期刊
MOVEMENT DISORDERS
卷 22, 期 14, 页码 2126-2129出版社
WILEY-LISS
DOI: 10.1002/mds.21723
关键词
Lesch-Nyhan disease; self injury; dystonia; internal pallidum; DBS
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. (C) 2007 Movement Disorder Society.
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