POST TIME: 1 January, 2018 00:00 00 AM
Epilepsy syndromes in children
Dr Wrishi Raphael

Epilepsy syndromes in children

Epilepsy is a neurological disorder marked by sudden recurrent episodes of sensory disturbance, loss of consciousness, or convulsions, associated with abnormal electrical activity in the brain. Epilepsy syndromes in adults are often due to poorly diagnosed and managed convulsion disorders in children. It is important for general practitioners and parents to know of epilepsy syndromes in children as untreated epileptic activity could result in grave neurological deficits in future.

Febrile seizures

Tonic- clonic seizures occur in 2- 5% of children usually aged 6 months to 5 years who have a high fever generally caused by a viral infection. The long-term prognosis

is good and less then 3-5% of children with febrile convulsion may have epilepsy in the long run.

Infantile spasms

( Hypsarrhythmia )

Also known as West syndrome, these are generalized tonic seizures with sudden flexion of the arms, forward flexion of the trunk and extension of the legs, lasting only a few seconds, with usual age onset between 3 and 7 months. They are usually restricted to the first 3 years of life and are replaced by other forms of attacks. Prognosis for cognitive development is also unfavorable.

The most effective therapy is corticotrophin (ACTH) IM injection. Otherwise, oral prednisolone, benzodiazepines or sodium valproate can be used.

Lennox- Gastaut Syndrome (myoclonic epilepsy of infancy)

This uncommon syndrome refers to a triad of severe difficult-to-control seizures (usually tonic with drop attack), mental retardation and characteristic EEG. The seizures usually begin between ages 1 and 6 years with a peak onset at 3 to 5 years. Prognosis is also poor.

Sodium valproate is the therapy of choice.

Benign Rolandic Epilepsy

This disorder usually begins in children aged 2-13 years with a peak age of 5 to 8 years. There is a strong family history of epilepsy. The feature is a simple partial motor or somatosensory seizure involving the face and mouth during sleep, producing a typical 'glugging' sound. The child usually wakes from sleep, goes to the parents and is unable to speak and has hemifacial contortions. It may progress to a tonic- clonic seizure. There is a characteristic EEG pattern. The prognosis is excellent as remission usually occurs around puberty. Carbamazepine is the therapy of choice.

Childhood absence epilepsy

These children present with frequent absence seizures (formerly 'petit mal'), often over a hundred daily. Peak age of onset is 5 to 7 years. The absence seizures can be very subtle. Signs include alteration of awareness (usually in the classroom), sudden onset, facial and other automatisms. Juvenile absence epilepsy presents later (n-15 years). First-line treatment is ethosuximide.

Juvenile Myoclonic Epilepsy (myoclonic epilepsy)

This is a triad of seizures: myoclonic jerks, tonic-clonic seizures and absences. Onset is around puberty but may occur earlier. The myoclonic jerks and tonic-clonic seizures usually occur in the early morning after waking. Mental development is usually normal but the disorder is usually life long and is well controlled with sodium valproate.

Medial Temporal Lobe Epilepsy

This syndrome of complex partial seizures, which usually last r to 3 minutes, is seen in childhood. Transient postictal confusion and speech dysfunction is common. Those with medically intractable seizures respond well to surgery.

Complex partial seizures are those seizures in which consciousness is clouded and the patient does not recall the complete seizure which differs from simple partial seizures as the patient will be able to recall the complete episode of the seizure.  

According to WHO around 50 million people worldwide have epilepsy. Nearly 80% of the people with epilepsy are found in developing regions. Epilepsy responds to treatment about 70% of the time, yet about three fourths of affected people in developing countries do not get the treatment they need.

The risk of premature death in people with epilepsy is two to three times higher than it is for the general population. The social stigma and discrimination related to epilepsy is also a real problem in developing countries and requires early intervention at an early age.(Reprint)