Journal
STEREOTACTIC AND FUNCTIONAL NEUROSURGERY
Volume 88, Issue 5, Pages 329-333Publisher
KARGER
DOI: 10.1159/000319961
Keywords
X-linked dystonia-parkinsonism; Globus pallidus internus; Cognitive impairment; Medication-resistant parkinsonism
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Funding
- Tyler's Hope for a Dystonia Cure Center of Excellence
- US National Parkinson Foundation Center of Excellence
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Background: X-linked dystonia-parkinsonism (XDP; DYT3; Lubag) is an adult-onset hereditary progressive dystonia/parkinsonism which is typically minimally responsive to pharmacological treatment. Case Report: 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. Conclusion: 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. Copyright (C) 2010 S. Karger AG, Basel
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