First case of X‐linked dystonia‐parkinsonism (“Lubag”) to demonstrate a response to bilateral pallidal stimulation
Identifieur interne : 003245 ( Istex/Curation ); précédent : 003244; suivant : 003246First case of X‐linked dystonia‐parkinsonism (“Lubag”) to demonstrate a response to bilateral pallidal stimulation
Auteurs : Virgilio Gerald H. Evidente [États-Unis] ; Mark K. Lyons [États-Unis] ; Mark Wheeler [États-Unis] ; Renee Hillman [États-Unis] ; Luann Helepolelei [États-Unis] ; Froukje Beynen [États-Unis] ; Dagmar Nolte [Allemagne] ; Ulrich Müller [Allemagne] ; Philip A. Starr [États-Unis]Source :
- Movement Disorders [ 0885-3185 ] ; 2007-09-15.
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Abstract
“Lubag” or X‐linked dystonia‐parkinsonism (XDP) is a genetic syndrome afflicting Filipino men. Intracranial surgical procedures for Lubag have been unsuccessful. We report a 45‐year‐old Filipino male with genetically confirmed XDP who underwent bilateral pallidal deep brain stimulation (DBS) surgery. The patient started to exhibit improvement on initial programming, most notably of his severe jaw‐opening dystonia. At 1‐year follow‐up, his Burke‐Fahn‐Marsden dystonia score and motor Unified Parkinson's Disease Rating Scale score were improved by 71% and 62%, respectively, with the stimulators on compared to stimulators off state. Bilateral pallidal DBS may be a viable option for Lubag patients with medically refractory symptoms. © 2007 Movement Disorder Society
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DOI: 10.1002/mds.21420
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Starr</name><affiliation wicri:level="1"><mods:affiliation>Department of Neurosurgery, University of San Francisco, San Francisco, California, USA</mods:affiliation><country xml:lang="fr">États-Unis</country><wicri:regionArea>Department of Neurosurgery, University of San Francisco, San Francisco, California</wicri:regionArea></affiliation></author></analytic><monogr></monogr><series><title level="j">Movement Disorders</title><title level="j" type="sub">Official Journal of the Movement Disorder Society</title><title level="j" type="abbrev">Mov. Disord.</title><idno type="ISSN">0885-3185</idno><idno type="eISSN">1531-8257</idno><imprint><publisher>Wiley Subscription Services, Inc., A Wiley Company</publisher><pubPlace>Hoboken</pubPlace><date type="published" when="2007-09-15">2007-09-15</date><biblScope unit="vol">22</biblScope><biblScope unit="issue">12</biblScope><biblScope unit="page" from="1790">1790</biblScope><biblScope unit="page" to="1793">1793</biblScope></imprint><idno type="ISSN">0885-3185</idno></series><idno type="istex">EDD5E1DC747B53D0538FE0947607C2E26710B3A7</idno><idno type="DOI">10.1002/mds.21420</idno><idno type="ArticleID">MDS21420</idno></biblStruct></sourceDesc><seriesStmt><idno type="ISSN">0885-3185</idno></seriesStmt></fileDesc><profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>DYT3</term><term>Lubag</term><term>XDP</term><term>X‐linked dystonia</term><term>X‐linked dystonia parkinsonism</term></keywords></textClass><langUsage><language ident="en">en</language></langUsage></profileDesc></teiHeader><front><div type="abstract" xml:lang="en">“Lubag” or X‐linked dystonia‐parkinsonism (XDP) is a genetic syndrome afflicting Filipino men. Intracranial surgical procedures for Lubag have been unsuccessful. We report a 45‐year‐old Filipino male with genetically confirmed XDP who underwent bilateral pallidal deep brain stimulation (DBS) surgery. The patient started to exhibit improvement on initial programming, most notably of his severe jaw‐opening dystonia. At 1‐year follow‐up, his Burke‐Fahn‐Marsden dystonia score and motor Unified Parkinson's Disease Rating Scale score were improved by 71% and 62%, respectively, with the stimulators on compared to stimulators off state. Bilateral pallidal DBS may be a viable option for Lubag patients with medically refractory symptoms. © 2007 Movement Disorder Society</div></front></TEI></record>Pour manipuler ce document sous Unix (Dilib)
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