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Late-onset Lafora's disease with typical intraneuronal inclusions
M A Kaufman1, A J Dwork, N J Willson
1Department of Neuropathology and Neurotoxicology, New York State Psychiatric Institute, NY 10032.
Abstract:
We describe a patient with progressive myoclonus epilepsy (late-onset Lafora's disease). Onset was in early adult life, and death was at age fifty-four. The initial symptoms were epileptic seizures and progressive dementia, with later occurrence of myoclonus. Lafora bodies were ubiquitous and in neuronal perikarya in many areas. Dust-like granular bodies predominated in the neuropil of cerebral cortex, substantia nigra, and striatum. Abnormal deposits were also found in the myocardium.
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