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Progressive dementia and epilepsy in a young adult

Unusual intraneuronal inclusions

Departments of Neurological Surgery and Medicine (Neurology) (Dr. Yerby), the Department of Pathology (Dr. Shaw), University of Washington School of Medicine, Seattle, WA, Portland, OR (Dr. Watson).

A 30-year-old woman had a syndrome of dementia, dystonia, myoclonus, and intention tremor. Brain biopsy showed PAS-positive inclusions of Lafora's disease, but electronmicroscopy demonstrated that the inclusions differed from previously reported Lafora bodies. This may represent a previously undescribed disorder.

Address correspondence and reprint requests to Dr. Yerby, Regional Epilepsy Center, Harborview Medical Center, 325 Ninth Avenue, Seattle, WA 98104.

Supported in part by NIH Grant No. NS17111–2 and the Robert Wood Johnson Clinical Scholars Program.

Accepted for publication April 10, 1985.

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