Deep brain stimulation for primary generalized dystonia: long-term outcomes

Arch Neurol. 2009 Apr;66(4):465-70. doi: 10.1001/archneurol.2009.20.


Background: Pallidal deep brain stimulation (DBS) is the best therapeutic option for patients with disabling primary generalized dystonia (PGD) that is refractory to medications. However, little is known about its long-term effects.

Objective: To describe long-term clinical outcomes in patients with PGD who underwent pallidal DBS.

Design: Case series.

Setting: University hospital.

Patients: Thirty consecutive patients with at least 2 years' follow-up after pallidal DBS for intractable PGD.

Interventions: Pallidal DBS and annual follow-up examinations up to 8 years after DBS implantation.

Main outcome measures: Clinical outcome as measured by changes in the Burke-Fahn-Marsden dystonia scale, incidence and prevalence of adverse events, total electrical energy delivered, and implantable pulse generator longevity.

Results: Twenty-three patients were followed for 3 years, 13 for 4 years, 9 for 5 years, 5 for 6 years, 5 for 7 years, and 1 for 8 years after DBS. Overall improvement at 1 year was maintained in all at successive yearly examinations. There were no intraoperative complications; hardware-related adverse events were infrequent. Rare stimulation-related adverse events primarily affected speech. Implantable pulse generators were replaced every 24 months on average in patients who received initial stimulation at 130-Hz frequency. No battery was replaced, for up to 48 months, in 20 patients initially stimulated using 60 Hz. Clinical outcome did not depend on high energies of stimulation.

Conclusions: Pallidal DBS is a safe and effective treatment for PGD, with improvement sustained for up to 8 years in 1 patient. Low energies of stimulation, although they did not affect clinical outcome, were associated with longer battery life.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Age of Onset
  • Deep Brain Stimulation*
  • Dystonia Musculorum Deformans / genetics
  • Dystonia Musculorum Deformans / physiopathology
  • Dystonia Musculorum Deformans / therapy
  • Dystonic Disorders / diagnosis
  • Dystonic Disorders / genetics
  • Dystonic Disorders / physiopathology
  • Dystonic Disorders / therapy*
  • Female
  • Follow-Up Studies
  • Globus Pallidus / physiopathology
  • Humans
  • Male
  • Middle Aged
  • Molecular Chaperones / genetics
  • Neurologic Examination
  • Neuronavigation
  • Treatment Outcome
  • Young Adult


  • Molecular Chaperones
  • TOR1A protein, human