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Botulinum toxin as a novel treatment for self-mutilation in Lesch–Nyhan syndrome

Published online by Cambridge University Press:  15 August 2005

Edward Dabrowski ,
Sarah A Smathers ,
Curt S Ralstrom ,
Michael A Nigro  and
Jimmie P Leleszi
Edward Dabrowski
Affiliation:
Division of Physical Medicine and Rehabilitation, Children's Hospital of Michigan, USA. Department of Pediatrics, School of Medicine, Wayne State University, USA. Michigan Institute for Neurological Disorders, USA.
Sarah A Smathers
Affiliation:
Michigan Institute for Neurological Disorders, USA.
Curt S Ralstrom
Affiliation:
Department of Pediatric Dentistry, Children's Hospital of Michigan, USA.
Michael A Nigro
Affiliation:
Department of Pediatrics, School of Medicine, Wayne State University, USA. Michigan Institute for Neurological Disorders, USA. Division of Neurology, Children's Hospital of Michigan, USA.
Jimmie P Leleszi
Affiliation:
Children's Hospital of Michigan, USA. Department of Psychiatry, School of Medicine, Wayne State University, USA.
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Abstract

Lesch–Nyhan syndrome (LNS) is an X-linked recessive disorder resulting from a deficiency of the metabolic enzyme hypozanthine–guanine phosphoribosyltransferase (HPRT). This syndrome presents with abnormal metabolic and neurological manifestations including hyperuricemia, mental retardation, spastic cerebral palsy (CP), dystonia, and self-mutilation. The mechanism behind the severe self-mutilating behavior exhibited by patients with LNS is unknown and remains one of the greatest obstacles in providing care to these patients. This report describes a 10-year-old male child with confirmed LNS who was treated for self-mutilation of his hands, tongue, and lips with repeated botulinum toxin A (BTX-A) injections into the bilateral masseters. Our findings suggest that treatment with BTX-A affects both the central and peripheral nervous systems, resulting in reduced self-abusive behavior in this patient.

Type
Case Report
Information
Developmental Medicine and Child Neurology , Volume 47 , Issue 9 , September 2005 , pp. 636 - 639
Copyright
© 2005 Mac Keith Press

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