Bilateral pallidal deep brain stimulation for X-linked dystonia-parkinsonism

World Neurosurg. 2014 Jul-Aug;82(1-2):241.e1-4. doi: 10.1016/j.wneu.2013.09.039. Epub 2013 Sep 25.

Abstract

Background: X-linked dystonia-parkinsonism (XDP) is a progressively debilitating movement disorder that begins with focal dystonia and eventually generalizes. It exclusively affects Filipino inhabitants of the island of Panay. We report a case of XDP successfully treated by deep brain stimulation (DBS) and review the literature.

Methods: A 36-year-old man with XDP failed medical management and underwent bilateral globus pallidus internus DBS. A search of the PubMed database was performed to identify all articles discussing DBS and XDP. "Stimulation," "DYT3," "Lubag," "torsion dystonia," and "dystonia-parkinsonism" were used as MeSH headings.

Results: The patient's postoperative course was notable for delayed emergence from anesthesia. When stimulation was started, he had immediate improvement in his symptoms, and at 6-month follow-up, he is able to ambulate with the assistance of a walker. Review of the literature revealed 5 previously reported cases of XDP treated with DBS.

Conclusions: The published experience with globus pallidus internus DBS for XDP has been very positive to date. Although long-term follow-up data are needed, early results provide optimism for patients with this debilitating disorder.

Keywords: DBS; DYT3; GPi; Pallidal stimulation; X-linked dystonia-parkinsonism.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Deep Brain Stimulation / methods*
  • Dystonic Disorders / etiology
  • Dystonic Disorders / genetics
  • Dystonic Disorders / therapy*
  • Electrodes, Implanted
  • Follow-Up Studies
  • Genetic Diseases, X-Linked / etiology
  • Genetic Diseases, X-Linked / genetics
  • Genetic Diseases, X-Linked / therapy*
  • Globus Pallidus / physiology*
  • Humans
  • Male
  • Muscle Relaxants, Central / therapeutic use
  • Neurologic Examination
  • Neurosurgical Procedures / methods
  • Parkinsonian Disorders / etiology
  • Parkinsonian Disorders / genetics
  • Parkinsonian Disorders / therapy*

Substances

  • Muscle Relaxants, Central

Supplementary concepts

  • Dystonia 3, Torsion, X-Linked