Clinico-etiological Profile of Epileptic Syndromes in Infants and Early Childhood

Epilepsy is the most common disabling neurological problem among children worldwide, and has a varying prevalence and etiological profile across the life-cycle. Onset of epilepsy prior to two-year age is more common than later in childhood.

It more commonly has a symptomatic cause and poor long-term outcome with respect to seizure-control and cognition. Previous work from India on epilepsy in infants has either focused on a single etiology, on one particular epilepsy syndrome, or on single seizure rather than epilepsy.

A recent prospective, population-based study showed an incidence of 75 per 100 000 live births prior to 6 months and 62 per 100 000 between 6 and 12 months, considerably higher than previous estimates from retrospective studies. These population-based studies are from high-resource countries, and it is noteworthy that acquired epilepsies have a higher incidence in resource-limited populations.

Traditionally, syndromes have been defined primarily by electroclinical features; however, in the last two decades, gene discovery in the epilepsies has allowed cohorts of cases with a shared genetic etiology to be studied. Consistent electroclinical phenotypes have emerged, with examples including CDKL5, MeCP2, PCDH19, STXBP1, and inv dup 15.

An epileptic syndrome is "an epileptic disorder characterised by a cluster of signs and symptoms customarily occurring together; these include such items as the type of seizure, aetiology, anatomy, precipitating factors, age of onset, severity, chronicity, diurnal and circadian cycling, and sometimes prognosis. However, in contradistinction to a disease, a syndrome does not necessarily have a common aetiology and prognosis.