Late-onset Lafora's disease with typical intraneuronal inclusions.
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.
Kaufman
M A
MA
Department of Neuropathology and Neurotoxicology, New York State Psychiatric Institute, NY 10032.
Dwork
A J
AJ
Willson
N J
NJ
John
S
S
Liu
J D
JD
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