–220. Kellaway, P., Hrachovy, R.A., Frost, J.D. Jr, and Zion, T. (1979) “Precise characterization and quantification of infantile spasms.” Annals of Neurology 6, 214–218. Kim, W.J., Park, S.C. and Lee, S.J. (1999) “The prognosis for control of seizures with medications in patients with MRI evidence for mesial temporal sclerosis.” Epilepsia 40, 3, 290–293. Knudsen, F.U., Paerregaard, A., Andersen, R., and Andresen, J. (1996) “Long term outcome of prophylaxis for febrile convulsions.” Archives of Disease of Childhood 74, 13–18. Ko, D.Y. and Sahai-Srivastava, S. (2005). “Temporal lobe epilepsy.” www.emedicine.com/NEURO/topic365.htm. Kosoff, E.H. (2005) “Nonpharmacologic treatment options for intractable epilepsy.” Profiles in Seizure Management 4, 1, 12–16. Kutscher, M.L. (2005) Kids in the Syndrome Mix of ADHD, LD, Asperger’s, Tourette’s, Bipolar, and More! London: Jessica Kingsley Publishers. Kuzneicky, R., Burgard, S., Belir, S. et al. (1996) “Qualitative MRI segmentation in mesial temporal sclerosis: clinical correlations.” Epilepsia 37, 5, 433–439. Lancman, M.E., Asconape, J., Brotherton, T. et al. (1995) “Juvenile Myoclonic Epilepsy: an underdiagnosed syndrome.” Journal of Epilepsy 8, 3, 215–218. Lombroso, C.T. (1967) “Sylvian seizures and midtemporal spike foci in children.” Archives of Neurology 17, 52–59. Lombroso, C.T. (1983) “A prospective study of infantile spasms: clinical and therapeutic correlations.” Epilepsia 24, 135–158. Luders, H., Lesser, R.P., Dinner, D.S. and Morris, H.H. III. (1987) “Benign focal epilepsy of childhood.” In H. Luders and R.P. Lesser (eds) Epilepsy: Electroclinical Syndrome. London: Springer-Verlag, pp.303–346.

REFERENCES

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Mackay, M.T., Weiss, S.K., Adams-Webber, T. et al. (2004) “Practice parameter: medical treatment of infantile spasms.” Report of the American Academy of Neurology and the Child Neurology Society. Neurology 62, 10, 1668–1681. Matsumoto, A., Watanabe, K., Negoro, T. et al. (1981) “Long-term prognosis after infantile spasms: a statistical study of prognostic factors in 200 cases.” Developmental Medicine and Child Neurology 23, 1, 51–65. Maytal, J., Shinnar, S., Moshe, S. et al. (1989) “Low morbidity and mortality of status epilepticus in children.” Pediatrics 83, 3, 323–331. Nelson, K. and Ellenberg, J.H. (1978) “Prognosis in children with febrile seizures.” Pediatrics 61, 5, 720–726. Panayiotopoulos, C.P. (2002) (ed) A Clinical Guide to Epileptic Syndromes and their Treatment. Oxfordshire, UK: Bladon Medical Publishing. Papavasiliou, A., Mattheou, D., Bazigou, H. et al. (2005) “Written language skills in children with benign childhood epilepsy with centrotemporal spikes.” Epilepsy Behavior 6, 1, 50–58. Pellock, J.M., Morton, L.D. and Watemberg, N. (1998) “New antiepileptic drug therapy for children.” The Neurologist (Suppl) 4, 5, s16–s22. Renganathan, R. and Delanty, N. (2003) “Juvenile Myoclonic Epilepsy: under-appreciated and under-diagnosed.” Postgraduate Medical Journal 79, 78–80. Riikonen, R. (1982) “A long-term follow-up study of 214 children with the syndrome of infantile spasms.” Neuropediatrics 13, 1, 14–23. Schachter, S. (2001) “Tiagabine.” In E. Wyllie (ed) The Treatment of Epilepsy: Principles and Practice (3rd edition). Philadelphia, PA: Lippincott Williams and Wilkins, pp.947–952. Serratosa, J.M. (2001) “Juvenile myoclonic epilepsy.” In E. Wyllie (ed). The Treatment of Epilepsy Principles and Practice (3rd edition). New York: Lippincott Williams and Wilkins, pp.491–507. Shevell, M.I., Rosenblatt, B., Watters, G.V. et al. (1996) “Pseudo-BECRS: intracranial focal lesions suggestive of a primary partial epilepsy syndrome.” Pediatric Neurology 14, 1, 31–35. Verity, C.M., Greenwood, R., and Golding, J. (1998) “Long-term intellectual and behavioral outcomes of children with febrile convulsions.” New England Journal of Medicine 338, 723–728. World Health Organization (2001) Fact sheet number 168, Revised February.

F U RTH E R R E A D I N G 1

Books Books intended for families Freeman, J., Vining, E. and Pillas, D. (2002) Seizures and Epilepsy in Childhood: A Guide for Parents (3rd edition). Baltimore, MD: The Johns Hopkins University Press. An excellent guide for parents of children with seizures. Devinsky, O. (2002) Epilepsy: Patient and Family Guide (2nd edition). Philadelphia, PA: F.A. Davis Company. Another excellent guide for families about adults and children with seizures.

Books intended for doctors Brown, T.R. and Holmes, G.L. (2004) Handbook of Epilepsy. Philadelphia, PA: Lippincott Williams and Wilkins. A brief yet truly useful guide intended for physicians, which gives more specific medical details. Wyllie, E. (ed) (2001) The Treatment of Epilepsy: Principles and Practice (3rd edition). Philadelphia, PA: Lippincott Williams and Wilkins. This is the 1285-page heavy-duty textbook for neurologists with a special interest in seizures.

Internet sources General epilepsy sites around the world www.epilepsyfoundation.org is a great starting place for information about seizures from a leading epilepsy support organization in the US.

1

•

Their “Answer Place” provides information about a large range of topics, such as legal rights and the issue of the military and seizures.

•

You can search about US driving laws in each state, currently at www.epilepsyfoundation.org/answerplace/Social/driving/statedrivinglaws.cfm.

Disclaimer: Views expressed in the books and Internet sites listed here do not necessarily represent the views of the author.

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FURTHER READING

145

www.ilae-epilepsy.org is the website of the International League Against Epilepsy. See detailed information on the:

• •

proposed new classification of seizures, and about individual types of seizures and the epilepsy syndromes. Both topics are currently found at www.ilae-epilepsy.org/Visitors/Centre/ctf/index.cfm, or click on the “Resources” section and then the “ILAE classification” heading.

www.epilepsy.com This is another great place to look up information. See their “Resources” section, which includes important information in the “Research Articles” section. www.PediatricNeurology.com This is the author’s website. The site covers all aspects of child neurology, including information on childhood seizures. www.ncbi.nlm.nih.gov/entrez/query.fcgi (or just enter “PubMed” into a search engine) is the PubMed entrance to the entire medical journal database. This is the database used by physicians. www.emedicine.com/NEURO/topic594.htm has information about neurological disease and driving. See also the Epilepsy Foundation of America website (above) for driving information. www.epilepsyontario.org is an excellent Canadian epilepsy site. Find video clips of different seizure types by searching the site for “video clips”. www.epilepsy.ca is another excellent Canadian epilepsy site. www.epilepsy.org.uk is a wonderful UK site. www.epilepsynse.org.uk is a very family friendly UK site, which has excellent information including detailed drug information. www.epilepsy.ie is based in Ireland. www.epilepsy.org.nz covers epilepsy in New Zealand. www.epilepsy.org.za covers epilepsy in South Africa.

Information about anticonvulsants www.drugs.com is a fantastic site that provides:

• • • •

information about each medication a drug-interaction checker images of the medications along with their strengths a pill identifier feature (look up the code on the pill).

www.epilepsy.com has excellent anticonvulsant information including sections for beginner, intermediate, and advanced levels of knowledge about pharmacology. (From the homepage, click on the section “Treatment” and then “Seizure Medicines.”) www.epilepsynse.org.uk has detailed anticonvulsant information. www.helpingpatients.org and www.RxHope.com have been set up by US pharmaceutical companies and support groups to help qualifying patients without prescription coverage get the medications that they need. www.ilae-epilepsy.org/Visitors/Chapters/AEDs/index.cfm offers a database to match generic names with corresponding brand names in different countries.

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Information on other treatment modalities www.epilepsy.com/epilepsy/vns.html has information about treatment with the vagal nerve stimulator. www.charliefoundation.org provides information about the ketogenic diet. www.familyvillage.wisc.edu/general/ketogeni.htm maintains a list of additional sites on the ketogenic diet.

A B O U T TH E AUT H O R S

Martin L. Kutscher MD received his B.A. from Columbia University and his M.D. from Columbia University’s College of Physicians and Surgeons in New York. He completed a pediatric internship and residency at Temple University’s St Christopher’s Hospital for Children. His neurology residency and pediatric neurology fellowship were completed at the Albert Einstein College of Medicine. He is board certified in Pediatrics and in Neurology, with Special Competency in Child Neurology. Dr. Kutscher is currently a member of the Departments of Pediatrics and of Neurology at the New York Medical College; and is a partner of Pediatric Neurological Associates, LLP based in White Plains, New York. Dr. Kutscher has more than 20 years of experience diagnosing and treating families affected by childhood seizures. His other books include Kids in the Syndrome Mix of ADHD, LD, Asperger’s, Tourette’s, Bipolar, and More!: The One Stop Guide For Parents, Teachers, and Other Professionals and The ADHD BOOK: Living Right Now! Gregory L. Holmes MD is Chair of the Section of Neurology at Dartmouth Medical School, after having served as the Director of the Center for Research in Pediatric Epilepsy at Children’s Hospital, Harvard Medical School, in Boston. Dr. Holmes has a long-standing interest in childhood epilepsy and is currently president of the American Epilepsy Society. Marissa A. Broadley MPH received her Masters in Public Health from New York Medical College, and has a special interest in epilepsy. Zachary Gottlieb and Eric Kutscher are students in Westchester County, New York. We gratefully acknowledge Dr. Ronald Jacobson for reviewing this manuscript and for his guidance through the years.

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BCECTS (benign childhood epilepsy with centrotemporal spikes) 91–4 benign early onset COE 96–7 benign rolandic epilepsy (BRE) 39, 91–4 Page numbers in italics refer to benzodiazepines 48, 50, 60–1, tables. 130 biking, risks of 32 birth control pills, effects of absence seizures 18, 20, 22, anticonvulsants 55, 62, 102, 109 78–9 ACTH (adrenocorticotropin blood tests 40, 51–2, 134 hormone) bone marrow problems 51, 55, side effects of 122–3 60, 65, 130, 131 treatment for infantile brain scans 36–7 spasms 124–5 ADHD (attention deficit hyper- brain surgery 68–9 breath holding spells 24, 27 activity disorder) 109 afebrile seizures 133, 136, 138 breathing failure following seizure akinetic seizures see atonic 31 seizures problems during seizures alcohol, dangers of 82 28 allergic reactions 51, 57, 58, 62, protecting airway during 64, 65 seizures 30 American Academy of Neurology 42, 122 antibiotics, interaction with CAE see childhood absence anticonvulsants 54 epilepsy anticonvulsants 56–65 calcium 55 behavioral effects 50 carbamazepine 48, 50, 56–7 choice of 47–50 Carbatrol see carbamazepine deciding on treatment with careers 80–1 42, 44 CAT scans see CT (Computermonitoring effects of ized Tomography) scans 51–2 Child Neurology Society 42, and pregnancy 78–80 122 stopping 45–6 childhood absence epilepsy titrating dosage of 52–3 (CAE) 18, 108–11 usage principles 53–5 childhood occipital epilepsies vitamin supplements 55 (COE) 95–9 Ativan see lorazepam children, advice for 70–5 Atkins diet, modified version classification 68 epilepsy syndromes 88–90 atonic seizures 21, 128 seizure types 20, 87 atypical absence seizures 127 clinical symptoms, medication auras 17–18, 19, 27, 71–2, 101, 51 102 clonazepam 50, 61

SUBJECT INDEX

148

“clonic” seizures 21 COE see childhood occipital epilepsies college 81–2 complex febrile seizures 133 complex partial seizures 22, 47–9, 101 cranial nerves, functions of 35 CT (Computerized Tomography) scans 36–7, 134 Depakene see valproic acid Depakote see divalproex sodium diagnosis 34–41 BCECTS 93 childhood absence epilepsy (CAE) 110 early onset COE 97 febrile seizures 133–4 infantile spasms 122 juvenile myoclonic epilepsy (JME) 114 late onset COE 98 Lennox–Gastaut syndrome 129 MTLE 101–2 Diastat see rectal diazepam diazepam 23–4, 60–1, 137 Dilantin see phenytoin diphenylhydantoin see phenytoin divalproex sodium 49, 59 driving 76–8 drop attacks see atonic seizures drug levels, monitoring 52 drugs, recreational 82 EEG (electroencephalogram) for children with BCECTS 93 febrile seizures 134 information for children 72–3 measurements 37–9 types of 39–40 emotional aspects, stopping medication 45–6 employment 80–1

INDEX

epilepsy classification of syndromes 88–90 definition 18 historical timeline 139–40 risks of developing 136–7 surgery 68–9, 103 epilepsy partialis continua 105 ethosuximide 48, 50, 110 eye examinations 35 fainting spells 24, 28 febrile seizures 101 felbamate 65, 130 Felbatol see felbamate flashing lights 27, 33, 74, 117 focal seizures see partial seizures folic acid 55, 79 gabapentin 48, 49, 63 Gabitril see tiagabine Gastaut type epilepsy 98–9 generalized epilepsies 89, 90 generalized seizures 19–21 anticonvulsants for 48, 49–5 link to generalized epilepsies 90 generic medications 53 genetic issues 80, 92, 94, 114 “grand mal” seizures see tonic-clonic seizures half-life of drugs 54 headaches 24, 28, 93, 98 hereditary issues 80, 94, 114 hippocampal sclerosis 100, 101, 102, 137 “homeopathic” treatments 69 hypsarrhythmia 118, 119, 122, 123 infantile spasms 118–25 injuries, protection against 21, 30, 33 International League Against Epilepsy (ILAE) 88, 140 Internet sources 144–6

“Jacksonian march” 22, 105 JAE see juvenile absence epilepsy JME see juvenile myoclonic epilepsy juvenile absence epilepsy (JAE) 111–12 juvenile myoclonic epilepsy (JME) 113–15 Keppra see levetiracetam ketogenic diet 66–7, 130 kidney stones, risk of developing 52, 63, 65, 67 Klonopin see clonazepam laboratory testing, medication 51–2 Lamictal see lamotrigine lamotrigine 48, 49, 50, 64, 130 late onset COE 98–9 lateral temporal lobe epilepsy (LTLE) 100, 102 Lennox–Gastaut syndrome 126–31 levetiracetam 48, 49, 50, 62 liver dysfunction 51, 60 liver function tests (LFTs) 52 local seizures see partial seizures lorazepam 50, 61 LTLE see lateral temporal lobe epilepsy lumbar punctures 41, 133 medical assistance, when to call for 31 medication anticonvulsants 47–65 compliance with 83 decisions on treatment with 42–6 mesial temporal lobe epilepsy (MTLE) 100–2 migraines 24, 98 “Moro” startle responses 119–20 motor area seizures 105

149

MRI (Magnetic Resonance Imaging) scans 36–7, 73, 134–5 MTLE see mesial temporal lobe epilepsy myoclonic seizures 21, 72, 114, 119–20 Mysoline see primidone neurological examinations 34–6 Neurontin see gabapentin nocturnal seizures 28, 93, 105 not-so-benign rolandic epilepsy 94 observations to make during a seizure 26–9 ophthalmoscopic examinations 35, 123 osteoporosis 58 oxcarbazepine 48, 49, 50, 62 Panayiotopoulos type epilepsy 96–7 pancreatitis 60 partial complex seizures 22, 27, 47–9, 101 partial epilepsies 89, 90, 100–7 partial seizures 19, 21–3, 88 anticonvulsants for 47–9 classification 20, 87 link to partial epilepsies 90 see also idiopathic focal epilepsies; symptomatic focal epilepsies PET (Positron Emission Tomography) scans 37 “petit mal” epilepsy see childhood absence epilepsy “petit mal” seizures see absence seizures phenobarbital 49, 50, 52, 58, 59, 137–8 phenytoin 48, 49, 50, 58, 130 physical examinations 34–6 polycystic ovarian syndrome 60

150

practice parameter, treatment of seizures 42–4, 123–4 pregnancy 54–5, 78–80 primidone 59 prolonged seizures 23–4, 42, 44, 104, 137 pseudo-seizures 25 psychomotor seizures see complex partial seizures

CHILDREN WITH SEIZURES

parietal lobe 106 temporal lobe 100–3

Valium see diazepam valproate 49, 64 valproic acid 48, 49, 54, 55, 59, 130 teenage issues 76–83 video games 73–4 Tegretol see carbamazepine temporal lobe epilepsies (TLEs) vigabatrin 48, 65, 123 vitamin supplements 55 100–3 VNS see vagal nerve stimulator temporal lobe seizures see complex partial seizures tiagabine 48, 49, 50, 65 West syndrome 118–25 rectal diazepam 23–4, 61, 97, titration 52–3 137 TLEs see temporal lobe Zarontin see ethosuximide recurrence of seizures 43 epilepsies Zonegran see zonisamide reflexes 35 “tonic” seizures 21, 127 zonisamide 48, 50, 6 relapse, risk of 45 tonic-clonic seizures 21, restriction of activities 32 116–17 Topamax see topiramate topiramate 48, 49, 50, 63, 130 Sabril see vigabatrin treatment 42–6 safety precautions 21, 30, 31, BCECTS 93–4 32–3 childhood absence epilepsy sedation, minimizing 54 (CAE) 110–11 seizures early onset COE 97 definition of 17–18 febrile seizures 137–8 diagnosis 34–41 generalized seizures 117 events that mimic 24–5 idiopathic epilepsy with explaining to children generalized tonic-clonic 70–5 seizures only 117 observation of 26–9 infantile spasms 122–5 types of 19–24, 87 juvenile myoclonic epilepsy what to do 30–1 (JME) 114–15 simple partial seizures 22, 47–9 Lennox–Gastaut syndrome skin rashes 51, 58, 62, 64, 65 130–1 sleep, importance of 80, 81 temporal lobe epilepsy smoking 82 (TLE) 102–3 SPECT (Single Photon treatment types Emission Computed “homeopathic” 69 Tomography) scans 37 ketogenic diet 66–7 “spinal tap” 41, 133–4 medication 47–65 sports 33 surgery 68–9 status epilepticus 23–4, 44 vagal nerve stimulator Stevens–Johnson syndrome 51, (VNS) 68 62, 64 Trileptal see oxcarbazepine supplemental sensory-motor area (SSMA) seizures 105 swimming 33, 82 urine tests 52, 63 symptomatic focal epilepsies frontal lobe 104–6 vagal nerve stimulator (VNS) occipital lobe 106 68, 103

AUTH O R INDEX

Hirtz, D. 24, 42, 43, 44 Holmes, G.L. 49, 97, 105, 127, 130, 131

Watemberg, N. 49 Janz, D. 117

AAP (American Academy of Pediatrics) 134 Annegers, J.F. 107 Beaussart, M. 92, 94 Benbadis, S. 110, 111 Berg, A.T. 43, 136 Berkovic, S.F. 110, 111 Blom, S. 92 Braathen, G. 94 Brown, T.R. 49, 97, 105, 127, 131 Camfield, C.S. 43 Dalla Bernadina, B. 92 Delanty, N. 115 Devinsky, O. 11, 25, 50, 54, 69, 76, 82 Dulac, O. 128, 130, 131 Elveback, L.R. 107 Engel, J. 93, 128, 130, 131 Epilepsy Foundation of America 11, 30, 70, 74, 76 Fejerman, N. 93 Foldvary, N. 103, 104, 105, 106, 107 Freeman, J. 33, 43, 45, 69, 77, 79, 80, 135 French, J. 49, 56 Gastaut, H. 127 Glauser, T.A. 128 Hauser, W.A. 107 Heijbel, J. 92, 94

Verity, C.M. 135 Vining, E.P. 33

Kellaway, P. 119 Kim, W.J. 102 Knudsen, F.U. 137 Ko, D.Y. 102, 103 Kosoff, E.H. 67, 68 Kutscher, M.L. 109 Lee, S.J. 102 Lombroso, C.T. 93, 124 Luders, H. 92 Mackay, M.T. 122, 123, 124 Mascha, E. 103 Matsumoto, A. 124, 125 Maytal, J. 135 Morita, D.A. 128 Morton, L.D. 49 Nashold, B. 103 Nelson, K. 135, 136 Panayiotopoulos, C.P. 49, 56, 96, 97, 98–9, 109, 110, 111, 116, 119 Papavasiliou, A. 94 Park, S.C. 102 Pellock, J.M. 49 Pillas, D.J. 33 Rasmuson, M. 92 Renganathan, R. 115 Riikonen, R. 124, 125 Sahai-Srivastava, S. 102, 103 Schachter, S. 65 Serratosa, J.M. 114 Shevell, M.I. 93

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