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
Journal of Child Neurology 2012, 27 (1): 117-20
The purpose of this case report is to review the management of a boy with Lesch-Nyhan syndrome with deep-brain stimulation who had remission of self-injurious behaviors as a result. This patient was treated with 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.

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