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Dopaminergic response in Parkinsonian phenotype of Machado-Joseph disease

✍ Scribed by Carsten Buhmann; Alexandra Bussopulos; Matthias Oechsner

Publisher
John Wiley and Sons
Year
2003
Tongue
English
Weight
125 KB
Volume
18
Category
Article
ISSN
0885-3185

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✦ Synopsis

Abstract

We report on a patient with genetically proven Machado‐Joseph Disease (MJD) presenting with signs indistinguishable from Parkinson's disease (PD), including levodopa response and typical levodopa‐induced motor fluctuations. Only after 10 years of prolonged benefit from levodopa and different dopamine agonists (DA), the patient developed cerebellar ataxia and pyramidal signs. Preferential D3‐receptor–stimulating dopamine agonists especially showed a benefit at the time, when D2 receptor binding was reduced in IBZM SPECT. This is the first report of a meaningful response to DA in MJD. © 2002 Movement Disorder Society

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