Bilateral pallidal deep brain stimulation for X-linked dystonia-parkinsonism.
BACKGROUND
X-linked dystonia-parkinsonism (XDP) is a progressively debilitating movement disorder that begins with focal dystonia and eventually generalizes. It exclusively affects Filipino inhabitants of the island of Panay. We report a case of XDP successfully treated by deep brain stimulation (DBS) and review the literature.


METHODS
A 36-year-old man with XDP failed medical management and underwent bilateral globus pallidus internus DBS. A search of the PubMed database was performed to identify all articles discussing DBS and XDP. "Stimulation," "DYT3," "Lubag," "torsion dystonia," and "dystonia-parkinsonism" were used as MeSH headings.


RESULTS
The patient's postoperative course was notable for delayed emergence from anesthesia. When stimulation was started, he had immediate improvement in his symptoms, and at 6-month follow-up, he is able to ambulate with the assistance of a walker. Review of the literature revealed 5 previously reported cases of XDP treated with DBS.


CONCLUSIONS
The published experience with globus pallidus internus DBS for XDP has been very positive to date. Although long-term follow-up data are needed, early results provide optimism for patients with this debilitating disorder.
Department of Neurosurgery, Baylor College of Medicine, Houston, Texas, USA.
Akash J
AJ
Patel
Department of Neurology, Baylor College of Medicine, Houston, Texas, USA.
Aliya I
AI
Sarwar
Department of Neurology, Baylor College of Medicine, Houston, Texas, USA.
Joseph
J
Jankovic
Department of Neurosurgery, Baylor College of Medicine, Houston, Texas, USA. Electronic address: ashwinv@bcm.edu.
Ashwin
A
Viswanathan
D000328:Adult; D046690:Deep Brain Stimulation / Q000379:methods*; D020821:Dystonic Disorders / Q000209:etiology / Q000235:genetics / Q000628:therapy*; D004567:Electrodes, Implanted; D005500:Follow-Up Studies; D040181:Genetic Diseases, X-Linked / Q000209:etiology / Q000235:genetics / Q000628:therapy*; D005917:Globus Pallidus / Q000502:physiology*; D006801:Humans; D008297:Male; D009125:Muscle Relaxants, Central / Q000627:therapeutic use; D009460:Neurologic Examination; D019635:Neurosurgical Procedures / Q000379:methods; D020734:Parkinsonian Disorders / Q000209:etiology / Q000235:genetics / Q000628:therapy*
D009125:Muscle Relaxants, Central
DBS; DYT3; GPi; Pallidal stimulation; X-linked dystonia-parkinsonism