Lateralized effect of pallidal stimulation on self-mutilation in Lesch-Nyhan disease.
J Neurosurg Pediatr
; 14(6): 594-7, 2014 Dec.
Article
en En
| MEDLINE
| ID: mdl-25303157
ABSTRACT
Lesch-Nyhan disease (LND) is an X-linked hereditary disorder caused by a deficiency of hypoxanthine-guanine phosphoribosyltransferase. This syndrome is characterized by hyperuricemia, self-mutilation, cognitive impairment, and movement disorders such as spasticity and dystonia. The authors describe the case of a 15-year-old boy who underwent bilateral placement of globus pallidus internus (GPi) deep brain stimulation (DBS) electrodes for the treatment of generalized dystonia. His self-mutilating behavior gradually disappeared several weeks after the start of GPi stimulation. The dystonia and self-mutilating behavior returned on the left side only after a right lead fracture. This case is the first reported instance of LND treated with DBS in which the stimulation was interrupted and the self-mutilation returned in a lateralized fashion. The findings indicate that the neurobehavioral aspect of LND is lateralized and that contralateral GPi stimulation is responsible for lateralized improvement in self-injurious behavior.
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Texto completo:
1
Base de datos:
MEDLINE
Asunto principal:
Conducta Autodestructiva
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Estimulación Encefálica Profunda
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Distonía
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Globo Pálido
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Lateralidad Funcional
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Síndrome de Lesch-Nyhan
Idioma:
En
Revista:
J Neurosurg Pediatr
Asunto de la revista:
NEUROCIRURGIA
/
PEDIATRIA
Año:
2014
Tipo del documento:
Article