Bilateral Pallidal Stimulation for X-Linked Dystonia Parkinsonism

• Published in: Archives of neurology (Chicago) Vol. 67; no. 8; pp. 1012 - 1015
• Main Authors: Wadia, Pettarusp M, Lim, Shen-Yang, Lozano, Andres M, Adams, John R, Poon, Yu-Yan, Diaz, Cristina Torres, Moro, Elena
• Format: Journal Article
• Language: English
• Published: United States American Medical Association 01.08.2010
• Subjects: Adult; Brain; Deep Brain Stimulation - methods; Dystonia - complications; Dystonia - genetics; Dystonia - therapy; Genetic Diseases, X-Linked - complications; Genetic Diseases, X-Linked - therapy; Genetics; Globus Pallidus - physiology; Humans; Male; Neurology; Parkinson's disease; Parkinsonian Disorders - complications; Parkinsonian Disorders - genetics; Parkinsonian Disorders - therapy; Severity of Illness Index
• ISSN: 0003-9942; 2168-6149; 1538-3687; 2168-6157
• DOI: 10.1001/archneurol.2010.187

OBJECTIVE To report the clinical benefits of bilateral deep brain stimulation (DBS) of the globus pallidus internus (GPi) in a patient with X-linked dystonia parkinsonism (XDP). DESIGN Case report. SETTING Tertiary referral center. PATIENT A 40-year-old Filipino man with genetically confirmed XDP and severely disabling generalized dystonia. INTERVENTION Bilateral GPi DBS. MAIN OUTCOME MEASURES The primary outcome measures were the Burke-Fahn-Marsden Dystonia Scale (BFMDS) severity and disability scores, and the secondary outcome measure was the Unified Parkinson Disease Rating Scores. RESULTS At the 1-year postoperative follow-up, there was 80.4% improvement in the BFMDS severity score and 66.7% improvement in the BFMDS disability score. CONCLUSION Bilateral GPi DBS seems to be very effective in improving dystonia in XDP.Arch Neurol. 2010;67(8):1012-1015-->