Antero-ventral internal pallidum stimulation improves behavioral disorders in Lesch–Nyhan disease
✍ Scribed by Laura Cif; Brigitte Biolsi; Sophie Gavarini; Aude Saux; Santiago Gil Robles; Cornel Tancu; Xavier Vasques; Philippe Coubes
- Book ID
- 102509565
- Publisher
- John Wiley and Sons
- Year
- 2007
- Tongue
- English
- Weight
- 85 KB
- Volume
- 22
- Category
- Article
- ISSN
- 0885-3185
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✦ Synopsis
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