Subacute sclerosing panencephalitis. An autopsy case with impaired cellular immunity

An autopsy case of subacute sclerosing panencephalitis (SSPE) in a 5-year-old boy, with rapid progression to a comatose state in 2 weeks after the onset of right hemiplegia, is described. The levels of antibody to measles virus in the serum and the cerebrospinal fluid were increased, and high levels of IgG in the latter were found. A characteristic pattern of electroencephalogram (EEG) showing periodic suppression of high voltage complexes was also found during the course of the disease. Microscopical examination revealed perivascular cuffing, numerous hypertrophied astrocytes with a diffuse gliosis and sporadic intranuclear inclusions in the brain. In addition to these typical findings of SSPE, impaired cellular immunity was recognized by delayed skin test in vivo, and pathologically severe atrophy of thymus, and follicular atrophy of spleen with amyloid deposition in the wall of the sheathed arteries were found.