AUTOSOMAL-DOMINANT PARKINSONISM WITH BENIGN COURSE AND TYPICAL LEWY-BODY PATHOLOGY

The few previously reported patients with familial parkinsonism and Le wy-body pathology in the substantia nigra displayed a variety of clini cal and pathologic syndromes. We now describe a family with very slowl y progressive Parkinson's disease (PD) that has, in most cases, respon ded 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 occurre d in three other generations. There has been a trend toward progressiv ely younger age of onset. One autopsied case showed a distribution of cell loss and Lewy bodies typical of PD. The hereditary pattern is mos t compatible with autosomal dominance. This kindred's illness shows th at a presumably single Mendelian dominant gene can cause the clinical and pathologic features of PD, and further extends the clinical spectr um of pathologically typical Lewy-body PD.