Movement Disorders (revue)

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Antero-Ventral Internal Pallidum Stimulation Improves Behavioral Disorders in Lesch-Nyhan Disease

Identifieur interne : 004386 ( Main/Merge ); précédent : 004385; suivant : 004387

Antero-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 :

RBID : Pascal:08-0069709

Descripteurs français

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:08-0069709

Le document en format XML

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</placeName>
</affiliation>
<affiliation wicri:level="1">
<inist:fA14 i1="02">
<s1>INSERM, U661</s1>
<s2>Montpellier</s2>
<s3>FRA</s3>
<sZ>1 aut.</sZ>
<sZ>3 aut.</sZ>
<sZ>5 aut.</sZ>
<sZ>6 aut.</sZ>
<sZ>7 aut.</sZ>
<sZ>8 aut.</sZ>
</inist:fA14>
<country>France</country>
<placeName>
<settlement type="city">Montpellier</settlement>
</placeName>
</affiliation>
<affiliation wicri:level="1">
<inist:fA14 i1="03">
<s1>Université de Montpellier I, CNRS UMR5203, Institut de Génomique Fonctionnelle</s1>
<s2>Montpellier</s2>
<s3>FRA</s3>
<sZ>1 aut.</sZ>
<sZ>3 aut.</sZ>
<sZ>6 aut.</sZ>
<sZ>7 aut.</sZ>
<sZ>8 aut.</sZ>
</inist:fA14>
<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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