A GREEK-AMERICAN KINDRED WITH AUTOSOMAL-DOMINANT, LEVODOPA-RESPONSIVEPARKINSONISM AND ANTICIPATION

Interest is increasing concerning the role of genetic factors in the e tiology of Parkinson's disease. We report the analysis of a Greek-Amer ican kindred with levodopa-responsive parkinsonism. Of the 98 individu als present in six generations of this pedigree, 16 individuals in thr ee successive generations have developed parkinsonism. Affected member s were examined both in Greece and in the United States. The clinical presentation consisted of asymmetric rigidity, resting tremor, bradyki nesia, and postural instability, and symptoms were responsive to levod opa, The disease appears to be inherited in an autosomal dominant mann er, The inheritance pattern and the development of parkinsonism in suc cessive generations on two continents challenges environmental factors as the primary cause in the pathogenesis of parkinsonism in this kind red, Anticipation is present in this pedigree. The affected members in the third generation developed symptoms at ages 50 to 71, in the four th at ages 40 to 55, and in the fifth at age 31 years. This is another example of a neurodegenerative disease with autosomal dominant inheri tance and anticipation. A molecular genetic analysis of this pedigree is in progress.