title: Late-onset Lafora's disease with typical intraneuronal inclusions.
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.
authors.affiliation: Department of Neuropathology and Neurotoxicology, New York State Psychiatric Institute, NY 10032.
authors.forename: M A
authors.initials: MA
authors.lastname: Kaufman
authors.forename: A J
authors.initials: AJ
authors.lastname: Dwork
authors.forename: N J
authors.initials: NJ
authors.lastname: Willson
authors.forename: S
authors.initials: S
authors.lastname: John
authors.forename: J D
authors.initials: JD
authors.lastname: Liu
mesh_terms: D001921:Brain / Q000473:pathology*; D002540:Cerebral Cortex / Q000473:pathology; D003342:Corpus Striatum / Q000473:pathology; D004831:Epilepsies, Myoclonic / Q000473:pathology*; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D002479:Inclusion Bodies / Q000648:ultrastructure*; D008875:Middle Aged; D009206:Myocardium / Q000473:pathology; D013378:Substantia Nigra / Q000473:pathology