Subacute Sclerosing Panencephalitis (SSPE) in a 10 ½ Year Old Male Child

Abstract

Subacute sclerosing panencephalitis (SSPE) is a progressive neurological disorder of childhood and early adolescence, caused by persistent defective measles virus. A 10 ½ yearold male child with h/o having normal milestones till the age of 15 months had an episode of measles for which child was hospitalized. After one year he showed gradual deterioration of already attained milestones but continued regression of milestones noticed, presented to the department at 10½ years with h/o not getting up from the bed. Patients usually have behavioural changes, myoclonus, dementia, visual disturbances, and pyramidal and extrapyramidal signs and can cause death within 1-3 years of presentations. The diagnosis is based upon characteristic clinical manifestations, the presence of characteristic periodic EEG discharges, and demonstration of raised antibody titre against measles in the plasma and cerebrospinal fluid. Treatment for SSPE is being researched. A combination of oral Isoprinosine and intraventricular interferon alfa appears to be the best effective treatment. Patients responding to treatment need to receive it lifelong. At present effective measles vaccination is the only solution to SSPE.