title: Chronic bilateral pallidal stimulation in a patient with DYT-1 positive primary generalized dystonia. A long-term follow-up study.
abstract: Primary generalized dystonia is a medically refractory progressive disease of the brain causing near total functional handicap of affected patients. The authors present the effectiveness of bilateral globus pallidus internus (GPi) stimulation in one patient with primary DYT-1 positive dystonia. Pharmacotherapy completely failed to control generalized dystonic movements. The patient was referred for surgical treatment and underwent bilateral implantation of DBS leads in the GPi. The formal objective assessment included the Burke-Fahn-Marsden dystonia rating scale (BFMDRS). Clinical and functional BFMDRS assessments were performed before and after surgery till 5 years postoperatively. All stimulation-induced side effects were reversible. Chronic bilateral pallidal stimulation is an effective and safe treatment in patients with primary generalized DYT-1 positive dystonia. The effectiveness of pallidal stimulation was proved by the objective validated BFMDRS assessment at 5 years follow-up examination.
authors: Klinika Neurochirurgii i Urazów Ooerodkowego Układu Nerwowego CMKP, ul. Kondratowicza 8, 03-242 Warszawa. mrsob@op.pl
authors: Michał
authors: M
authors: Sobstyl
authors: Mirosław
authors: M
authors: Zabek
authors: Henryk
authors: H
authors: Koziara
authors: Sebastian
authors: S
authors: Dzierzecki
mesh_terms: D000328:Adult; D046690:Deep Brain Stimulation / Q000295:instrumentation*; D004421:Dystonia / Q000235:genetics / Q000601:surgery / Q000628:therapy*; D004567:Electrodes, Implanted; D005500:Follow-Up Studies; D005917:Globus Pallidus / Q000503:physiopathology*; D006801:Humans; D008297:Male; D018832:Molecular Chaperones; D019635:Neurosurgical Procedures / Q000379:methods; D016896:Treatment Outcome
chemical_list: D018832:Molecular Chaperones; C108175:TOR1A protein, human