title: Pallidal deep brain stimulation in patients with cranial-cervical dystonia (Meige syndrome).
abstract: 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.
authors.affiliation: Department of Neurology, University of California, San Francisco, California 94143, USA. jill.ostrem@ucsf.edu
authors.forename: Jill L
authors.initials: JL
authors.lastname: Ostrem
authors.forename: William J
authors.initials: WJ
authors.lastname: Marks
authors.forename: Monica M
authors.initials: MM
authors.lastname: Volz
authors.forename: Susan L
authors.initials: SL
authors.lastname: Heath
authors.forename: Philip A
authors.initials: PA
authors.lastname: Starr
mesh_terms: D000328:Adult; D000368:Aged; D046690:Deep Brain Stimulation / Q000009:adverse effects / Q000295:instrumentation*; D004567:Electrodes, Implanted; D005260:Female; D005500:Follow-Up Studies; D005917:Globus Pallidus / Q000503:physiopathology*; D006801:Humans; D008297:Male; D008538:Meige Syndrome / Q000503:physiopathology / Q000628:therapy*; D008838:Microcomputers; D008875:Middle Aged; D009460:Neurologic Examination; D010865:Pilot Projects; D011712:Pyramidal Tracts / Q000503:physiopathology; D014103:Torticollis / Q000503:physiopathology / Q000628:therapy; D016896:Treatment Outcome