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The few previously reported patients with familial parkinsonism and Lewy- body pathology in the substantia nigra displayed a variety of clinical and pathologic syndromes.We now describe a family with very slowly progressive Parkinson's disease(PD)that has,in most cases,responded poorly to levodopa and includes subjective visual difficulty.Four personally confirmed cases- with onset at ages 35,25,16 and 16-have occurred in three generations,and four suspicious cases have occurred in three other generations.There has been a trend toward progressively younger age of onset.One autopsied case showed a distribution of cell loss and Lewy bodies typical of PD.The hereditary pattern is most compatible with autosomal dominance.This kindred's illness shows that a presumably single Mendelian dominant gene can cause the clinical and pathologic features of PD,and further extends the clinical spectrum of pathologically typical Lewy-body PD. |
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