• Laura Cif, Brigitte Biolsi, Sophie Gavarini, Aude Saux, Santiago Gil Robles, Cornel Tancu, Xavier Vasques, and Philippe Coubes.
• CHRU Montpellier, Service de Neurochirurgie, Montpellier, France.
• Mov. Disord. 2007 Oct 31; 22 (14): 2126-9.

AbstractThe 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.(c) 2007 Movement Disorder Society.

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