Differential response of dystonia and parkinsonism following globus pallidus internus deep brain stimulation in X-linked dystonia-parkinsonism (Lubag).
BACKGROUND
X-linked dystonia-parkinsonism (XDP; DYT3; Lubag) is an adult-onset hereditary progressive dystonia/parkinsonism which is typically minimally responsive to pharmacological treatment.


METHODS
We report a 63- year-old man with a diagnosis of XDP who underwent bilateral globus pallidus internus deep brain stimulator (GPi-DBS) placement. His course initially began with right hand tremor and dystonia at age 57 and progressed to also include bradykinesia and rigidity. The patient tolerated the procedure without significant complications. GPi-DBS improved his right hand dystonia, but did not significantly improve his parkinsonism.


CONCLUSIONS
DBS may be a therapeutic option for select cases of XDP, but its specific indications must be carefully discussed, as the available cases have had mixed responses. Whether other targets may be more effective is not known.
Oyama
Genko
G
Department of Neurology, University of Florida College of Medicine/Shands Hospital, Movement Disorders Center, McKnight Brain Institute, Gainesville, FL 32610, USA.
Fernandez
Hubert H
HH
Foote
Kelly D
KD
Zeilman
Pam
P
Hwynn
Nelson
N
Jacobson
Charles E
CE
Malaty
Irene A
IA
Rodriguez
Ramon L
RL
Okun
Michael S
MS
D046690:Deep Brain Stimulation; D004421:Dystonia / Q000628:therapy*; D040181:Genetic Diseases, X-Linked / Q000628:therapy*; D005917:Globus Pallidus / Q000601:surgery*; D006801:Humans; D008297:Male; D008875:Middle Aged; D020734:Parkinsonian Disorders / Q000628:therapy*; D016896:Treatment Outcome