Pallidal deep brain stimulation in patients with cranial-cervical dystonia (Meige syndrome)

Mov Disord. 2007 Oct 15;22(13):1885-91. doi: 10.1002/mds.21580.


Idiopathic cranial-cervical dystonia (ICCD) is an adult-onset dystonia syndrome affecting orbicularis oculi, facial, oromandibular, and cervical musculature. ICCD is frequently difficult to treat medically. Deep brain stimulation (DBS) of the globus pallidus internus (GPi) is a highly effective treatment for idiopathic generalized dystonia, however less is known about the effect of GPi DBS on ICCD. In this article, we present the results from a pilot study assessing the effect of GPi DBS in a series of patients with ICCD. Six patients underwent bilateral stereotactic implantation of DBS leads into the sensorimotor GPi. Patients were evaluated with the Burke-Fahn-Marsden dystonia rating scale (BFMDRS) and Toronto western spamodic torticollis rating scale (TWSTRS) before surgery and 6 months postoperatively. At 6 months, patients showed a 72% mean improvement in the BFMDRS total movement score (P < 0.028, Wilcoxin signed rank test). The mean BFMDRS disability score showed a trend toward improvement (P < 0.06). The total TWSTRS score improved 54% (P < 0.043). Despite improvement in dystonia, mild worsening of motor function was reported in previously nondystonic body regions with stimulation in 4 patients. Although GPi DBS was effective in these patients, the influence of GPi DBS on nondystonic body regions deserves further investigation.

Publication types

  • Research Support, N.I.H., Extramural

MeSH terms

  • Adult
  • Aged
  • Deep Brain Stimulation / adverse effects
  • Deep Brain Stimulation / instrumentation*
  • Electrodes, Implanted
  • Female
  • Follow-Up Studies
  • Globus Pallidus / physiopathology*
  • Humans
  • Male
  • Meige Syndrome / physiopathology
  • Meige Syndrome / therapy*
  • Microcomputers
  • Middle Aged
  • Neurologic Examination
  • Pilot Projects
  • Pyramidal Tracts / physiopathology
  • Torticollis / physiopathology
  • Torticollis / therapy
  • Treatment Outcome