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

AbstractIdiopathic 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.
AuthorsJill L Ostrem, William J Marks Jr, Monica M Volz, Susan L Heath, Philip A Starr (Affiliation: Department of Neurology, University of California, San Francisco, California 94143, USA. jill.ostrem at ucsf.edu)
JournalMovement disorders : official journal of the Movement Disorder Society (Mov Disord) Vol. 22 Issue 13 Pg. 1885-91 (Oct 15 2007) ISSN: 0885-3185 United States
PMID17618522 (Publication Type: Journal Article, Research Support, N.I.H., Extramural)
Copyright2007 Movement Disorder Society
Topics
  • Adult
  • Aged
  • Deep Brain Stimulation (adverse effects, instrumentation)
  • Electrodes, Implanted
  • Female
  • Follow-Up Studies
  • Globus Pallidus (physiopathology)
  • Humans
  • Male
  • Meige Syndrome (physiopathology, therapy)
  • Microcomputers
  • Middle Aged
  • Neurologic Examination
  • Pilot Projects
  • Pyramidal Tracts (physiopathology)
  • Torticollis (physiopathology, therapy)
  • Treatment Outcome