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From the Department of Neurology (Drs. Ohta, Tsuji, and Miruno), Jichi Medical School, Minamikawachi-machi, Kawachi-gun, Tochigi-ken 32904, Japan; the Department of Neurolngy (Drs. Atsumi and Miyatake), Brain Research Institute, Niigata University, Asahimachi-dori, Niigata 951, Japan; and the Department of Internal Medicine (Dr. Yahagi), Yarnagata Central Hospital, Yamagata 990, Japan.
GM1 gangliosidosis is usually a pediatric disease caused by hereditary acid P-galactosidase deficiency. There have been a few cases in adults. We saw a 51-year-old Japanese man with type 3 GM1 gangliosidosis who was manifesting dementia, dysarthria, gait disturbance, and limb rigidity. Radiologic studies showed platyspondylia, acetabular hypoplasia, and flattened femoral heads. Biochemical analysis revealed generalized acid P-galactosidase deficiency.
Address correspondence and reprint requests to Dr. Ohta. Department of Neurology, Jichi Medical School, Minamikawachi-machi. Kawachi-gun, Tochigiken. Japan 329-04.
Accepted for publication January 31,1985.
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