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NEUROLOGY 1982;32:1295
© 1982 American Academy of Neurology

Adult dystonic lipidosis

Clinical, histologic, and biochemical findings of a neurovisceral storage disease

W. T. Longstreth, Jr., M.D., Joel R. Daven, M.D., Donald F. Farrell, M.D., John W. Bolen, M.D. and Thomas D. Bird, M.D.

Division of Neurology (Drs. Longstreth, Daven, Farrrell, and Bird) and Department of Pathology (Dr. Bolen), University of Washington School of Medicine, Seattle, WA, and Department of Neurology (Dr. Bird), Veterans Administration Medical Center, Seattle, WA.

A 43-year-old man presented with splenomegaly and a 20-year history of a neurological disorder that included vertical supranuclear ophthalmologic, mild dementia, and a movement disorder. Adult dystonic lipid sis was diagnosed from the clinical picture and demonstration of foamy and sea-blue histiocytes in bone marrow. Ultrastructural patterns in cytolysosomes suggested accumulation of neutral fat and phospholipids. Liver content of this monoacylglycerol) phosphate was increased, probably because the number of liposome had increased. Sphingomyelinase activity was normal in cultured skin fibroblasts. Juvenile and adult dystonic lipidosis form a clinically, histological, and biochemically distinct neurovisceral storage disease that differs from Niemann-Pick disease.

Address correspondence and reprint requests to Dr. Longstreth, Division of Neurology, RG-20, University of Washington, Seattle, WA 98195.

Dr. Longstreth was a Fellow in the Robert Wood Johnson Clinical Scholars Program during completion of this work.

Accepted for publication April 8, 1982.




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