Dopaminergic response in Parkinsonian phenotype of Machado-Joseph disease

Mov Disord. 2003 Feb;18(2):219-21. doi: 10.1002/mds.10322.

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.

Publication types

  • Case Reports

MeSH terms

  • Atrophy / pathology
  • Basal Ganglia / pathology
  • Benzamides / pharmacokinetics
  • Cerebellum / pathology
  • Contrast Media
  • Corpus Striatum / metabolism
  • Dopamine Agonists / therapeutic use*
  • Humans
  • Machado-Joseph Disease / complications
  • Machado-Joseph Disease / genetics*
  • Magnetic Resonance Imaging
  • Male
  • Middle Aged
  • Occipital Lobe / metabolism
  • Parkinsonian Disorders / drug therapy*
  • Parkinsonian Disorders / etiology
  • Parkinsonian Disorders / genetics*
  • Phenotype
  • Pons / pathology
  • Pyrrolidines / pharmacokinetics
  • Tomography, Emission-Computed, Single-Photon
  • Treatment Outcome

Substances

  • Benzamides
  • Contrast Media
  • Dopamine Agonists
  • Pyrrolidines
  • 3-iodo-2-hydroxy-6-methoxy-N-((1-ethyl-2-pyrrolidinyl)methyl)benzamide