本文首次报导经尸检证实的、常染色体显性遗传的帕金森氏病两个大家系。在两家系中共发现41例患者,其中31例分布在家系1(调查8代共246人)的连续4代成员中;10例分布在家系2(调查6代共232人)的连续3代成员中。两家系都是在1890年至1920年从意大利南部的一个小山村迁入美国。作者对两家系上溯至17世纪中叶,未发现两家系间有血缘关系。 41例患者中男性23例,女性18例,平均 This article reports for the first time by autopsy confirmed autosomal dominant Parkinson’s disease two major families. A total of 41 patients were found in the two families, of which 31 cases were distributed in 4 consecutive generations in family 1 (a total of 246 surveyed in 8 generations) and 10 consecutive generations in 3 (family 6) in. Both families moved to the United States from 1890 to 1920 from a small mountain village in southern Italy. The authors date the two families back to the mid-seventeenth century and found no relationship between the two families. Among the 41 patients, 23 were male and 18 were female, on average