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The substrate for olivopontocerebellar atrophy parkinsonism is obscure due to the lack of clinical and pathological reports and the absence of studies on dopamine receptors in this entity.We describe a patient with olivopontocerebellar atrophy whose clinical presentation was levodopa- responsive parkinsonism in whom pathological examination disclosed pronounced nigral cell loss with no striatal damage.Autoradiographic labeling with 3H-spiperone showed normal densities of D2 dopamine striatal receptors.These data show that indistinguishable nigral,presynaptic parkinsonism occurs in patients with idiopathic Parkinson's disease and in patients with olivopontocerebellar atrophy,and also how a favorable response to levodopa is neither synonymous with idiopathic Parkinson's disease,nor does it exclude multiple-system,atrophy-related parkinsonism. |
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