Subacute Sclerosing Panencephalitis

Abstract

Subacute sclerosing panencephalitis (SSPE) is one of the slow virus infections of the CNS. It is a rare disorder, but it is the commonest of the chronic virus infections to affect children. It is caused by measles virus. SSPE is a disease of childhood and adolescence, with an age range of 4–20 years and a peak incidence at 7–10 years. There is a racial difference in incidence of SSPE: in the United States the incidence is four times higher in Whites than in Blacks. Boys outnumber girls by 3 or 2 to 1. Children who acquire measles infection under 2 years of age are more likely to develop SSPE. The mean age of infection is 12–14 months. In exceptional cases SSPE can also occur after live measles immunization, but the rate of incidence is highly reduced after immunization. Since the introduction of measles vaccination, and since coverage in The Netherlands has reached 95%, the incidence of SSPE has gone down to one case annually. In Japan, 10–25 new cases a year are reported.