Kristen Park, M.D.
Children ’ s Hospital of Philadelphia

• Electrically: an abnormally synchronous firing of neurons that disrupts normal brain activity
• Clinically: has a variety of manifestations from abnormal sensations in a body part to convulsions

PARTIAL
• Involving part of the brain
• Simple partial
• Complex partial
GENERALIZED
• Involving the whole brain
• Absence
• Tonic
• Tonic-clonic
• Atonic
• Myoclonic

• Anything that irritates the brain can result in seizures. They are often a symptom of an underlying process. In these cases they are considered provoked.
– Infections like meningitis or encephalitis
– Trauma
– Drugs
– Electrolyte imbalances
– Tumors and strokes
– Fever

• In many cases, there is no discernible immediate cause.
• Seizures in this instance are considered
unprovoked.
• In these cases there may be a genetic basis, a malformation of cortical development, or a remote injury to the brain resulting in neurons that generate seizures.

• Epilepsy is the occurrence of more than one unprovoked seizure.
• Can occur as a particular syndrome that has a defined course and or treatment.
– Childhood absence epilepsy
– Juvenile myoclonic epilepsy
– Benign rolandic epilepsy
– Lennox-Gastaut syndrome

• Normal children with normal EEGs have a recurrence rate of 18% at 1y and 24% at 2y
• An abnormal EEG increases the risk to 42% at 1y and 52% at 2y
• Children with developmental disabilities and an abnormal EEG have an even higher risk

• Prevalence: total number of new and existing cases of a disease
– 2.3-2.5 million people in the United States (1%)
• Incidence: number of newly diagnosed cases each year
– 150,000-200,000 cases each year
– Mostly in young children and the elderly

• There is an increased risk of epilepsy in children with autistic spectrum disorders with estimates ranging from 7-42%
• 59% of newly diagnosed patients have partial epilepsy

• Not as easy as you might think!
• The black, white, and gray zones.
Definitely a seizure
Definitely not a seizure

• Syncope
• Gastroesophageal reflux
• Stroke
• Tics or other dyskinesias
• Psychiatric disorders
• Parasomnias

• Epilepsy is a clinical diagnosis
– An abnormal EEG does not a diagnosis of epilepsy make.
• Epileptiform abnormalities can be found in approximately 2% of the population
• They can be found in 13-83% of children with autistic spectrum disorders
• One study found them in 30% of children with ADHD
– A normal EEG does not exclude the diagnosis of epilepsy
• Sampling
• Many people with epilepsy have normal EEGs between seizures

• Scalp recording of the cumulative activity of the brain ’ s neurons
• Every person has a complement of brain wave frequencies, organization, and patterns associated with sleep or other activities.
• In many conditions, disruption of cortical brain wave activity occurs resulting in slowing, disorganization, and/or foci of irritability (spikes – groups of neurons firing together)

• What is a neurologist to do?
• Clinical judgement
– Characteristics of event: stereotyped, does not respect situation or time, makes physiologic sense
• Provocative procedures
– Sleep deprivation
– Hyperventilation
• Video EEG
– The so called “ gold standard ” in diagnosis as it is able to correlate the EEG with examination of the behavior of interest

• Medication should usually be instituted after the second unprovoked seizure
• 40-80% of patients will respond to the first anti-epileptic drug (AED)
• Of those who fail the first AED, 42% will go on to remission with a second agent

• Treatment goal should be “ no seizures, no side effects, and optimal quality of life ”
• Medication is chosen based on several factors:
– Type of seizure or epilepsy syndrome
– Coexisting medical conditions
– Dosing and monitoring
• Efficacy data not available for the majority of situations

• A misnomer as these drugs do not cure epilepsy but are designed to prevent or suppress seizures
• Approximately 15 drugs on the market, not all of which are available in the United States

• Phenobarbital
• Phenytoin (Dilantin)
• Carbamazepine
(Tegretol)
• Oxcarbazepine
(Trileptal)
• Felbamate (Felbatol)
• Valproate (Depakote)
• Benzodiazepines
• Lamotrigine (Lamictal)
• Topiramate (Topamax)
• Zonisamide (Zonegran)
• Levetiracetam (Keppra)
• Ethosuximide (Zarontin)
• Vigabatrin (Sabril)
• Gabapentin (Neurontin)
• Pregabalin (Lyrica)

• Many of these medications are also used to treat other conditions like migraine, pain, depression, etc.
• Since they are designed to suppress the central nervous system, neurologic side effects are commonly reported and can include:
– Dizziness
– Fatigue
– Cognitive slowing
– Etc.
• In addition, they may affect other organ systems within the body that may need to be monitored
– Liver
– Kidneys
– Blood

• 25% of children will not have good control of seizures with medication
• In this case, alternative treatments should be considered:
– Surgery
– Vagus nerve stimulator
– Ketogenic diet

• Designed for specific situations in which the focus of the seizures can be identified and is amenable to resection.
• Can be very effective if chosen cautiously with seizure freedom rates of 70-80% in children.

• Seizure freedom rate slightly less than that of medications
• Often used as adjunctive therapy
• Efficacy takes up to two years
• Has both a tonic mechanism to decrease seizure frequency as well as the ability to stop seizures after onset by activating the device with a magnet

• Known since biblical times that seizures decrease during times of fasting
– Matthew 17:15-21
“ Lord, have mercy on my son ” , he said.
“ He has seizures and is suffering greatly. He often falls into the fire, or into the water. I brought him to your disciples and they could not heal him.
” …Jesus replied… ” Bring the boy here to me…this kind does not go out except by prayer and fasting.
”

• Created a high fat, low carbohydrate, low protein diet that physiologically resembles this state
• True mechanism of action still debated
• Can be very effective in selected children but is strict in its requirements

• Not always.
• As the brains of children mature, they may
“ outgrow ” the tendency to have seizures.
• Anti-epileptic medication can be stopped successfully in many cases after a duration of seizure freedom (2-5 years).
• Under the best conditions, 69% of children will be able to be taken off medication without recurrence.

• Place the child in a safe area away from sharp objects, ledges, etc.
• Do not restrain the child.
• Turn the child onto his side so that he will not choke on vomit or saliva
• Loosen tight clothing and remove glasses
• Do not place anything into his mouth
– It is not possible to swallow one ’ s tongue and this can cause broken teeth and/or jaw
• Do not place your hand under his head
– The force of the seizure may break the fragile bones of the hand

• Vomiting
• Increased salivation
• Urinary and bowel incontinence
• Biting of the cheek or tongue
• Cyanosis of the lips or fingers and shallow breathing
• Post-ictal confusion, aggression, lethargy, headache, etc.

• First time seizure
• In a child with epilepsy:
– Seizure >5 minutes
• Most seizures last 1-2 minutes
– Back to back seizures without recovery in between
– Atypical or concerning features

• Seizures and Epilepsy in Childhood: A guide for parents. Second Edition. John
M. Freeman, M.D., Eileen P. G. Vining, M.D., and Diana J. Pillas. The Johns Hopkins
University Press, Baltimore. 1997.
• Clinician ’ s Manual on Pediatric Epilepsy: A guide to diagnosis, treatment, and future directions. Dennis J. Dlugos, M.D.
Consensus Medical Press. 2006.

• www.efa.org
• www.epilepsy.com
• Your local Epilepsy Foundation affiliate