Two siblings who exhibited hereditary parkinsonism with pyramidal signs and cerebellar ataxia are reported, Anticholinergics had a dramatic beneficial effect in both cases, but levodopa did not. This responsiveness, which is similar to that reported in patients with Joseph's disease, suggests dysfunction of an ''indirect pathway'' involving the globus pallidus and the subthalamic nucleus, in addition to that of the nigrostriatal system. We propose a new hereditary variant of early onset Parkinson's disease, distinct from the levodopa sensitive forms of juvenile Parkinson's disease.
机构:Departments of Pharmacology and Experimental Therapeutics and Psychiatry and the Behavioral Sciences, Johns Hopkins University School of Medicine, Baltimore
COYLE, JT
;
SNYDER, SH
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机构:Departments of Pharmacology and Experimental Therapeutics and Psychiatry and the Behavioral Sciences, Johns Hopkins University School of Medicine, Baltimore
机构:Departments of Pharmacology and Experimental Therapeutics and Psychiatry and the Behavioral Sciences, Johns Hopkins University School of Medicine, Baltimore
COYLE, JT
;
SNYDER, SH
论文数: 0引用数: 0
h-index: 0
机构:Departments of Pharmacology and Experimental Therapeutics and Psychiatry and the Behavioral Sciences, Johns Hopkins University School of Medicine, Baltimore