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 dystoniaand 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 internusDBS. 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 internusDBS 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.