Children with Epilepsy

FEBRILE SEIZURES

Febrile seizures are provoked seizures which occur in the setting of increased body temperature at 102 degrees or above in children between ages of 18 months and 3 years. There is no underlying identifiable central nervous system condition such as infection or others as a cause.

Incidence of febrile seizure is increased in the families of the children with febrile seizures. Children of parents with history of febrile seizures have increased risk of febrile seizures.

Febrile seizures usually occur as the body temperature rises rapidly. Early detection of ongoing sickness may help to prevent temperature rise and may prevent febrile seizures.

Febrile seizures are classified as simple and complex. Simple febrile seizure last less than 15 minutes with normal neurological exam and lack of preceding other unusual symptoms. The child will have shaking or jerking movements of the limbs with brief loss of consciousness. Majority of children will have only one time event. There is usually no need for hospitalization and further investigation.

Complex febrile seizures occur longer than 15 minutes and can recur in 24 hours. Usually there is underlying central nervous pathology. The seizure may start as focal seizure which then evolves into convulsion. These kids may have abnormal neurological exam. The offspring of parents with epilepsy have increased risk of complex febrile seizures. Children with complex febrile seizures have increased risk of having epilepsy as they grow up.

Treatment of simple febrile seizures is to control the fever.  Usually there is no need for antiepileptic drugs. If seizures recur or  last long then use of antiepileptic medication may be needed. Rectal diazepam can be administered by parents at home. Intravenous antiepileptic drugs can be used in hospital settings when needed. Children with complex febrile seizures need to have further investigation such as imaging of central nervous system, electroencephalogram and other tests to rule out underlying central nervous system pathology.

SOME EPILEPSY SYNDROMES IN CHILDREN

Benign Rolandic Epilepsy: The most common epilepsy syndrome in children. It occurs at age between 2 years and 12 years. It happens during sleep. It starts as focal motor seizures involving the tongue, lips and mouth. The children can have numbness and tingling over mouth and tongue which evolve into forced vocalization and drooling. It can be followed by grand mal seizures. These children have normal neurological exam. Their electroencephalograms (EEG) shows characteristic epileptiform discharges over the central and temporal areas of the brain. They are usually seen on one side but can be bilateral. Imaging of brain does not show any abnormality. Most often the children will have only one seizure. Rarely medical treatment is needed since it happens usually during sleep and infrequently. If medication is needed, there is good response to antiepileptic drugs. These children usually outgrow  seizures. Antiepileptic drugs can be discontinued after seizure freedom for 2 years.

Childhood Absence Seizures: The old name is Petit Mal seizures. Onset age is between ages of 4 to 10 years. There is strong genetic predisposition. It is characterized by brief loss of awareness, staring and pause of previous activities. The duration is usually less than 10 seconds but can be longer. There is no confusional state afterwards. The children can continue their ongoing activities. Most often these seizures are noticed by teachers at school. Initially complaints to parents from teachers are frequent daydreaming or decreased attention during classes. There is typical EEG pattern to diagnose this syndrome. It is called 3 Hz spike and wave pattern. Hyperventilation can trigger seizures. Ethosuximide, Valproate and Lamotrigine are drug of choices. A recent study compared these three medications in children with absence seizures. Ethosuximide was found to be provide the best seizure control. The long term prognosis is excellent. Most of children outgrow this syndrome.

Juvenile Myoclonic Epilepsy (JME): The onset age is between 12 and 18 years. Three seizure types can be seen. Myoclonic, absence and generalized tonic clonic seizures. Myoclonic seizures predominate this syndrome. Myoclonic seizures are involuntary brief jerk of the extremities, trunk or eyelids. Mostly affect the upper body, shoulder and arms.  The most common presentation is early morning jerks which may cause the child to drop things from his hands. If it happens in lower extremities, it may cause falls. Usually there is intact awareness during brief myoclonic jerks. In the presence of triggers such as sleep deprivation or alcohol use, myoclonic jerks can lead to generalized tonic clonic seizures. Electroencephalogram pattern in JME is generalized spike, polyspikes and slow waves. The antiepileptic drug of choices in JME is valproate, lamotrigine, topiramate, zonisamide and levetiracetam. The decision to choose the best medication requires consideration of multiple factors. Valproate should not be the initial choice in young females due to teratogenicity.  Unlike childhood absence seizures, children cannot outgrow JME. It requires lifelong treatment. Seizures are usually easily controlled with antiepileptic drugs.

Infantile Spasms (West Syndrome)

It is epilepsy syndrome seen in infants younger than 2 years. It can involve children up to age 7 years. The infant presents with spams involving the limbs. Spasms cause flexion of limbs and trunk lasting for a few seconds. They can cluster. The children with infantile spams have abnormal neurological exam, imaging studies and show developmental delay. Electroencephalogram appears chaotic with loss of normal background. It shows hypsarrhythmia described as with high voltage disorganized background with multifocal epileptiform discharges.

Prognosis is not good. Majority of children will continue to have seizures throughout their life. Seizure types may evolve into other types of seizures as well.

ACTH hormone in addition to other antiepileptic drugs such as vigabatrin, valproic acid, zonisamide, topiramate and benzodiazepines is recommended treatment. Vitamin B6 and ketogenic diet can be tried in treatment resistant cases. Vigabatrin is recently approved by FDA in treatment of infantile spasms.

Lenox-Gastaut Syndrome (LGS):

It is epilepsy syndrome with multiple different types of seizures. Patients with LGS have mental retardation and abnormal neurological examination. EEG pattern is slow spike and wave discharges. Onset is between ages 1-9 years old. About half of children with LGS start as infantile spasms. About in one third of cases underlying etiology is unknown. Work up in LGS should include investigation to rule out conditions such as metabolic and neurodegenerative diseases. LGS usually causes refractory seizures.

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