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Department of Neuropathology, (Drs. Cummings and Duchen), Institute of Neurology, National Hospital for Nervous Diseases, London, England, and the Neurobehavior Unit, Brentwood Veterans Administration Medical Center and Department of Neurology (Dr. Cummings), UCLA School of Medicine, Los Angeles, CA.
Address correspondence and reprint requests to Dr. Cummings, Neurobehavior Unit, Ward 208C, VA Medical Center Brentwood, 11301 Wilshire Boulevard, Los Angeles, CA 90073.
The clinical and neuropathologic findings of five cases of Pick disease were studied. All had severe anterior temporal atrophy and abnormal neurons with highly argyrophilic cytoplasm of Pick bodies. The amygdala was abnormal in every case and had severe involvement of all nuclear subdivisions. Behaviorally, the patients exhibited elements of Kluver-Bucy syndrome early in the disease. Language abnormalities were also prominent. Memory and spatial orientation were frequently spared until late. Computed tomography (CT) demonstrated marked lobar atrophy in one of two patients examined. The early appearance of Kluver-Bucy syndrome and the late occurrence of amnesia and spatial disorientation allow clinical identificaiton of this variant of Pick disease.
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