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Olivopontocerebellar atrophy

Immunocytochemical and Golgi observations

Neurology Service, Veterans Administration Medical Center, and the Department of Neurology, Albany Medical College, Albany. NY.

Brain tissue was obtained promptly after death from a patient with autosomal dominant olivoponto-cerebellar atrophy and studied by immunocytochemistry and a Golgi technique. Antiglutamic acid decarboxylase showed severe loss of Purkinje cells and their terminals in the dentate nucleus. Stains for neuron-specific enolase (NSE) and microtubule-associated proteins (MAP) confirmed the integrity of the dentate nucleus. Basket and stellate cells revealed secondary changes, but Golgi neurons were intact. Methods for NSE and MAP disclosed dendritic alterations and loss of neurons in the basis pontis and inferior olivary nuclei. Golgi impregnation of Purkinje cells showed loss of major dendrites, paucity of spiny branchlets, and axonal expansions.

Address correspondence and reprint requests to Dr. Koeppen, Neurology Service (127), VA Medical Center, Albany, NY 12208.

^*Deceased.

Supported by the Veterans Administration, the National Ataxia Foundation, and the Alexander von Humboldt Foundation.

Presented in part before the American Association of Neuropathologists, Boston, MA, June 14, 1985.

Accepted for publication March 5, 1986.