Bilateral pallidal stimulation for X-linked dystonia parkinsonism

• Main Authors: Wadia, Pettarusp M., Lim, Shen-Yang, Lozano, Andres M., Adams, John R., Poon, Yu-Yan, Diaz, Cristina Torres, Moro, Elena
• Format: Article
• Published: American Medical Association 2010
• Subjects: R Medicine
• Online Access: http://eprints.um.edu.my/12605/; https://jamanetwork.com/journals/jamaneurology/fullarticle/1106588

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.