Deep brain stimulation in myoclonus-dystonia syndrome

Laura Cif, Enza Maria Valente, Simone Hemm, Christine Coubes, Nathalie Vayssiere, Stéphanie Serrat, Annalisa Di Giorgio, Philippe Coubes

Research output: Contribution to journalArticlepeer-review


Myoclonus-dystonia syndrome (MDS) is an autosomal dominant disorder characterized by bilateral myoclonic jerks. An 8-year-old boy presenting with early onset, medically intractable, MDS due to a mutation in the ε-sarcoglycan gene (SGCE) underwent chronic bilateral stimulation of the globus pallidus internus, which eliminates both myoclonus and dystonia. We conclude that deep brain stimulation can be an effective and safe treatment for MDS.

Original languageEnglish
Pages (from-to)724-727
Number of pages4
JournalMovement Disorders
Issue number6
Publication statusPublished - Jun 2004


  • Dystonia
  • Myoclonus
  • Neurosurgery

ASJC Scopus subject areas

  • Clinical Neurology
  • Neuroscience(all)


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