Antero-Ventral Internal Pallidum Stimulation Improves Behavioral Disorders in Lesch-Nyhan Disease
Identifieur interne : 004386 ( Main/Merge ); précédent : 004385; suivant : 004387Antero-Ventral Internal Pallidum Stimulation Improves Behavioral Disorders in Lesch-Nyhan Disease
Auteurs : Laura Cif [France] ; Brigitte Biolsi [France] ; Sophie Gavarini [France] ; Aude Saux [France] ; Santiago Gil Robles [France] ; Cornel Tancu [France] ; Xavier Vasques [France] ; Philippe Coubes [France]Source :
- Movement disorders [ 0885-3185 ] ; 2007.
Descripteurs français
- Pascal (Inist)
English descriptors
Abstract
The Lesch-Nyhan syndrome is an X-linked recessive disorder caused by a deficiency in hypoxanthine-guanine phosphoribosyl transferase, a purine salvage enzyme. Affected individuals exhibit a characteristic neuro-behavioral disorder with delayed acquisition of motor skills, dystonia, severe self-mutilations, and aggressive behavior. Deep brain stimulation has been previously proposed for controlling isolated involuntary movements and psychiatric disorders. We applied a double bilateral simultaneous stimulation to limbic and motor internal pallidum in one patient for controlling both behavioral and movement disorders, respectively. The injurious compulsions disappeared; dystonia and dyskinesia were decreased at 28 months follow-up.
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Pascal:08-0069709Le document en format XML
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<sourceDesc><biblStruct><analytic><title xml:lang="en" level="a">Antero-Ventral Internal Pallidum Stimulation Improves Behavioral Disorders in Lesch-Nyhan Disease</title>
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<country>France</country>
<placeName><settlement type="city">Montpellier</settlement>
</placeName>
</affiliation>
</author>
</analytic>
<series><title level="j" type="main">Movement disorders</title>
<title level="j" type="abbreviated">Mov. disord.</title>
<idno type="ISSN">0885-3185</idno>
<imprint><date when="2007">2007</date>
</imprint>
</series>
</biblStruct>
</sourceDesc>
<seriesStmt><title level="j" type="main">Movement disorders</title>
<title level="j" type="abbreviated">Mov. disord.</title>
<idno type="ISSN">0885-3185</idno>
</seriesStmt>
</fileDesc>
<profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Behavioral disorder</term>
<term>Dystonia</term>
<term>Nervous system diseases</term>
<term>Pallidum</term>
<term>Self injury</term>
</keywords>
<keywords scheme="Pascal" xml:lang="fr"><term>Pathologie du système nerveux</term>
<term>Trouble du comportement</term>
<term>Dystonie</term>
<term>Pallidum</term>
<term>Automutilation</term>
</keywords>
</textClass>
</profileDesc>
</teiHeader>
<front><div type="abstract" xml:lang="en">The Lesch-Nyhan syndrome is an X-linked recessive disorder caused by a deficiency in hypoxanthine-guanine phosphoribosyl transferase, a purine salvage enzyme. Affected individuals exhibit a characteristic neuro-behavioral disorder with delayed acquisition of motor skills, dystonia, severe self-mutilations, and aggressive behavior. Deep brain stimulation has been previously proposed for controlling isolated involuntary movements and psychiatric disorders. We applied a double bilateral simultaneous stimulation to limbic and motor internal pallidum in one patient for controlling both behavioral and movement disorders, respectively. The injurious compulsions disappeared; dystonia and dyskinesia were decreased at 28 months follow-up.</div>
</front>
</TEI>
<affiliations><list><country><li>France</li>
</country>
<settlement><li>Montpellier</li>
</settlement>
</list>
<tree><country name="France"><noRegion><name sortKey="Cif, Laura" sort="Cif, Laura" uniqKey="Cif L" first="Laura" last="Cif">Laura Cif</name>
</noRegion>
<name sortKey="Biolsi, Brigitte" sort="Biolsi, Brigitte" uniqKey="Biolsi B" first="Brigitte" last="Biolsi">Brigitte Biolsi</name>
<name sortKey="Cif, Laura" sort="Cif, Laura" uniqKey="Cif L" first="Laura" last="Cif">Laura Cif</name>
<name sortKey="Cif, Laura" sort="Cif, Laura" uniqKey="Cif L" first="Laura" last="Cif">Laura Cif</name>
<name sortKey="Coubes, Philippe" sort="Coubes, Philippe" uniqKey="Coubes P" first="Philippe" last="Coubes">Philippe Coubes</name>
<name sortKey="Coubes, Philippe" sort="Coubes, Philippe" uniqKey="Coubes P" first="Philippe" last="Coubes">Philippe Coubes</name>
<name sortKey="Coubes, Philippe" sort="Coubes, Philippe" uniqKey="Coubes P" first="Philippe" last="Coubes">Philippe Coubes</name>
<name sortKey="Gavarini, Sophie" sort="Gavarini, Sophie" uniqKey="Gavarini S" first="Sophie" last="Gavarini">Sophie Gavarini</name>
<name sortKey="Gavarini, Sophie" sort="Gavarini, Sophie" uniqKey="Gavarini S" first="Sophie" last="Gavarini">Sophie Gavarini</name>
<name sortKey="Gil Robles, Santiago" sort="Gil Robles, Santiago" uniqKey="Gil Robles S" first="Santiago" last="Gil Robles">Santiago Gil Robles</name>
<name sortKey="Gil Robles, Santiago" sort="Gil Robles, Santiago" uniqKey="Gil Robles S" first="Santiago" last="Gil Robles">Santiago Gil Robles</name>
<name sortKey="Saux, Aude" sort="Saux, Aude" uniqKey="Saux A" first="Aude" last="Saux">Aude Saux</name>
<name sortKey="Tancu, Cornel" sort="Tancu, Cornel" uniqKey="Tancu C" first="Cornel" last="Tancu">Cornel Tancu</name>
<name sortKey="Tancu, Cornel" sort="Tancu, Cornel" uniqKey="Tancu C" first="Cornel" last="Tancu">Cornel Tancu</name>
<name sortKey="Tancu, Cornel" sort="Tancu, Cornel" uniqKey="Tancu C" first="Cornel" last="Tancu">Cornel Tancu</name>
<name sortKey="Vasques, Xavier" sort="Vasques, Xavier" uniqKey="Vasques X" first="Xavier" last="Vasques">Xavier Vasques</name>
<name sortKey="Vasques, Xavier" sort="Vasques, Xavier" uniqKey="Vasques X" first="Xavier" last="Vasques">Xavier Vasques</name>
</country>
</tree>
</affiliations>
</record>
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