title: Antero-ventral internal pallidum stimulation improves behavioral disorders in Lesch-Nyhan disease.
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.
authors.affiliation: CHRU Montpellier, Service de Neurochirurgie, Montpellier, France.
authors.forename: Laura
authors.initials: L
authors.lastname: Cif
authors.forename: Brigitte
authors.initials: B
authors.lastname: Biolsi
authors.forename: Sophie
authors.initials: S
authors.lastname: Gavarini
authors.forename: Aude
authors.initials: A
authors.lastname: Saux
authors.forename: Santiago Gil
authors.initials: SG
authors.lastname: Robles
authors.forename: Cornel
authors.initials: C
authors.lastname: Tancu
authors.forename: Xavier
authors.initials: X
authors.lastname: Vasques
authors.forename: Philippe
authors.initials: P
authors.lastname: Coubes
mesh_terms: D000293:Adolescent; D001526:Behavioral Symptoms / Q000209:etiology / Q000628:therapy*; D004599:Electric Stimulation Therapy / Q000379:methods*; D005917:Globus Pallidus / Q000503:physiopathology / Q000528:radiation effects*; D006801:Humans; D007926:Lesch-Nyhan Syndrome / Q000150:complications; D008297:Male