Autosomal Dominant Lewy Body Parkinsinism in a Four-Generation Family


C. H. Waters
Bok Engelsk
Omfang
6 s.
Opplysninger
We report a family with autosomal dominant parkinsonism. The propositus developed parkinsonism at a relatively young age (45 years) and came to autopsy after 6-year illness. She had typical features of Parkinson's disease except for an absence of rest tremor, althought this was present in other family members. A diagnosis of Lewy Body parkinsonism was confirmed by neuropathological examination. Additional pathological features included the present of cortical Lewy Bodies and anti-ubiquitin-positive neurites in the cornu Ammonis 2 and 3 (CA2-3) region of hippocampus. This kindred is similar both clinically and patholocically to a few previously reported pedigrees, futher strengthening recent evidence of genetic etiology of some form of Parkinson's disease.
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