Antero-ventral internal pallidum stimulation improves behavioral disorders in Lesch-Nyhan disease.
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.
Cif
Laura
L
CHRU Montpellier, Service de Neurochirurgie, Montpellier, France.
Biolsi
Brigitte
B
Gavarini
Sophie
S
Saux
Aude
A
Robles
Santiago Gil
SG
Tancu
Cornel
C
Vasques
Xavier
X
Coubes
Philippe
P
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