Subacute sclerosing panencephalitis. Correlation of clinical, neurophysiologic and neuropathologic findings.

Abstract

Clinical, neurophysiologic and neuropathologic findings were correlated in five cases of subacute sclerosing panencephalitis (SSPE). In the early stages, the disease chiefly affects the occipital areas, then spreads to the anterior portion of the cerebral hemispheres. Subcortical structures, brain stem, and spinal cord are involved later. Involvement of the brain stem by intranuclear inclusion bodies appears to be an almost constant finding. The EEG complexes typical of early SSPE indicate relative preservation of the cortex. Later, when marked cortical damage occurs, these EEG changes disappear. Neuropathologic findings—neuronaI abnormalities, gIial and inflammatory changes, demyelination, and inclusion bodies—most likely are dependent on the clinical stage at the time of brain biopsy or autopsy.