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NEUROLOGY 1983;33:994
© 1983 American Academy of Neurology

Non-infantile neuronopathic Gaucher's disease

A clinicopathologic study

Marc D. Winkelman, MD, Betty Q. Banker, MD, Maurice Victor, MD and Hugo W. Moser, MD

Departments of Pathology and Neurology (Drs. Winkelman, Banker, and Victor), Cleveland Metropolitan General Hospital, Case Western Reserve University School of Medicine, Cleveland, OH, and from the John F. Kennedy Institute and the Department of Neurology (Dr. Moser), the Johns Hopkins University School of Medicine, Baltimore, MD.

Two siblings with Gaucher's disease developed a chronic, slowly progressive neurologic disorder in early adult life. Stimulus-sensitive myoclonus, generalized seizures, supranuclear gaze palsies, and cerebellar ataxia were the main clinical features. Autopsy disclosed perivascular Gaucher cells in the brain, and nerve cell loss and neuronophagia in the brainstem, cerebellum, and spinal cord. From these observations and the literature, there seem to be three clinically distinct neurologic syndromes in Gaucher's disease, depending on the age at which symptoms begin, but the neu-ropathologic changes are essentially the same in all of them. A modified classification of Gaucher's disease, based on these observations, is proposed.

Address correspondence and reprint requests to Dr. Winkelman, Department of Pathology, Cleveland Metropolitan General Hospital, 3395 Scrantm Road, Cleveland, OH 44109.

Accepted for publication December 9, 1982.




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