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Spinocerebellar ataxia type 2 with levodopa-responsive parkinsonism culminating in motor neuron disease

✍ Scribed by Jon Infante; José Berciano; Victor Volpini; Jordi Corral; José Miguel Polo; Julio Pascual; Onofre Combarros

Publisher: John Wiley and Sons
Year: 2004
Tongue: English
Weight: 865 KB
Volume: 19
Category: Article
ISSN: 0885-3185
DOI: 10.1002/mds.20090

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✦ Synopsis

Abstract

We describe an exceptional spinocerebellar ataxia type 2 (SCA2) phenotype combining cerebellar ataxia, levodopa‐responsive parkinsonism, and motor neuron symptoms. We conclude that motor neuron symptoms and signs may be a striking manifestation in SCA2, masking pre‐existing cerebellar and extrapyramidal semeiology. © 2004 Movement Disorder Society

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## Abstract Among 242 patients with apparently sporadic Parkinson's disease, a 70‐year‐old man with a CAG repeat number of 37 in the __SCA2__ gene was identified. He has remained responsive to levodopa 14 years after onset and has had no overt signs suggesting cerebellar dysfunction. Although it is