Pallidal deep-brain stimulation associated with complete remission of self-injurious behaviors in a patient with lesch-nyhan syndrome: A case report

Laura L. Deon, Miriam A. Kalichman, Cynthia L. Booth, Konstantin V. Slavin, Deborah J Gaebler-Spira*

*Corresponding author for this work

Research output: Contribution to journalReview article

20 Scopus citations

Abstract

Thepurposeof this case report is to reviewthemanagement of a boy with Lesch-Nyhan syndromewith deep-brain stimulationwhohad remission of self-injurious behaviors as a result.This patientwas treatedwith intrathecal baclofen and, later, with deep-brain stimulation to reduce hypertonia. Goals were to improve wheelchair positioning for school attendance and to reduce the use of restraints for comfort. Intrathecal baclofen was implanted twice and decreased the hypertonia, but both were explanted because of infection. Deep-brain stimulation was initiated 2.5 years ago, and since that time, comfort and function have improved and caregiver burden has decreased. Improvements in dystonia with deep-brain stimulation have also occurred, and self-injurious behaviors have resolved.

Original languageEnglish (US)
Pages (from-to)117-120
Number of pages4
JournalJournal of Child Neurology
Volume27
Issue number1
DOIs
StatePublished - Jan 1 2012

Keywords

  • Care and comfort
  • Deep-brain stimulation
  • Dystonia
  • Lesch-Nyhan
  • Self-injurious behaviors

ASJC Scopus subject areas

  • Pediatrics, Perinatology, and Child Health
  • Clinical Neurology

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