Antero-Ventral Internal Pallidum Stimulation Improves Behavioral Disorders in Lesch-Nyhan Disease
Identifieur interne : 001796 ( PascalFrancis/Checkpoint ); précédent : 001795; suivant : 001797Antero-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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<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>
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<seriesStmt><title level="j" type="main">Movement disorders</title>
<title level="j" type="abbreviated">Mov. disord.</title>
<idno type="ISSN">0885-3185</idno>
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<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>
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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 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>
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<fA08 i1="01" i2="1" l="ENG"><s1>Antero-Ventral Internal Pallidum Stimulation Improves Behavioral Disorders in Lesch-Nyhan Disease</s1>
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<fA11 i1="01" i2="1"><s1>CIF (Laura)</s1>
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<fA11 i1="04" i2="1"><s1>SAUX (Aude)</s1>
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<fA11 i1="05" i2="1"><s1>GIL ROBLES (Santiago)</s1>
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<fA11 i1="06" i2="1"><s1>TANCU (Cornel)</s1>
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<fA11 i1="07" i2="1"><s1>VASQUES (Xavier)</s1>
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<fA11 i1="08" i2="1"><s1>COUBES (Philippe)</s1>
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<fA14 i1="01"><s1>CHRU Montpellier, Service de Neurochirurgie</s1>
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<fA14 i1="03"><s1>Université de Montpellier I, CNRS UMR5203, Institut de Génomique Fonctionnelle</s1>
<s2>Montpellier</s2>
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<sZ>1 aut.</sZ>
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<fC01 i1="01" l="ENG"><s0>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.</s0>
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<fC02 i1="03" i2="X"><s0>002B17A03</s0>
</fC02>
<fC03 i1="01" i2="X" l="FRE"><s0>Pathologie du système nerveux</s0>
<s5>01</s5>
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<fC03 i1="01" i2="X" l="ENG"><s0>Nervous system diseases</s0>
<s5>01</s5>
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<fC03 i1="01" i2="X" l="SPA"><s0>Sistema nervioso patología</s0>
<s5>01</s5>
</fC03>
<fC03 i1="02" i2="X" l="FRE"><s0>Trouble du comportement</s0>
<s5>02</s5>
</fC03>
<fC03 i1="02" i2="X" l="ENG"><s0>Behavioral disorder</s0>
<s5>02</s5>
</fC03>
<fC03 i1="02" i2="X" l="SPA"><s0>Trastorno conducta</s0>
<s5>02</s5>
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<fC03 i1="03" i2="X" l="FRE"><s0>Dystonie</s0>
<s5>03</s5>
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<fC03 i1="03" i2="X" l="ENG"><s0>Dystonia</s0>
<s5>03</s5>
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<fC03 i1="03" i2="X" l="SPA"><s0>Distonía</s0>
<s5>03</s5>
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<fC03 i1="04" i2="X" l="FRE"><s0>Pallidum</s0>
<s5>09</s5>
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<fC03 i1="04" i2="X" l="ENG"><s0>Pallidum</s0>
<s5>09</s5>
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<fC03 i1="04" i2="X" l="SPA"><s0>Pallidum</s0>
<s5>09</s5>
</fC03>
<fC03 i1="05" i2="X" l="FRE"><s0>Automutilation</s0>
<s5>10</s5>
</fC03>
<fC03 i1="05" i2="X" l="ENG"><s0>Self injury</s0>
<s5>10</s5>
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<fC03 i1="05" i2="X" l="SPA"><s0>Automutilación</s0>
<s5>10</s5>
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<s5>37</s5>
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<s5>38</s5>
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<s5>39</s5>
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<s5>39</s5>
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<s5>44</s5>
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<s5>46</s5>
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<fN21><s1>035</s1>
</fN21>
<fN44 i1="01"><s1>OTO</s1>
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<affiliations><list><country><li>France</li>
</country>
<settlement><li>Montpellier</li>
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</list>
<tree><country name="France"><noRegion><name sortKey="Cif, Laura" sort="Cif, Laura" uniqKey="Cif L" first="Laura" last="Cif">Laura Cif</name>
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<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>
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<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>
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