We performed a case-control study on 100 patients with Parkinson's dis
ease, their spouses and the same number of sex- and age-matched neurol
ogical controls to clarify if family history of Parkinson's disease or
essential tremor may increase the risk for the disease. We included i
n the study 68 male and 32 female parkinsonian patients with a mean ag
e +/- SD of 62.0 +/- 9.9 years and a mean disease duration of 7.5 +/-
5.7. The odds ratio for familial Parkinson's disease was 13.4 (95% con
fidence limits = 6.5-27.7) and for familial essential tremor 3.1 (95%
confidence limits = 1.5-6.3). We also reviewed the genetic features of
122 parkinsonian patients with at least one affected relative. The pr
esence of secondary cases among both first-degree (n = 83) and less cl
ose relatives (n = 72) suggests that sharing environmental factors doe
s not explain the familial aggregation of the disease. Secondary cases
were significantly more frequent in the paternal than in the maternal
line (70 vs. 39). The presence of secondary cases among both siblings
(46) and parents (37) and the unilateral distribution of ancestral se
condary cases suggest an autosomal dominant inheritance with incomplet
e penetrance.