Children With Seizures

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 9
INTRODUCTION 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 87
12. Idiopathic Focal Epilepsies: Benign Childhood
Epilepsy with Centrotemporal Spikes (BCECTS) 91
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
Juvenile Myoclonic Epilepsy (JME) 113
Marissa A. Broadley
Idiopathic Epilepsy with Generalized Tonic-clonic
Seizures Only 116
Infantile Spasms (West Syndrome) 118
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 emo-
tionally 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
9
10 CHILDREN WITH SEIZURES
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 comprehen-
sively 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-physi-
cians 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 dif-
ficult 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 differ-
ent 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 knowledge-
able 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 reassur-
ing, 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.
11
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, psy-
chologists, nurses, speech therapists, physical therapists, and occupational thera-
pists. 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 Reso-
nance 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 syn-
dromes 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. None-
theless, 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, communi-
cating 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 neurotrans-
mitter 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 move-
ments 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 some-
thing 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
17
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 sleepi-
ness 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 clas-
sification 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
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 • Focal seizures (that start initially in part of
throughout the whole brain) one hemisphere)
° Absence seizures ° Simple partial seizures (consciousness
Tonic-clonic seizures remains normal during the seizure)
°
° Myoclonic seizures ° Complex partial seizures (consciousness
is altered during the seizure)
° Atonic seizures
° 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 gen-
eralized 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.
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.
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 con-
sciousness. There is usually a well-defined aura followed by a confused trance. Typ-
ically, 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 Absent or mild Prominent

fluttering
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.
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 knowl-
edge 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 neurolo-
gists 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. Never-
theless, 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
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 con-
cluded 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 recur-
rence 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.
• 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.
• 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.
• 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.
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, electroencepha-
logram, 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 descrip-
tion.
So, Table 2.1 shows the helpful observations that you can make.
26
WHAT TO OBSERVE DURING A SEIZURE 27
Table 2.1 Observations during a seizure
Feature of the spell Importance of that feature
Was there an aura? An aura is A well-defined aura identifies the seizure as being of focal onset.
the prodrome or “warning” to (Note that even though a seizure has no identifiable aura, it may
the seizure. still be of focal onset.)
How did the seizure start? Did A localized onset to the seizure makes the seizure likely to have
the seizure start in just one started in one specific part of the brain. (Note that the focal
part of the body and then onset may spread so quickly to the rest of the brain—and thus,
spread, or did it involve the body—that even though a seizure has no observed focal start, it
whole body equally from the may still be of focal onset.)
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 These symptoms might suggest a cardiac problem.

(feelings of a rapid heart beat)
or a feeling of
light-headedness?
Were there: These markers help classify the seizure. They are often
• smacking or licking of the symptoms of a partial complex seizure, but can also sometimes
lips be seen less prominently in an absence seizure.
• eyelid fluttering
• picking or fumbling hand
movements?
Was the person able to In a moment of simple inattention such as boredom, the person
respond to any outside will respond to a stimulus such as being called loudly or having
stimulus? 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”) Rhythmic clonic movements are strong markers for a seizure
and/or later harsh jerking event, although a few jerks can follow other conditions such as
(“clonic”) movements? syncope.
Was the jaw clenched or was A clenched jaw is a strong marker for a seizure event, as is
the tongue bitten? tongue biting—although the latter can also occur with any kind
of fall.
Continued on next page

Table 2.1 cont.
Feature of the spell Importance of that feature
Was there incontinence of Incontinence is typically considered a marker for a seizure, but
urine or bowels? can occur with fainting spells as well.
Was there any color change or These symptoms indicate cardiovascular/respiratory aspects of
breathing problem? the spell—either as a cause or an effect of the event.
How long did the actual When these events happen, they seem to last for an eternity. Try
seizure last? 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 Whenever one type of seizure is found, a search for other
suggestive of other types of seizure types should be undertaken. Have there been spells of
seizures? 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 The seizures of some epilepsies occur typically during sleep.
nocturnal seizures? 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.
WHAT TO OBSERVE DURING A SEIZURE 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!
30
WHAT TO DO DURING A SEIZURE 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 repercus-
sions 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 fol-
lowing, 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.)
32
ACCIDENT PRECAUTIONS 33
• 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
34
MEDICAL EVALUATION OF SEIZURES 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
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 pref-
erably, with an MRI scan (Magnetic Resonance Imaging). The advantages and dis-
advantages 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 Usually requires sedation for children less than
children five years of age
Can detect most emergency masses, but can Typically more accurate at detecting lesions
miss small 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. When used, MRI contrast dye is usually very
The contrast dye used for CT scans has some safe
occasional risks
Poor ability to image the temporal lobes (which Visualizes the entire brain well
are a frequent source of seizures) and the
bottom parts of the brain
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 (“Single-
Photon 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 mea-
suring 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 stimu-
late—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 partic-
ularly 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.
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.
• 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
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 signifi-
cantly 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.
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 ques-
tions 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 reason-
able 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? 43
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 typi-
cally 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.
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? 45
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 discontinua-
tion (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
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 medi-
cation 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 informa-
tion 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, includ-
ing safety, side effects, and efficacy for the type of epilepsy being treated. As dem-
onstrated 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
Seizure Type Older Anticonvulsants (often used first because of extensive experience) New Anticonvulsants
carbamazepine valproic ethosuximide benzodiazepines gabapentin lamotrigine topiramate tiagabine levetiracetam zonisamide vigabatrin
(Tegretol) acid (Zarontin) (Klonopin) (Neurontin) (Lamictal) (Topamax) (Gabitril) (Keppra) (Zonegran) (Sabril)
oxcarbazepine* (Depakote) (Ativan) (not approved
(Trileptal) (Depakane) (Tranxene) in USA)
phenytoin
(Dilantin)
phenobarbital
Simple partial
Partial
Complex partial üüüü üüü üü üüü üüü üüü üüü üüüü üüü üüü
Tonic/clonic üüüü üüüü üü üüü üüü üüü Avoid in üüü üüü
primarily
Not generalized, Avoid, see
see below below
approved
Generalized
Absence phenytoin üüüü üüüü üüüü üüü üü May cause üüü May worsen
carbamazepine absence
(and ?oxcarb) status
may worsen
Minor motor carbamazepine üüüü üüüü May worsen üü üüü May worsen
(myoclonic and (and ?oxcarb)
atonic) may worsen
*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

TREATMENT WITH MEDICATIONS 49
You can see that oxcarbazepine (Trileptal), carbamazepine (Tegretol), phenytoin

(Dilantin), and phenobarbital are targeted mainly at the partial seizures, but addi-
tionally 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 pheno-
barbital, 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 Administra-
tion (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.
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 gen-
eralized 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 signifi-
cant changes in cognitive function from the anticonvulsants (Devinsky 2002,
p.225). Therefore, any change in the child’s behavior or intellect should be dis-
cussed 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.)
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.
• 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
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 formula-
tion 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 regard-
ing 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:
• 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
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.
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 informa-
tion 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.
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 prepara-
tions 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 insignifi-
cant—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 inap-
propriate 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 interac-
tions, 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.
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 mineraliza-
tion 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 phe-
nobarbital 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.
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 phenobar-
bital. 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
• tremor
• brittle hair (which is usually not a significant problem, and can be reduced
with selenium and zinc vitamin supplements).
• 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).
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 child-
hood 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:
• 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 benzodiaz-
epine. 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 pro-
longed seizures (“status epilepticus” on p.23).
This class of medication is actually fairly safe. It is a “what-you-see-is-
what-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 pharma-
cist. 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 depend-
ency 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
restriction comes from the limited number of children who were studied during
the original trials. Fortunately, recent US laws will now require that new medica-
tions 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 con-
junction 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 general-
ized 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, unsteadi-
ness, 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 compre-
hensive 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.
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-what-
you-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, includ-
ing 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 gener-
alized 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 tem-
perature. 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 distur-
bances 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 con-
junction with a drug level, complete blood count, and urine analysis.
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:
• add-on therapy for children two years or older with partial seizures
• add-on therapy for children two years or older with the generalized
seizures (tonic-clonic, atonic, and myoclonic) of Lennox–Gastaut
syndrome
• monotherapy (treatment with just one anticonvulsant) for adults with
partial seizures who are converting from other anticonvulsants.
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 con-
versely 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.
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 associ-
ated 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 pos-
sibility 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-intu-
itively, 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 signifi-
cant 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 treat-
ments 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
66
TREATMENT OPTIONS OTHER THAN MEDICATION 67
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-con-
trolled 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.
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 excel-
lent 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
TREATMENT OPTIONS OTHER THAN MEDICATION 69
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 particu-
larly 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 ade-
quately 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.
• 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.
• 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.
• 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.
70
FOR KIDS TO READ WITH THEIR PARENTS 71
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.
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:
• Sometimes, we might bite our tongue. It may hurt a little, but usually isn’t
serious.
• 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. Some-
times 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
good night’s sleep—especially if they have a seizure condition. However, some-

times 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 amuse-
ment 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
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 experi-
ence 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.
• 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).
• No climbing higher than about six feet (the height of an adult).
• 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.
• Use common sense.
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
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 partic-
ular 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 dis-
orders 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.
• 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
76
FOR TEENS WITH EPILEPSY 77
have only had one recent seizure in your life. Discuss this with your
doctor.
• 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.
• 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.
1. Get enough sleep!!!

2. Do not use alcohol or recreational drugs!
3. Take your medication as your doctor recommends—do not miss doses!
4. Do not drive if you “feel” like you may have a seizure.
5. 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 comfort-
able 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
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.
• 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.
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 accommoda-
tions. 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 experi-
ence 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.
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 signifi-
cant 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 alto-
gether. 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 ciga-
rette-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!
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
87
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 general-
ized 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 syn-
dromes, while remaining consistent with both the old and currently proposed clas-
sification 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 indi-
vidual type of seizure can be a building block in many kinds of epilepsy syn-
dromes. For example, absence seizure events can be part of the epilepsy syn-
dromes 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

“petit mal” epilepsy): • Benign childhood epilepsy with centrotemporal spikes • Neonatal seizures (not discussed in
° Childhood (a.k.a. benign rolandic epilepsy) this text)
° Juvenile • Benign childhood occipital epilepsy:
• Juvenile myoclonic epilepsy ° early onset (Panayiotopoulos type)
• Epilepsy with generalized ° 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.
Table 11.3 Relationship between generalized seizures and generalized epilepsies
Generalized (start both hemispheres)
Type of seizure Type of epilepsy syndrome
Childhood Juvenile Epilepsy with Infantile spasms Lennox–

and juvenile myoclonic tonic-clonic (EEG: Gastaut (EEG:
absence epilepsy seizures only hypsarhythmia) slow spike/
epilepsy wave)
(EEG:
3-per-second
spike/wave)
Absence ++ + +
Tonic/Clonic 10% risk + ++ +
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 Symptomatic focal epilepsy syndromes
Benign childhood Childhood Temporal lobe Frontal lobe

epilepsy with occipital epilepsies: epilepsies
centrotemporal epilepsies: Mesial temporal Parietal lobe
spikes (a.k.a. Early onset Lateral temporal epilepsies
benign rolandic Late onset Occipital lobe
epilepsy) epilepsies
Partial simple + + +
Partial complex + + +
Partial seizures + + + +
with secondary
generalization
Key: + common
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)
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.
91
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 particu-
larly 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). There-
fore, 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.
BENIGN CHILDHOOD EPILEPSY WITH CENTROTEMPORAL SPIKES 93
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 promi-
nent 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 impor-
tant 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 recur-
rent 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
outgrown his or her seizures. A recent study has suggested that a one-year treat-
ment 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 associ-
ated 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
• another 19 percent will just have the spikes on EEG, but not have actual
seizures
• 10 percent will have a history of BCECTS in one of the parents (Heijbel et
al. 1975).
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).
95
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 text-
books 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)
• typical onset with nausea and/or vomiting (which does not occur with
BCECTS)
• eyes and head usually turn to one side, or the child stares straight ahead
• 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 anti-
convulsant 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.
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 move-
ments. 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 per-
formed 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
CHILDHOOD OCCIPITAL EPILEPSIES 99
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.
100
THE TEMPORAL LOBE EPILEPSIES 101
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 sub-
sequently 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 hippo-
campus is a part of the brain that controls emotion and memory storage, and “scle-
rosis” means a scarring of tissue. Although this damage is often caused by devel-
opments 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 illu-
sions.
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.
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 general-
ized 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 tradi-
tional 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).
THE TEMPORAL LOBE EPILEPSIES 103
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 symp-
tomatic 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 child-
hood 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.
104
OTHER TYPES OF SYMPTOMATIC FOCAL EPILEPSIES 105
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.
• 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. Partic-
ularly 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 malforma-
tions, old scars from birth or head trauma, or encephalitis. The MRI will usually do
OTHER TYPES OF SYMPTOMATIC FOCAL EPILEPSIES 107
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.
108
CHILDHOOD AND JUVENILE ABSENCE EPILEPSY 109
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 Occurs only during “down time,” such as while
activity, such as while talking or eating. bored or watching TV.
Touching or loudly calling to the child does The spell stops when the child is called loudly
NOT end the spell. or touched.
There may be associated symptoms such as eye There are no such associated symptoms.
fluttering, lip smacking, or body twitching.
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
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 child-
hood 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
• 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
• 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.
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
CHILDHOOD AND JUVENILE ABSENCE EPILEPSY 111
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 prog-
nosis 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 dif-
ference 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 convul-
sive 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.
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

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.
113
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 adoles-
cence, 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 com-
plexes. However, a patient may have JME and have a normal EEG. CT scans and
MRI are generally normal.
JUVENILE MYOCLONIC EPILEPSY 115

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

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 awaken-
ing—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.
116
IDIOPATHIC EPILEPSY WITH GENERALIZED TONIC-CLONIC SEIZURES 117
How is the syndrome diagnosed?

In addition to the typical history as above, the EEG shows generalized spike dis-
turbances 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. Anti-
convulsants 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 appropri-
ate lifestyle and medication treatment, though, the seizures can usually be brought
under control.
Chapter 19

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.
118
INFANTILE SPASMS (WEST SYNDROME) 119
What are infantile spasms, and who has them?

The term “infantile spasms” refers to an epilepsy syndrome seen in infants typi-
cally 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 condi-
tion. 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.
Table 19.1 Distinguishing a normal baby “Moro” startle response from a myoclonic seizure
Normal Myoclonic
“Moro” startle response seizure
What is the age of the infant at Maximal frequency at birth, Rare at birth, and then get
onset? and then get less frequent more frequent
Stop by 4–5 months of age Begin from several months of
age to 2 years
Are they induced by stimulus Yes Not typically

such as loud noise, fright, or
being lowered backwards?
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 neurolog-
ical 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 cur-
rently “hidden” or unknown.
Symptomatic or “remote” group

Other children, like John (above), have the seizures as a result of some clear neuro-
logical 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 symptom-
atic 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
• 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)
• 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
• high muscle tone of the infant (“spasticity”) causing arching
• Benign Neonatal Sleep Myoclonus (just brief startles only during early
sleep with a normal EEG)
• 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
• myoclonus that is a secondary effect of certain tumors.
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 abnor-
malities; chromosomes; ophthalmology examination; viral titers; and possibly
lumbar puncture (“spinal tap”). Sometimes, a PET scan can pick up particularly
active foci of seizures.

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 neurolo-
gists in the UK, vigabatrin was the initial treatment used (Mackay et al. 2004).
The American Academy of Neurology, in conjunction with the Child Neurol-
ogy Society, reviewed the literature of 159 articles. Trying to apply strict scientific
standards, they produced a practice parameter with the following conclusions:
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 treat-
ment.
° 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.
• 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 infan-
tile 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.

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

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:
126
LENNOX–GASTAUT SYNDROME 127
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.
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.
• 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 continu-
ing 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 intellec-
tual 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
• 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
• 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).

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.
• 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.

Unfortunately, children with Lennox–Gastaut rarely do well from either an intel-
lectual or a seizure control perspective (Brown and Holmes 2004; Dulac and Engel
2003). Even children from the cryptogenic group rarely have a preserved develop-
ment. 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)
• generalized (85–95 percent of febrile seizures involve both sides of the body
equally)
• occur only once per 24-hour period
• usually occur within 24 hours of onset of high fever—typically greater
than 39 degrees Centigrade (102.2 degrees Fahrenheit)
132
FEBRILE SEIZURES 133
• 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 physi-
ological 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.
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 individual-
ized, 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 individual-
ized, 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.
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 men-
ingitis) (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).
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 2.3%
• complex febrile seizures 1.4%
• family history of afebrile seizures 3.6%
Had febrile seizures with two risk factors above 9.6%
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
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 useful-
ness of this advice is lacking. Appropriate use of fever medications should be dis-
cussed 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 phenobarbi-
tal. 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. Typi-
cally, 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 pro-
longed. 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 respira-
tory 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 hyperactiv-
ity/irritability in the child, occasional sleepiness, unlikely allergic reactions, and the
(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 particu-
larly 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 signifi-
cantly 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! Recap-
ping 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.
139
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.
141
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 143
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.
• 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.
1 Disclaimer: Views expressed in the books and Internet sites listed here do not necessarily
represent the views of the author.
144
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.
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 Neurol-
ogy, 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.
147
BCECTS (benign childhood “clonic” seizures 21
epilepsy with COE see childhood occipital
SUBJECT centrotemporal spikes) epilepsies
INDEX 91–4
benign early onset COE 96–7
college 81–2
complex febrile seizures 133
benign rolandic epilepsy (BRE) complex partial seizures 22,
39, 91–4 47–9, 101
Page numbers in italics refer to benzodiazepines 48, 50, 60–1, cranial nerves, functions of 35
tables. 130 CT (Computerized Tomogra-
biking, risks of 32 phy) scans 36–7, 134
absence seizures 18, 20, 22, birth control pills, effects of
102, 109 anticonvulsants 55, 62, Depakene see valproic acid
ACTH (adrenocorticotropin 78–9 Depakote see divalproex sodium
hormone) blood tests 40, 51–2, 134 diagnosis 34–41
side effects of 122–3 bone marrow problems 51, 55, BCECTS 93
treatment for infantile 60, 65, 130, 131 childhood absence epilepsy
spasms 124–5 brain scans 36–7 (CAE) 110
ADHD (attention deficit hyper- brain surgery 68–9 early onset COE 97
activity disorder) 109 breath holding spells 24, 27 febrile seizures 133–4
afebrile seizures 133, 136, 138 breathing infantile spasms 122
akinetic seizures see atonic failure following seizure juvenile myoclonic epilepsy
seizures 31 (JME) 114
alcohol, dangers of 82 problems during seizures late onset COE 98
allergic reactions 51, 57, 58, 62, 28 Lennox–Gastaut syndrome
64, 65 protecting airway during 129
American Academy of Neurol- seizures 30 MTLE 101–2
ogy 42, 122 Diastat see rectal diazepam
antibiotics, interaction with CAE see childhood absence diazepam 23–4, 60–1, 137
anticonvulsants 54 epilepsy Dilantin see phenytoin
anticonvulsants 56–65 calcium 55 diphenylhydantoin see
behavioral effects 50 carbamazepine 48, 50, 56–7 phenytoin
choice of 47–50 Carbatrol see carbamazepine divalproex sodium 49, 59
deciding on treatment with careers 80–1 driving 76–8
42, 44 CAT scans see CT (Computer- drop attacks see atonic seizures
monitoring effects of ized Tomography) scans drug levels, monitoring 52
51–2 Child Neurology Society 42, drugs, recreational 82
and pregnancy 78–80 122
stopping 45–6 childhood absence epilepsy EEG (electroencephalogram)
titrating dosage of 52–3 (CAE) 18, 108–11 for children with BCECTS
usage principles 53–5 childhood occipital epilepsies 93
vitamin supplements 55 (COE) 95–9 febrile seizures 134
Ativan see lorazepam children, advice for 70–5 information for children
Atkins diet, modified version classification 72–3
68 epilepsy syndromes 88–90 measurements 37–9
atonic seizures 21, 128 seizure types 20, 87 types of 39–40
atypical absence seizures 127 clinical symptoms, medication emotional aspects, stopping
auras 17–18, 19, 27, 71–2, 101, 51 medication 45–6
102 clonazepam 50, 61 employment 80–1
148
INDEX 149
epilepsy “Jacksonian march” 22, 105 MRI (Magnetic Resonance

classification of syndromes JAE see juvenile absence Imaging) scans 36–7, 73,
88–90 epilepsy 134–5
definition 18 JME see juvenile myoclonic MTLE see mesial temporal lobe
historical timeline 139–40 epilepsy epilepsy
risks of developing 136–7 juvenile absence epilepsy (JAE) myoclonic seizures 21, 72, 114,
surgery 68–9, 103 111–12 119–20
epilepsy partialis continua 105 juvenile myoclonic epilepsy Mysoline see primidone
ethosuximide 48, 50, 110 (JME) 113–15
eye examinations 35 neurological examinations
Keppra see levetiracetam 34–6
fainting spells 24, 28 ketogenic diet 66–7, 130 Neurontin see gabapentin
febrile seizures 101 kidney stones, risk of develop- nocturnal seizures 28, 93, 105
felbamate 65, 130 ing 52, 63, 65, 67 not-so-benign rolandic epilepsy
Felbatol see felbamate Klonopin see clonazepam 94
flashing lights 27, 33, 74, 117
focal seizures see partial seizures laboratory testing, medication observations to make during a
folic acid 55, 79 51–2 seizure 26–9
Lamictal see lamotrigine ophthalmoscopic examinations
gabapentin 48, 49, 63 lamotrigine 48, 49, 50, 64, 130 35, 123
Gabitril see tiagabine late onset COE 98–9 osteoporosis 58
Gastaut type epilepsy 98–9 lateral temporal lobe epilepsy oxcarbazepine 48, 49, 50, 62
generalized epilepsies 89, 90 (LTLE) 100, 102
generalized seizures 19–21 Lennox–Gastaut syndrome Panayiotopoulos type epilepsy
anticonvulsants for 48, 126–31 96–7
49–5 levetiracetam 48, 49, 50, 62 pancreatitis 60
link to generalized liver dysfunction 51, 60 partial complex seizures 22, 27,
epilepsies 90 liver function tests (LFTs) 52 47–9, 101
generic medications 53 local seizures see partial seizures partial epilepsies 89, 90, 100–7
genetic issues 80, 92, 94, 114 lorazepam 50, 61 partial seizures 19, 21–3, 88
“grand mal” seizures see LTLE see lateral temporal lobe anticonvulsants for 47–9
tonic-clonic seizures epilepsy classification 20, 87
lumbar punctures 41, 133 link to partial epilepsies 90
half-life of drugs 54 see also idiopathic focal
headaches 24, 28, 93, 98 medical assistance, when to call epilepsies; symptomatic
hereditary issues 80, 94, 114 for 31 focal epilepsies
hippocampal sclerosis 100, medication PET (Positron Emission
101, 102, 137 anticonvulsants 47–65 Tomography) scans 37
“homeopathic” treatments 69 compliance with 83 “petit mal” epilepsy see child-
hypsarrhythmia 118, 119, 122, decisions on treatment hood absence epilepsy
123 with 42–6 “petit mal” seizures see absence
mesial temporal lobe epilepsy seizures
infantile spasms 118–25 (MTLE) 100–2 phenobarbital 49, 50, 52, 58,
injuries, protection against 21, migraines 24, 98 59, 137–8
30, 33 “Moro” startle responses phenytoin 48, 49, 50, 58, 130
International League Against 119–20 physical examinations 34–6
Epilepsy (ILAE) 88, 140 motor area seizures 105 polycystic ovarian syndrome 60
Internet sources 144–6
practice parameter, treatment parietal lobe 106 Valium see diazepam

of seizures 42–4, 123–4 temporal lobe 100–3 valproate 49, 64
pregnancy 54–5, 78–80 valproic acid 48, 49, 54, 55, 59,
primidone 59 teenage issues 76–83 130
prolonged seizures 23–4, 42, Tegretol see carbamazepine video games 73–4
44, 104, 137 temporal lobe epilepsies (TLEs) vigabatrin 48, 65, 123
pseudo-seizures 25 100–3 vitamin supplements 55
psychomotor seizures see temporal lobe seizures see VNS see vagal nerve stimulator
complex partial seizures 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
Sabril see vigabatrin topiramate 48, 49, 50, 63, 130
safety precautions 21, 30, 31, treatment 42–6
32–3 BCECTS 93–4
sedation, minimizing 54 childhood absence epilepsy
seizures (CAE) 110–11
definition of 17–18 early onset COE 97
diagnosis 34–41 febrile seizures 137–8
events that mimic 24–5 generalized seizures 117
explaining to children idiopathic epilepsy with
70–5 generalized tonic-clonic
observation of 26–9 seizures only 117
types of 19–24, 87 infantile spasms 122–5
what to do 30–1 juvenile myoclonic epilepsy
simple partial seizures 22, 47–9 (JME) 114–15
skin rashes 51, 58, 62, 64, 65 Lennox–Gastaut syndrome
sleep, importance of 80, 81 130–1
smoking 82 temporal lobe epilepsy
SPECT (Single Photon (TLE) 102–3
Emission Computed treatment types
Tomography) scans 37 “homeopathic” 69
“spinal tap” 41, 133–4 ketogenic diet 66–7
sports 33 medication 47–65
status epilepticus 23–4, 44 surgery 68–9
Stevens–Johnson syndrome 51, vagal nerve stimulator
62, 64 (VNS) 68
supplemental sensory-motor Trileptal see oxcarbazepine
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
Hirtz, D. 24, 42, 43, 44 Verity, C.M. 135
Holmes, G.L. 49, 97, 105, 127, Vining, E.P. 33
AUTH O R 130, 131
INDEX Watemberg, N. 49
Janz, D. 117
AAP (American Academy of Kellaway, P. 119

Pediatrics) 134 Kim, W.J. 102
Annegers, J.F. 107 Knudsen, F.U. 137
Ko, D.Y. 102, 103
Beaussart, M. 92, 94 Kosoff, E.H. 67, 68
Benbadis, S. 110, 111 Kutscher, M.L. 109
Berg, A.T. 43, 136
Berkovic, S.F. 110, 111 Lee, S.J. 102
Blom, S. 92 Lombroso, C.T. 93, 124
Braathen, G. 94 Luders, H. 92
Brown, T.R. 49, 97, 105, 127,
131 Mackay, M.T. 122, 123, 124
Mascha, E. 103
Camfield, C.S. 43 Matsumoto, A. 124, 125
Maytal, J. 135
Dalla Bernadina, B. 92 Morita, D.A. 128
Delanty, N. 115 Morton, L.D. 49
Devinsky, O. 11, 25, 50, 54, 69,
76, 82 Nashold, B. 103
Dulac, O. 128, 130, 131 Nelson, K. 135, 136
Elveback, L.R. 107 Panayiotopoulos, C.P. 49, 56,

Engel, J. 93, 128, 130, 131 96, 97, 98–9, 109, 110, 111,
Epilepsy Foundation of 116, 119
America 11, 30, 70, 74, 76 Papavasiliou, A. 94
Park, S.C. 102
Fejerman, N. 93 Pellock, J.M. 49
Foldvary, N. 103, 104, 105, Pillas, D.J. 33
106, 107
Freeman, J. 33, 43, 45, 69, 77, Rasmuson, M. 92
79, 80, 135 Renganathan, R. 115
French, J. 49, 56 Riikonen, R. 124, 125
Gastaut, H. 127 Sahai-Srivastava, S. 102, 103

Glauser, T.A. 128 Schachter, S. 65
Serratosa, J.M. 114
Hauser, W.A. 107 Shevell, M.I. 93
Heijbel, J. 92, 94
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And to my amazing wife.

PART 1: OVERVIEW OF SEIZURES AND THEIR CARE

PART 2: INDIVIDUAL EPILEPSY SYNDROMES

FURTHER READING 144

ABOUT THE AUTHORS 147

SUBJECT INDEX 148

AUTHOR INDEX 151

My introduction to epilepsy occurred during my freshman year of high school.

If you’re reading this…

Knowledge is an antidote to fear

The scope of the problem

Who is this book for?

Organization of this book

Cause for humility on the part of doctors

How does the term “seizure” relate to the term “epilepsy?”

Introduction to “generalized” and “partial” seizures

Types of generalized seizures

Table 1.1 A simplified classification of seizure types

Types of partial seizures

Simple partial seizures

Complex partial seizures

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 Absent or mild Prominent

Post-ictal confusion Absent Prominent

Partial seizures with secondary generalization

Whatever happened to the names “petit mal” and “grand

What is “status epilepticus?”

Status epilepticus is a medical emergency

What events can mimic seizures?

• Behavioral rage attacks can usually be distinguished from seizures.

Table 2.1 Observations during a seizure

Feature of the spell Importance of that feature

Were there palpitations These symptoms might suggest a cardiac problem.

Continued on next page

Table 2.1 cont.

Feature of the spell Importance of that feature

Try your best

• Do not attempt mouth-to-mouth or cardio-pulmonary resuscitation

Balancing risks and restrictions

Reasonable accident precautions

The medical work-up of a child with suspected seizures involves a history,

History, physical, and neurological examinations

your nose or perform complicated movements. The cerebellum is rarely

Pictures of the brain: CT, MRI, and PET scans

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

Better at detecting fresh blood Less sensitive at detecting fresh blood

Uses X-rays Uses magnet and radio waves

The electroencephalogram (EEG)

Figure 5.1 Schematic of EEG findings