Bilateral pallidal stimulation for x-linked dystonia parkinsonism.
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).


METHODS
Case report.


METHODS
Tertiary referral center. Patient A 40-year-old Filipino man with genetically confirmed XDP and severely disabling generalized dystonia. Intervention Bilateral GPi DBS.


METHODS
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.


CONCLUSIONS
Bilateral GPi DBS seems to be very effective in improving dystonia in XDP.
Wadia
Pettarusp M
PM
Movement Disorders Centre, Toronto Western Hospital, 399 Bathurst St, McL7-402, Toronto, Ontario, Canada, M5T 2S8. elena.moro@uhn.on.ca.
Lim
Shen-Yang
SY
Lozano
Andres M
AM
Adams
John R
JR
Poon
Yu-Yan
YY
Torres Diaz
Cristina V
CV
Diaz
Cristina Torres
CT
Moro
Elena
E
D000328:Adult; D046690:Deep Brain Stimulation / Q000379:methods*; D004421:Dystonia / Q000150:complications / Q000235:genetics / Q000628:therapy*; D040181:Genetic Diseases, X-Linked / Q000150:complications / Q000628:therapy*; D005917:Globus Pallidus / Q000502:physiology; D006801:Humans; D008297:Male; D020734:Parkinsonian Disorders / Q000150:complications / Q000235:genetics / Q000628:therapy*; D012720:Severity of Illness Index