Subacute spongiform encephalopathy

Abstract

Two cases are presented with a subacute progressive encephalopathy characterized by dementia, aphasia, peculiar hyper-kinesias, and myoclonic movements, and, after four to nine months, terminating in death. The prominent neuropathologic findings are cortical degeneration, especially pronounced in the frontal and the occipital lobes, with aeuronal loss, astrocytic gliosis, and a status spongiosus. On the basis of the clinical, pathologic, and electro-encephalographic findings, the cases are classified as subacute spongiform encephalopathy in accordance with the cases reported by Nevin and associates. The literature is reviewed, with a discussion of classification, differential diagnosis, and etiology.