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Antero-ventral internal pallidum stimulation improves behavioral disorders in Lesch-Nyhan disease.

Identifieur interne : 001E31 ( Ncbi/Curation ); précédent : 001E30; suivant : 001E32

Antero-ventral internal pallidum stimulation improves behavioral disorders in Lesch-Nyhan disease.

Auteurs : Laura Cif [France] ; Brigitte Biolsi ; Sophie Gavarini ; Aude Saux ; Santiago Gil Robles ; Cornel Tancu ; Xavier Vasques ; Philippe Coubes

Source :

Movement disorders : official journal of the Movement Disorder Society [ 0885-3185 ] ; 2007.

RBID : pubmed:17853483

English descriptors

KwdEn :
- Adolescent, Behavioral Symptoms (etiology), Behavioral Symptoms (therapy), Electric Stimulation Therapy (methods), Globus Pallidus (physiopathology), Globus Pallidus (radiation effects), Humans, Lesch-Nyhan Syndrome (complications), Male.
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.

DOI: 10.1002/mds.21723
PubMed: 17853483

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pubmed:17853483

Le document en format XML

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<affiliation wicri:level="1"><nlm:affiliation>CHRU Montpellier, Service de Neurochirurgie, Montpellier, France.</nlm:affiliation>
<country xml:lang="fr">France</country>
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<author><name sortKey="Biolsi, Brigitte" sort="Biolsi, Brigitte" uniqKey="Biolsi B" first="Brigitte" last="Biolsi">Brigitte Biolsi</name>
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<author><name sortKey="Gavarini, Sophie" sort="Gavarini, Sophie" uniqKey="Gavarini S" first="Sophie" last="Gavarini">Sophie Gavarini</name>
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<author><name sortKey="Saux, Aude" sort="Saux, Aude" uniqKey="Saux A" first="Aude" last="Saux">Aude Saux</name>
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<author><name sortKey="Robles, Santiago Gil" sort="Robles, Santiago Gil" uniqKey="Robles S" first="Santiago Gil" last="Robles">Santiago Gil Robles</name>
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<author><name sortKey="Tancu, Cornel" sort="Tancu, Cornel" uniqKey="Tancu C" first="Cornel" last="Tancu">Cornel Tancu</name>
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<author><name sortKey="Vasques, Xavier" sort="Vasques, Xavier" uniqKey="Vasques X" first="Xavier" last="Vasques">Xavier Vasques</name>
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<author><name sortKey="Coubes, Philippe" sort="Coubes, Philippe" uniqKey="Coubes P" first="Philippe" last="Coubes">Philippe Coubes</name>
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<country xml:lang="fr">France</country>
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<author><name sortKey="Gavarini, Sophie" sort="Gavarini, Sophie" uniqKey="Gavarini S" first="Sophie" last="Gavarini">Sophie Gavarini</name>
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<author><name sortKey="Saux, Aude" sort="Saux, Aude" uniqKey="Saux A" first="Aude" last="Saux">Aude Saux</name>
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<author><name sortKey="Robles, Santiago Gil" sort="Robles, Santiago Gil" uniqKey="Robles S" first="Santiago Gil" last="Robles">Santiago Gil Robles</name>
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<author><name sortKey="Vasques, Xavier" sort="Vasques, Xavier" uniqKey="Vasques X" first="Xavier" last="Vasques">Xavier Vasques</name>
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<author><name sortKey="Coubes, Philippe" sort="Coubes, Philippe" uniqKey="Coubes P" first="Philippe" last="Coubes">Philippe Coubes</name>
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<series><title level="j">Movement disorders : official journal of the Movement Disorder Society</title>
<idno type="ISSN">0885-3185</idno>
<imprint><date when="2007" type="published">2007</date>
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<term>Behavioral Symptoms (etiology)</term>
<term>Behavioral Symptoms (therapy)</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>Male</term>
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<keywords scheme="MESH" qualifier="complications" xml:lang="en"><term>Lesch-Nyhan Syndrome</term>
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<keywords scheme="MESH" qualifier="etiology" xml:lang="en"><term>Behavioral Symptoms</term>
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<keywords scheme="MESH" qualifier="therapy" xml:lang="en"><term>Behavioral Symptoms</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 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.</div>
</front>
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	Movement Disorders (revue)
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Movement Disorders (revue)

Antero-ventral internal pallidum stimulation improves behavioral disorders in Lesch-Nyhan disease.

Antero-ventral internal pallidum stimulation improves behavioral disorders in Lesch-Nyhan disease.

Source :

English descriptors

Abstract

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Le document en format XML

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