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  • Title: Bilateral pallidal deep brain stimulation for X-linked dystonia-parkinsonism.
    Author: Patel AJ, Sarwar AI, Jankovic J, Viswanathan A.
    Journal: World Neurosurg; 2014; 82(1-2):241.e1-4. PubMed ID: 24076056.
    Abstract:
    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.
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