Movement Disorders (revue)

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Antero‐ventral internal pallidum stimulation improves behavioral disorders in Lesch–Nyhan disease

Identifieur interne : 003170 ( Istex/Corpus ); précédent : 003169; suivant : 003171

Antero‐ventral internal pallidum stimulation improves behavioral disorders in Lesch–Nyhan disease

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

Source :

RBID : ISTEX:FAB3446595192B831E717048AC9CE33BB3E6A43A

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 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

Links to Exploration step

ISTEX:FAB3446595192B831E717048AC9CE33BB3E6A43A

Le document en format XML

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<caption>First sequence. Patient before surgery in his ordinary environment. Several items of the BPI‐1 (Behavior Problem Inventory) are positive. Self‐mutilation behavior with severe lip biting and lip amputation. The hands are protected by gloves and permanently tied to the bed. Aggressive behavior against his environment with kicking/hitting his father. The patient is cruel with his mother during the breakfast. He is spitting the food on his mother.Second sequence. After surgery in the hospital. At rest, the four limbs are not tied to the bed and to the wheelchair, and protection of the fingers by the gloves is not necessary anymore. Bilateral simple manipulations are possible.</caption>
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