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Pallidal stimulation relieves myoclonus–dystonia syndrome
  1. C M Magariños-Ascone1,2,
  2. I Regidor2,
  3. J C Martínez-Castrillo3,
  4. M Gómez-Galán1,
  5. R Figueiras-Méndez2
  1. 1Neurología Experimental, Dipartimento di Investigación, Hospital Ramón y Cajal, Madrid, Spain
  2. 2Unidad de Cirugía Funcional, Hospital Ramón y Cajal
  3. 3Neurología, Hospital Ramón y Cajal
  1. Correspondence to:
 Dr Carlos Magariños-Ascone
 Neurología Experimental, Dipartimento di Investigación, Hospital Ramón y Cajal, Madrid 28034, Spain;


A patient with myoclonus–dystonia syndrome was treated by implanting electrodes in the internal segment of the globus pallidus (GPi) and applying deep brain stimulation. Surgery was done in two sessions. The most affected limb was treated first and the other limb one year later. Neuronal recordings showed that most pallidal neurones discharged in bursts at a relatively low firing rate (mean (SD), 46 (18) Hz) compared with cells in the GPi in patients with Parkinson’s disease. Neurones modified the rate and mode of discharge with dystonic postures and rapid involuntary contractions of limb muscles. Neurological examination at 24 months after surgery showed a decline of 47.8% and 78.5% in the Burke–Fahn–Marsden and disability rating scales, respectively.

  • BFM, Burke–Fahn–Marsden scale
  • DBS, deep brain stimulation
  • DRS, disability rating subscale
  • Gpi, internal segment of the globus pallidus
  • myoclonus–dystonia syndrome
  • pallidal stimulation

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  • Competing interests: none declared