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Pathology of olivopontocerebellar atrophy with glutamate dehydrogenase deficiency

From the Neurology and Laboratory Services, Veterans Administration Medical Center, Lyons, NJ; the Department of Neurology, UMDNJ-Rutgers Medical School and the Neurology Service, The Middlesex General University Hospital, New Brunswick, NJ.

We report the neuropathologic findings in the first patient with recognized glutamate dehydrogenase (GDH) deficiency to come to postmortem examination. He had progressive cerebellar ataxia beginning at age 21. He died at age 47 of pulmonary emboli. Postmortem examination revealed pancerebellar, olivary, and mild pontine atrophy, demyelination of the posterior columns, degeneration of anterior horn and dorsal root ganglion cells, and reduction of myelinated fibers in the sural nerve. In addition, there was neuronal storage of lipopigment diffusely throughout the CNS and the autonomic neurons, with cell distention, atrophy, and loss in selected areas.

Address correspondence and reprint requests to Dr. Chokroverty, P.O. Box 308, Lyons, NJ 07939-0500.

Accepted for publication March 8, 1984.