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Keywords:

  • spinocerebellar ataxia type 2;
  • resting tremor;
  • action tremor;
  • thalamic stimulation

Abstract

We report on a patient with spinocerebellar ataxia type 2 (SCA 2) with an unusual clinical presentation, including severe, disabling resting and action tremor and the successful treatment of this tremor syndrome with chronic thalamic stimulation. Using [123I]β-CIT single photon emission computed tomography, we document a marked degeneration of the nigrostriatal dopaminergic system in SCA 2. © 2002 Movement Disorder Society