Antero‐ventral internal pallidum stimulation improves behavioral disorders in Lesch–Nyhan disease
Identifieur interne : 003020 ( Main/Curation ); précédent : 003019; suivant : 003021Antero‐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 ] ; 2007-10-31.
Descripteurs français
- Pascal (Inist)
English descriptors
- KwdEn :
- Adolescent, Behavioral Symptoms (etiology), Behavioral Symptoms (therapy), Behavioral disorder, DBS, Dystonia, Electric Stimulation Therapy (methods), Globus Pallidus (physiopathology), Globus Pallidus (radiation effects), Humans, Lesch-Nyhan Syndrome (complications), Lesch–Nyhan disease, Male, Nervous system diseases, Pallidum, Self injury, dystonia, internal pallidum, self injury.
- MESH :
- complications : Lesch-Nyhan Syndrome.
- etiology : Behavioral Symptoms.
- methods : Electric Stimulation Therapy.
- physiopathology : Globus Pallidus.
- radiation effects : Globus Pallidus.
- therapy : Behavioral Symptoms.
- Adolescent, Humans, Male.
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 neurobehavioral 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. © 2007 Movement Disorder Society
Url:
DOI: 10.1002/mds.21723
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<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 neurobehavioral 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. © 2007 Movement Disorder Society</div>
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<s2>Montpellier</s2>
<s3>FRA</s3>
<sZ>1 aut.</sZ>
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</inist:fA14>
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</placeName>
</affiliation>
</author>
<author><name sortKey="Vasques, Xavier" sort="Vasques, Xavier" uniqKey="Vasques X" first="Xavier" last="Vasques">Xavier Vasques</name>
<affiliation wicri:level="1"><inist:fA14 i1="02"><s1>INSERM, U661</s1>
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</inist:fA14>
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<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>
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</inist:fA14>
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</placeName>
</affiliation>
</author>
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</inist:fA14>
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</placeName>
</affiliation>
<affiliation wicri:level="1"><inist:fA14 i1="02"><s1>INSERM, U661</s1>
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</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>
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<sZ>7 aut.</sZ>
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</inist:fA14>
<country>France</country>
<placeName><settlement type="city">Montpellier</settlement>
</placeName>
</affiliation>
</author>
</titleStmt>
<publicationStmt><idno type="wicri:source">INIST</idno>
<idno type="inist">08-0069709</idno>
<date when="2007">2007</date>
<idno type="stanalyst">PASCAL 08-0069709 INIST</idno>
<idno type="RBID">Pascal:08-0069709</idno>
<idno type="wicri:Area/PascalFrancis/Corpus">001466</idno>
<idno type="wicri:Area/PascalFrancis/Curation">001853</idno>
<idno type="wicri:Area/PascalFrancis/Checkpoint">001796</idno>
<idno type="wicri:doubleKey">0885-3185:2007:Cif L:antero:ventral:internal</idno>
<idno type="wicri:Area/Main/Merge">004386</idno>
</publicationStmt>
<sourceDesc><biblStruct><analytic><title xml:lang="en" level="a">Antero-Ventral Internal Pallidum Stimulation Improves Behavioral Disorders in Lesch-Nyhan Disease</title>
<author><name sortKey="Cif, Laura" sort="Cif, Laura" uniqKey="Cif L" first="Laura" last="Cif">Laura Cif</name>
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<s3>FRA</s3>
<sZ>1 aut.</sZ>
<sZ>2 aut.</sZ>
<sZ>4 aut.</sZ>
<sZ>5 aut.</sZ>
<sZ>6 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="02"><s1>INSERM, U661</s1>
<s2>Montpellier</s2>
<s3>FRA</s3>
<sZ>1 aut.</sZ>
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</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>
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<country>France</country>
<placeName><settlement type="city">Montpellier</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Biolsi, Brigitte" sort="Biolsi, Brigitte" uniqKey="Biolsi B" first="Brigitte" last="Biolsi">Brigitte Biolsi</name>
<affiliation wicri:level="1"><inist:fA14 i1="01"><s1>CHRU Montpellier, Service de Neurochirurgie</s1>
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<s3>FRA</s3>
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</inist:fA14>
<country>France</country>
<placeName><settlement type="city">Montpellier</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Gavarini, Sophie" sort="Gavarini, Sophie" uniqKey="Gavarini S" first="Sophie" last="Gavarini">Sophie Gavarini</name>
<affiliation wicri:level="1"><inist:fA14 i1="02"><s1>INSERM, U661</s1>
<s2>Montpellier</s2>
<s3>FRA</s3>
<sZ>1 aut.</sZ>
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<sZ>5 aut.</sZ>
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<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>
<author><name sortKey="Saux, Aude" sort="Saux, Aude" uniqKey="Saux A" first="Aude" last="Saux">Aude Saux</name>
<affiliation wicri:level="1"><inist:fA14 i1="01"><s1>CHRU Montpellier, Service de Neurochirurgie</s1>
<s2>Montpellier</s2>
<s3>FRA</s3>
<sZ>1 aut.</sZ>
<sZ>2 aut.</sZ>
<sZ>4 aut.</sZ>
<sZ>5 aut.</sZ>
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<sZ>8 aut.</sZ>
</inist:fA14>
<country>France</country>
<placeName><settlement type="city">Montpellier</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Gil Robles, Santiago" sort="Gil Robles, Santiago" uniqKey="Gil Robles S" first="Santiago" last="Gil Robles">Santiago Gil Robles</name>
<affiliation wicri:level="1"><inist:fA14 i1="01"><s1>CHRU Montpellier, Service de Neurochirurgie</s1>
<s2>Montpellier</s2>
<s3>FRA</s3>
<sZ>1 aut.</sZ>
<sZ>2 aut.</sZ>
<sZ>4 aut.</sZ>
<sZ>5 aut.</sZ>
<sZ>6 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="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>
</author>
<author><name sortKey="Tancu, Cornel" sort="Tancu, Cornel" uniqKey="Tancu C" first="Cornel" last="Tancu">Cornel Tancu</name>
<affiliation wicri:level="1"><inist:fA14 i1="01"><s1>CHRU Montpellier, Service de Neurochirurgie</s1>
<s2>Montpellier</s2>
<s3>FRA</s3>
<sZ>1 aut.</sZ>
<sZ>2 aut.</sZ>
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</inist:fA14>
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<placeName><settlement type="city">Montpellier</settlement>
</placeName>
</affiliation>
<affiliation wicri:level="1"><inist:fA14 i1="02"><s1>INSERM, U661</s1>
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</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>
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</inist:fA14>
<country>France</country>
<placeName><settlement type="city">Montpellier</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Vasques, Xavier" sort="Vasques, Xavier" uniqKey="Vasques X" first="Xavier" last="Vasques">Xavier Vasques</name>
<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>
<author><name sortKey="Coubes, Philippe" sort="Coubes, Philippe" uniqKey="Coubes P" first="Philippe" last="Coubes">Philippe Coubes</name>
<affiliation wicri:level="1"><inist:fA14 i1="01"><s1>CHRU Montpellier, Service de Neurochirurgie</s1>
<s2>Montpellier</s2>
<s3>FRA</s3>
<sZ>1 aut.</sZ>
<sZ>2 aut.</sZ>
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<sZ>5 aut.</sZ>
<sZ>6 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="02"><s1>INSERM, U661</s1>
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<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>
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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>
</INIST>
<ISTEX><TEI wicri:istexFullTextTei="biblStruct"><teiHeader><fileDesc><titleStmt><title xml:lang="en">Antero‐ventral internal pallidum stimulation improves behavioral disorders in Lesch–Nyhan disease</title>
<author><name sortKey="Cif, Laura" sort="Cif, Laura" uniqKey="Cif L" first="Laura" last="Cif">Laura Cif</name>
</author>
<author><name sortKey="Biolsi, Brigitte" sort="Biolsi, Brigitte" uniqKey="Biolsi B" first="Brigitte" last="Biolsi">Brigitte Biolsi</name>
</author>
<author><name sortKey="Gavarini, Sophie" sort="Gavarini, Sophie" uniqKey="Gavarini S" first="Sophie" last="Gavarini">Sophie Gavarini</name>
</author>
<author><name sortKey="Saux, Aude" sort="Saux, Aude" uniqKey="Saux A" first="Aude" last="Saux">Aude Saux</name>
</author>
<author><name sortKey="Robles, Santiago Gil" sort="Robles, Santiago Gil" uniqKey="Robles S" first="Santiago Gil" last="Robles">Santiago Gil Robles</name>
</author>
<author><name sortKey="Tancu, Cornel" sort="Tancu, Cornel" uniqKey="Tancu C" first="Cornel" last="Tancu">Cornel Tancu</name>
</author>
<author><name sortKey="Vasques, Xavier" sort="Vasques, Xavier" uniqKey="Vasques X" first="Xavier" last="Vasques">Xavier Vasques</name>
</author>
<author><name sortKey="Coubes, Philippe" sort="Coubes, Philippe" uniqKey="Coubes P" first="Philippe" last="Coubes">Philippe Coubes</name>
</author>
</titleStmt>
<publicationStmt><idno type="wicri:source">ISTEX</idno>
<idno type="RBID">ISTEX:FAB3446595192B831E717048AC9CE33BB3E6A43A</idno>
<date when="2007" year="2007">2007</date>
<idno type="doi">10.1002/mds.21723</idno>
<idno type="url">https://api.istex.fr/document/FAB3446595192B831E717048AC9CE33BB3E6A43A/fulltext/pdf</idno>
<idno type="wicri:Area/Istex/Corpus">003170</idno>
<idno type="wicri:Area/Istex/Curation">003170</idno>
<idno type="wicri:Area/Istex/Checkpoint">001B28</idno>
<idno type="wicri:doubleKey">0885-3185:2007:Cif L:antero:ventral:internal</idno>
<idno type="wicri:source">PubMed</idno>
<idno type="RBID">pubmed:17853483</idno>
<idno type="wicri:Area/PubMed/Corpus">002528</idno>
<idno type="wicri:Area/PubMed/Curation">002528</idno>
<idno type="wicri:Area/PubMed/Checkpoint">002977</idno>
<idno type="wicri:Area/Ncbi/Merge">001E31</idno>
<idno type="wicri:Area/Ncbi/Curation">001E31</idno>
<idno type="wicri:Area/Ncbi/Checkpoint">001E31</idno>
<idno type="wicri:doubleKey">0885-3185:2007:Cif L:antero:ventral:internal</idno>
<idno type="wicri:Area/Main/Merge">003F42</idno>
</publicationStmt>
<sourceDesc><biblStruct><analytic><title level="a" type="main" xml:lang="en">Antero‐ventral internal pallidum stimulation improves behavioral disorders in Lesch–Nyhan disease</title>
<author><name sortKey="Cif, Laura" sort="Cif, Laura" uniqKey="Cif L" first="Laura" last="Cif">Laura Cif</name>
<affiliation wicri:level="1"><country xml:lang="fr">France</country>
<wicri:regionArea>CHRU Montpellier, Service de Neurochirurgie, Montpellier</wicri:regionArea>
<placeName><settlement type="city">Montpellier</settlement>
</placeName>
</affiliation>
<affiliation wicri:level="1"><country xml:lang="fr">France</country>
<wicri:regionArea>INSERM, U661, Montpellier</wicri:regionArea>
<placeName><settlement type="city">Montpellier</settlement>
</placeName>
</affiliation>
<affiliation wicri:level="1"><country xml:lang="fr">France</country>
<wicri:regionArea>Université de Montpellier I, CNRS UMR5203, Institut de Génomique Fonctionnelle, Montpellier</wicri:regionArea>
<placeName><settlement type="city">Montpellier</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Biolsi, Brigitte" sort="Biolsi, Brigitte" uniqKey="Biolsi B" first="Brigitte" last="Biolsi">Brigitte Biolsi</name>
<affiliation wicri:level="1"><country xml:lang="fr">France</country>
<wicri:regionArea>CHRU Montpellier, Service de Neurochirurgie, Montpellier</wicri:regionArea>
<placeName><settlement type="city">Montpellier</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Gavarini, Sophie" sort="Gavarini, Sophie" uniqKey="Gavarini S" first="Sophie" last="Gavarini">Sophie Gavarini</name>
<affiliation wicri:level="1"><country xml:lang="fr">France</country>
<wicri:regionArea>INSERM, U661, Montpellier</wicri:regionArea>
<placeName><settlement type="city">Montpellier</settlement>
</placeName>
</affiliation>
<affiliation wicri:level="1"><country xml:lang="fr">France</country>
<wicri:regionArea>Université de Montpellier I, CNRS UMR5203, Institut de Génomique Fonctionnelle, Montpellier</wicri:regionArea>
<placeName><settlement type="city">Montpellier</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Saux, Aude" sort="Saux, Aude" uniqKey="Saux A" first="Aude" last="Saux">Aude Saux</name>
<affiliation wicri:level="1"><country xml:lang="fr">France</country>
<wicri:regionArea>CHRU Montpellier, Service de Neurochirurgie, Montpellier</wicri:regionArea>
<placeName><settlement type="city">Montpellier</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Robles, Santiago Gil" sort="Robles, Santiago Gil" uniqKey="Robles S" first="Santiago Gil" last="Robles">Santiago Gil Robles</name>
<affiliation wicri:level="1"><country xml:lang="fr">France</country>
<wicri:regionArea>CHRU Montpellier, Service de Neurochirurgie, Montpellier</wicri:regionArea>
<placeName><settlement type="city">Montpellier</settlement>
</placeName>
</affiliation>
<affiliation wicri:level="1"><country xml:lang="fr">France</country>
<wicri:regionArea>INSERM, U661, Montpellier</wicri:regionArea>
<placeName><settlement type="city">Montpellier</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Tancu, Cornel" sort="Tancu, Cornel" uniqKey="Tancu C" first="Cornel" last="Tancu">Cornel Tancu</name>
<affiliation wicri:level="1"><country xml:lang="fr">France</country>
<wicri:regionArea>CHRU Montpellier, Service de Neurochirurgie, Montpellier</wicri:regionArea>
<placeName><settlement type="city">Montpellier</settlement>
</placeName>
</affiliation>
<affiliation wicri:level="1"><country xml:lang="fr">France</country>
<wicri:regionArea>INSERM, U661, Montpellier</wicri:regionArea>
<placeName><settlement type="city">Montpellier</settlement>
</placeName>
</affiliation>
<affiliation wicri:level="1"><country xml:lang="fr">France</country>
<wicri:regionArea>Université de Montpellier I, CNRS UMR5203, Institut de Génomique Fonctionnelle, Montpellier</wicri:regionArea>
<placeName><settlement type="city">Montpellier</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Vasques, Xavier" sort="Vasques, Xavier" uniqKey="Vasques X" first="Xavier" last="Vasques">Xavier Vasques</name>
<affiliation wicri:level="1"><country xml:lang="fr">France</country>
<wicri:regionArea>INSERM, U661, Montpellier</wicri:regionArea>
<placeName><settlement type="city">Montpellier</settlement>
</placeName>
</affiliation>
<affiliation wicri:level="1"><country xml:lang="fr">France</country>
<wicri:regionArea>Université de Montpellier I, CNRS UMR5203, Institut de Génomique Fonctionnelle, Montpellier</wicri:regionArea>
<placeName><settlement type="city">Montpellier</settlement>
</placeName>
</affiliation>
</author>
<author><name sortKey="Coubes, Philippe" sort="Coubes, Philippe" uniqKey="Coubes P" first="Philippe" last="Coubes">Philippe Coubes</name>
<affiliation wicri:level="1"><country xml:lang="fr">France</country>
<wicri:regionArea>CHRU Montpellier, Service de Neurochirurgie, Montpellier</wicri:regionArea>
<placeName><settlement type="city">Montpellier</settlement>
</placeName>
</affiliation>
<affiliation wicri:level="1"><country xml:lang="fr">France</country>
<wicri:regionArea>INSERM, U661, Montpellier</wicri:regionArea>
<placeName><settlement type="city">Montpellier</settlement>
</placeName>
</affiliation>
<affiliation wicri:level="1"><country xml:lang="fr">France</country>
<wicri:regionArea>Université de Montpellier I, CNRS UMR5203, Institut de Génomique Fonctionnelle, Montpellier</wicri:regionArea>
<placeName><settlement type="city">Montpellier</settlement>
</placeName>
</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="2007-10-31">2007-10-31</date>
<biblScope unit="vol">22</biblScope>
<biblScope unit="issue">14</biblScope>
<biblScope unit="page" from="2126">2126</biblScope>
<biblScope unit="page" to="2129">2129</biblScope>
</imprint>
<idno type="ISSN">0885-3185</idno>
</series>
<idno type="istex">FAB3446595192B831E717048AC9CE33BB3E6A43A</idno>
<idno type="DOI">10.1002/mds.21723</idno>
<idno type="ArticleID">MDS21723</idno>
</biblStruct>
</sourceDesc>
<seriesStmt><idno type="ISSN">0885-3185</idno>
</seriesStmt>
</fileDesc>
<profileDesc><textClass><keywords scheme="KwdEn" xml:lang="en"><term>Adolescent</term>
<term>Behavioral Symptoms (etiology)</term>
<term>Behavioral Symptoms (therapy)</term>
<term>DBS</term>
<term>Electric Stimulation Therapy (methods)</term>
<term>Globus Pallidus (physiopathology)</term>
<term>Globus Pallidus (radiation effects)</term>
<term>Humans</term>
<term>Lesch-Nyhan Syndrome (complications)</term>
<term>Lesch–Nyhan disease</term>
<term>Male</term>
<term>dystonia</term>
<term>internal pallidum</term>
<term>self injury</term>
</keywords>
<keywords scheme="MESH" qualifier="complications" xml:lang="en"><term>Lesch-Nyhan Syndrome</term>
</keywords>
<keywords scheme="MESH" qualifier="etiology" xml:lang="en"><term>Behavioral Symptoms</term>
</keywords>
<keywords scheme="MESH" qualifier="methods" xml:lang="en"><term>Electric Stimulation Therapy</term>
</keywords>
<keywords scheme="MESH" qualifier="physiopathology" xml:lang="en"><term>Globus Pallidus</term>
</keywords>
<keywords scheme="MESH" qualifier="radiation effects" xml:lang="en"><term>Globus Pallidus</term>
</keywords>
<keywords scheme="MESH" qualifier="therapy" xml:lang="en"><term>Behavioral Symptoms</term>
</keywords>
<keywords scheme="MESH" xml:lang="en"><term>Adolescent</term>
<term>Humans</term>
<term>Male</term>
</keywords>
</textClass>
<langUsage><language ident="en">en</language>
</langUsage>
</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 neurobehavioral 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. © 2007 Movement Disorder Society</div>
</front>
</TEI>
</ISTEX>
</double>
</record>
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