Treatment of subacute sclerosing panencephalitis: an overview.

Abstract

Subacute sclerosing panencephalitis (SSPE) is a rare central nervous system degenerative disease that occurs primarily in children and adolescents. It is believed to be caused by a measles-like virus. Initial symptoms usually present as a variety of personality changes followed by myoclonus with progression of mental and motor deterioration, which leads to death within a few months to years. New experimental treatment with inosiplex has been shown to be helpful for patients stricken with this progressive neurological disease. A response to inosiplex therapy is best in patients with a slowly progressing form of the disease. Inosiplex treatment is safe with few adverse effects. The duration of treatment appears to be lifelong since many patients relapse when inosiplex therapy is discontinued. This article reviews the etiology, pathogenesis, and experimental treatment of SSPE.