CEREBELLAR DEGENERATION ASSOCIATED WITH HUMAN-IMMUNODEFICIENCY-VIRUS INFECTION

Cerebellar disorders associated with HIV infection are typically the r esult of discrete cerebellar lesions resulting from opportunistic infe ctions such as toxoplasmosis and progressive multifocal leukoencephalo pathy or primary CNS lymphoma. Clinical symptoms and pathologic abnorm alities related to the cerebellum may also be observed with HIV dement ia. A primary cerebellar degeneration with HN has not previously been reported. Ten patients were identified over an 8-year period at five m edical centers. All patients had clinical, laboratory, and radiologic evaluations, and three had neuropathologic examinations. Patients pres ented with progressively unsteady gait, slurred speech, and limb clums iness. Examination revealed gait ataxia, impaired limb coordination, d ysarthria, and abnormal eye movements. Cognition, strength, and sensor y function remained normal. CD4 lymphocyte counts varied between 10 an d 437 cells/mm(3). Neuroimaging studies showed prominent cerebellar at rophy. Neuropathology showed focal degeneration of the cerebellar gran ular cell layer and unusual focal axonal swellings in the brainstem an d spinal cord. Cultures, histopathology, and immunochemical studies sh owed no conclusive evidence of infection. We report a syndrome of unex plained degeneration of the cerebellum occurring in association with H IV infection.