Sharing the vision epilepsy: life’s turning point Gavin Dimitri

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THE EPILEPSY REPORT OCTOBER 2008

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                          THE EPILEPSY REPORT OCTOBER 2008

kinds of seizures. If you do not have 

access to this kind of training, there is 

still something very useful you can do.

Take advantage of the greatest 

advancement in seizure observation 

in the 3,000-year history of epilepsy. 

Videotape your child’s seizures. You 

may have to set up the camera and run 

it for days or weeks before you catch a 

seizure. Just rewind the tape and use it 

again. Be sure your child’s entire body 

fits in the picture frame. If your child 

has seizures in her sleep, use a nightlight 

in the bedroom. Most video cameras 

will record OK in low light. Turn up the 

thermostat so the child can sleep without 

a sheet or covers on top, and dress the 

child in pajamas that have trousers, not 

skirts, so that leg activity can be easily 

seen. Once you have the seizures on 

tape, the doctor can go over it frame by 

frame if necessary to come up with a 

more accurate diagnosis.

EEG

Sometimes the doctor can get around 

observation problems with an EEG 

(electroencephalogram.) The EEG can 

detect the tiny electrical activity of the 

brain that is a part of the “wave.” If the 

doctor is lucky enough to catch a seizure 

while the child is hooked up to the EEG 

machine, she may be able to tell what 

kind of seizure it is by seeing where the 

“wave” starts, where it spreads, and how 

fast it goes “up and down.” Notice I said 

“lucky.” When the child is not having 

seizure activity in the brain, the EEG 

will find only normal electrical activity. 

Guess what is happening most of the 

time for most children with epilepsy? 

No seizures. This is why your child can 

have epilepsy even though he or she has 

had many EEGs that all failed to find 

any seizure activity.

If diagnosis is a real issue for 

your child, a special kind of EEG 

can be performed. It is commonly 

called intensive monitoring or video 

monitoring. This is done in the hospital. 

The child is hooked up to an EEG 

machine in a special hospital room that 

contains a video camera. The video 

and EEG run 24 hours a day, usually 

for one to five days until seizures are 

recorded. In addition to being able to 

wait for the child to have a seizure, this 

procedure allows the doctor to see what 

is happening on the outside of the child 

(the video) and at the same moment see 

what is happening on the inside (the 

EEG.) Intensive monitoring is also an 

important step when considering surgery 

for the treatment of epilepsy (more about 

that later.)

Seizures & brain disorders

Seizures are like sneezes – they 

are the symptom of another disorder. 

Seizures are a symptom of some kind 

of brain disorder. The fact that your 

child has seizures does not tell you 

what the brain disorder is. It could 

be slight tissue damage from a brain 

infection or stroke, a small abnormality 

in brain development, or a genetic 

variation in brain function – any of a 

hundred different things. In about 75% 

of children this disorder is so small or 

so subtle it can’t be found, even with 

the most up-to-date brain scans. The 

only way we know something is there 

is because the child is having seizures. 

Many children will have what I call 

the “million dollar workup” of medical 

tests, only to find nothing. That is 

actually the best news you can have. 

As a rule of thumb, the larger and more 

detectable the brain disorder the seizures 

are a symptom of, the more difficult 

the seizures will be to control. There 

are exceptions to this rule, but this 

sometimes inaccurate rule of thumb can 

alert you to what you might expect.

Another rule of thumb is seizures that 

start in the first year of life tend to be 

troublesome. Again there are exceptions. 

While seizures that start in the first year 

of life may be particularly difficult to 

control, it is usually the underlying brain 

disturbance that is the biggest problem. 

Some of these get worse over time and 

some don’t. Much of the attention in 

treatment may be focused on the brain 

disorder rather than the symptom of 

“seizures.” Many of the genetic and 

developmental abnormalities that lead to 

severe seizures and brain disturbances 

occur in the first year. Fortunately these 

are rare. Knowing if your child has one 

of these types of epilepsy (usually called 

an epilepsy syndrome because they 

include several kinds of  seizures and/or 

other important symptoms) is important 

for understanding what you and your 

child may face in the coming years. 

(See Table 1 on Epilepsy Syndromes.) 

Seizures that begin in childhood after 

the first year of life tend to be easier to 

control.

Be sure to get the diagnosis from 

your doctor. Have him or her write 

down the full medical name of the type 

of seizures or epilepsy syndrome your 

child has. You will need this in order to 

do your own research into the treatment 

and possible outcomes of your child’s 

seizures. Diagnoses such as “grand mal” 

or “petit mal” are not adequate. There 

is an International Classification of 

Epileptic Seizures and an International 

Classification of Epilepsies and Epileptic 

Syndromes that provides medical names 

used in books and articles on diagnosis 

and treatment, including those on the 

Internet. In addition there are other 

names for certain kinds of epilepsy that 

are generally used and accepted. These 

types of epilepsy are often named after 

the person(s) who first reported their 

discovery.

While seizures may appear 

frightening, there is not much evidence 

“regular” seizures in and of themselves 

cause noticeable brain damage. 

Researchers have recently been able to 

show seizures do affect the “wiring” 

of the brain in subtle ways. Epilepsy 

scientists argue about how important this 

is to thinking, memory, and intelligence. 

Psychological testing shows epilepsy 

can result in memory disturbances, but 

good research has only been able to 

show decreases in intelligence after 20 

– 30 years of poorly controlled seizures. 

Again there are arguments about 

whether and how often these changes 

are enough to impair the quality of the 

person’s life. This issue is made all the 

more confusing because epilepsy goes 

away with a surprising frequency. If 

seizures changed the brain so much, how 

could epilepsy go away so often?

The brain disorder for which the 

seizures are just a symptom can play the 

major role in life outcomes. If the brain 

disorder also left the child mentally 

or developmentally challenged, this 

can limit adult success. In such cases, 

treatment and rehabilitation of the 

brain disturbance is often much more 

important to long-term outcomes than 

are the seizures. There is a minority of 

children with epilepsy whose seizures 

are so frequent or so severe that epilepsy 

is the major problem. These seizures 

need to be treated as aggressively as 

possible.

Syndrome Name

Age of 

onset

Description

Ease of 

control

West Syndrome 

(Infantile Spasms)

3 mos – 

2 years

Sudden flexing of body (child looks like it is curling up) or less common sudden extension (arms 

and legs thrust out.) Spasms occur in clusters and can range from very mild to strong. As high 

as one-fifth may die in early life, usually due to aspiration pneumonia – greatest risk is in children 

with a known cause for the spasms. About 90% show mental deterioration. Spasms usually stop 

by age 5, but half or more go on to have other epilepsy, 30% - 40% get Lennox-Gastaut.

Difficult

Tuberous Sclerosis

3 mos –

20 years

Over 80% develop epilepsy. TS can result in several kinds of seizures. Up to 70% have Infantile 

Spasms, with about 25% having partial or secondarily generalized seizures. Mental retardation 

40% - 60%. One quarter to one half have infantile autism. Most problems caused by tubers, not 

epilepsy. Surgical treatment becoming an option.

Difficult

Sturge-Weber Syndrome

Usually 

before 2 

years

Up to 90% develop epilepsy. Usually Partial or Secondarily Generalized seizures. Seizures 

can stop for a while, then return. About half are controlled. Surgery is an option for refractory 

seizures.

Easy to 

difficult

Severe Myoclonic Epilepsy of

Infancy (Dravet Syndrome)

Before 3 

years

Often starts with febrile convulsion. Myoclonic seizures eventually follow, may cause falls. 

Absences in 40% - 90%, partial seizures 50%. Mental retardation always, sometimes severe, 

becomes stable.

Never 

controlled

Myoclonic Astatic Epilepsy

of Infancy (Doose Syndrome)

1 – 5 

years

Strong Myoclonic or Atonic seizures resulting in falls. 50% have Absences with myoclonic 

jerks. 75% have Tonic-Clonic at some time. About half get seizure control. Hyperactivity, poor 

attention, moodiness may be present.

Easy to 

difficult

Lennox-Gastaut Syndrome

1 – 8 

years

Generalized seizures usually including two or more of the following: Tonic, Atonic, Myoclonic, 

Absence, more rarely other types. Seizures often daily. Non-convulsive status epilepticus 

common, sometimes subtle, and can be made worse with overmedication. Behavioral,

cognitive, and social impairment common.

Very 

difficult

Epilepsy with Continuous

Spikes and Waves During

Slow Sleep

1 – 11 

years

Often starts as a Clonic seizure, then evolves into Simple Partial, Complex Partial or Tonic-

Clonic types. Absence status can occur in nearly half. Seizures stop near puberty, however 

mental development is usually impaired at time of onset, severe in half. Severe behavioral

problems may occur with various degrees of hyperactivity and aggressiveness. Memory poor.

Usually 

easy, 

seizures go 

away

Landau-Kleffner Syndrome

3 – 8 

years

The main symptom is loss of oral language abilities over the course of days. Behavioral 

disturbances and hyperactivity are common. Seizures in 70%, though abnormal EEG in 100%. 

Partial, Absence, and/or Tonic-Clonic seizures may be present and do not affect outcome. 

Intelligence usually maintained.

Usually 

easy

Childhood Absence Epilepsy

4 – 8 

years

Usually very frequent Absence seizures throughout the day. Tonic-Clonic seizures in 40%. Good 

prognosis. Most become seizure free.

Usually 

easy

Juvenile Absence Epilepsy

10 – 17 

years

Usually only one or a few Absence seizures per day. Absences can sometimes be triggered by 

doing math or spatial tasks. Tonic-Clonic seizures occur in 80%. Long-term outcomes not well 

known.

Usually 

easy

Juvenile Myoclonic Epilepsy

12 – 18 

years

Usually mild to moderate myoclonic jerks of shoulders, typically after awakening. 90% have 

Tonic-Clonic seizures. One third have Absences. Sleep deprivation, fatigue, and alcohol are 

often triggers. Normal intelligence. Lifetime medication often required.

Usually 

easy

Table 2: Epilepsy Syndromes

For most children it is the emotional 

and social reactions people have to 

seizures and epilepsy (especially in 

the family) that may have more to do 

with how the person turns out. Studies 

have found seizure types and number 

of seizures have little to do with how 

successful a person with epilepsy 

might be in adult life. Some people 

with frequent seizures are great social 

and financial successes. Some people 

who have had only a couple seizures in 

their life become disabled. Obviously, 

there is a lot of opportunity to change 

these reactions and improve outcomes. 

This will be the topic of future articles. 

For now we will stick with the medical 

aspects. In next month’s article I will 

discuss the incredible range of treatment 

options available to you and your child – 

and how to get the most out of each. 

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