Subacute Sclerosing Panencephalitis (SSPE)

Abstract

IN 1933, Dawson¹ described a rare form of encephalitis which was typified by intranuclear inclusion bodies. Since that time, a number of reports have appeared stressing clinical and neuropathologic features which we now recognize as characteristic. It is a disease of childhood and young adulthood, usually beginning with personality changes, followed by myoclonic seizures, progressive mental and motor disability, leading to death in about six months. The major neuropathologic changes are seen in the cerebral cortex and subjacent white matter and consist of neuronal loss, demyelination, perivascular lymphocytic infiltration and gliosis, in addition to cytoplasmic and nuclear inclusions within neuroglia and neurons. In the past this illness has been reported under a number of terms including Dawson's disease, Van Bogaert's disease, subacute sclerosing leukoencephalitis, nodular panencephalitis, and diffuse encephalitis with sclerosing inflammation of the hemisphere white matter²; it is now called subacute sclerosing panencephalitis (SSPE). Although focal retinal