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NEUROLOGY 1986;36:1323
© 1986 American Academy of Neurology

Klüver-Bucy syndrome and amyotrophic lateral sclerosis

A case report with biochemistry, morphometrics, and Golgi study

D. W. Dickson, MD, D. S. Horoupian, MD, L. J. Thal, MD, P. Davies, PhD, S. Walkley, DVM, PhD and R. D. Terry, MD

Departments of Pathology (Neuropathology) (Drs. Dickson, Horoupian, and Davies) and Neuroscience (Dr. Walkley), Albert Einstein College of Medicine, Bronx, NY; the Department of Neurology (Dr. Thal), VA Medical Center, San Diego, CA; and the Department of Neuroscience (Dr. Terry). University of California at San Diego, San Diego, CA.

We studied a patient with amyotrophic lateral sclerosis and the Klüver-Bucy syndrome. At autopsy there was extensive degeneration of the limbic system with the brunt of the changes in the medial temporal lobe, especially the entorhinal cortex and subiculum. Degenerative changes were also seen in the substantia nigra and lower motor neurons. Morphometric and biochemical studies implied a disease process that affected small, possibly somatostatinergic, cortical neurons. These latter findings and the lobar distribution of cortical atrophy were consistent with Pick's disease, but Pick bodies and ballooned neurons were not present.

Address correspondence and reprint requests to Dr. Dickson, Department of Pathology (Neuropathology), Albert Einstein College of Medicine, K-440,1300 Morris Park Avenue, Bronx, NY 10461.

Accepted for publication February 16, 1986.




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