Dopaminergic response in Parkinsonian phenotype of Machado-Joseph disease.
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.
Buhmann
Carsten
C
Department of Neurology, University Hospital Eppendorf, Hamburg, Germany. buhmann@uke.uni-hamburg.de
Bussopulos
Alexandra
A
Oechsner
Matthias
M
D001284:Atrophy / Q000473:pathology; D001479:Basal Ganglia / Q000473:pathology; D001549:Benzamides / Q000493:pharmacokinetics; D002531:Cerebellum / Q000473:pathology; D003287:Contrast Media; D003342:Corpus Striatum / Q000378:metabolism; D018491:Dopamine Agonists / Q000627:therapeutic use*; D006801:Humans; D017827:Machado-Joseph Disease / Q000150:complications / Q000235:genetics*; D008279:Magnetic Resonance Imaging; D008297:Male; D008875:Middle Aged; D009778:Occipital Lobe / Q000378:metabolism; D020734:Parkinsonian Disorders / Q000188:drug therapy* / Q000209:etiology / Q000235:genetics*; D010641:Phenotype; D011149:Pons / Q000473:pathology; D011759:Pyrrolidines / Q000493:pharmacokinetics; D015899:Tomography, Emission-Computed, Single-Photon; D016896:Treatment Outcome
D001549:Benzamides; D003287:Contrast Media; D018491:Dopamine Agonists; D011759:Pyrrolidines; C055743:3-iodo-2-hydroxy-6-methoxy-N-((1-ethyl-2-pyrrolidinyl)methyl)benzamide