Lateralized effect of pallidal stimulation on self-mutilation in Lesch-Nyhan disease

Taylor J. Abel, Brian D. Dalm, Andrew J. Grossbach, Adam W. Jackson, Teri Thomsen, Jeremy D.W. Greenlee

Research output: Contribution to journalArticle

13 Scopus citations

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.

Original languageEnglish (US)
Pages (from-to)594-597
Number of pages4
JournalJournal of Neurosurgery: Pediatrics
Volume14
Issue number6
DOIs
StatePublished - Dec 2014

Keywords

  • Deep brain stimulation
  • Functional neurosurgery
  • Limbic globus pallidus
  • Surgery
  • Treatment
  • Ventral striatum

ASJC Scopus subject areas

  • Surgery
  • Pediatrics, Perinatology, and Child Health
  • Clinical Neurology

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