title: Mixed results for GPi-DBS in the treatment of cranio-facial and cranio-cervical  dystonia symptoms.
abstract: The aim of the study is to determine clinical outcomes in patients undergoing Globus Pallidus Internus Deep Brain Stimulation (GPi-DBS) for cranio-facial and cranio-cervical dystonia (Meige) symptoms. A total of 6 patients seen between 2002 and 2010 with cranio-facial and cranio-cervical dystonia symptoms were identified from the University of Florida Institutional Review Board approved database. Patients were videotaped using a standardized protocol, and tapes were randomized and blindly reviewed by a movement disorders neurologist. The Unified Dystonia Rating Scale improved 31.6 ± 23.2% (range: 3.4-63.2%) at 6 months and 63.7 ± 35.3% (range: 6.3-100%) at 12 months. The Burke-Fahn-Marsden Dystonia Rating Scale improved 45.3 ± 29.5% (range: 4.7-75.0%) at 6 months and 61.8 ± 30.9% (range: 16.6-100%) at 12 months. One patient significantly had a very large improvement with little evidence of residual dystonia. Blepharospasm improved in all patients, whereas speech and swallowing did not improve in this cohort. Two patients improved with unilateral GPi-DBS, although one required a contralateral DBS later in the disease course. Two patients were managed with low frequency stimulation (<100 Hz). Two patients had less than 20% benefit. GPi-DBS for cranio-facial and cranio-cervical symptoms is an effective strategy to manage a subset of patients who remain unresponsive to optimized medical management. Unilateral stimulation may be an option for some patients, but it remains unclear whether response to single-sided stimulation will be sustainable. The mixed results of this GPi-DBS case series highlight the need for a careful re-examination of selection criteria, alternative brain targets, and possibly rescue leads for patients who are non-responders to the GPi target.
authors.affiliation: Department of Neurology, Center for Movement Disorders and Neurorestoration, University of Florida, Gainesville, FL 32611, USA.
authors.forename: Natlada
authors.initials: N
authors.lastname: Limotai
authors.forename: Criscely
authors.initials: C
authors.lastname: Go
authors.forename: Genko
authors.initials: G
authors.lastname: Oyama
authors.forename: Nelson
authors.initials: N
authors.lastname: Hwynn
authors.forename: Theresa
authors.initials: T
authors.lastname: Zesiewicz
authors.forename: Kelly
authors.initials: K
authors.lastname: Foote
authors.forename: Roongroj
authors.initials: R
authors.lastname: Bhidayasiri
authors.forename: Irene
authors.initials: I
authors.lastname: Malaty
authors.forename: Pam
authors.initials: P
authors.lastname: Zeilman
authors.forename: Ramon
authors.initials: R
authors.lastname: Rodriguez
authors.forename: Michael S
authors.initials: MS
authors.lastname: Okun
mesh_terms: D000328:Adult; D000368:Aged; D046690:Deep Brain Stimulation; D005260:Female; D005500:Follow-Up Studies; D005917:Globus Pallidus; D006801:Humans; D008297:Male; D008538:Meige Syndrome / Q000628:therapy*; D008875:Middle Aged; D012189:Retrospective Studies; D016896:Treatment Outcome