Lesch-Nyhan syndrome: successful prevention of lower lip ulceration caused by self-mutilation by use of mouth guard

Int J Oral Maxillofac Surg. 1994 Feb;23(1):37-8. doi: 10.1016/s0901-5027(05)80324-8.

Abstract

Lesch-Nyhan syndrome (LNS) is a disorder caused by congenital absence of a purine metabolic enzyme, hypoxanthine guanine phosphoribosyl-transferase (HGPRT). This syndrome is characterized clinically by hyperuricemia and neurologic features including choreoathetoid spasticity, self-mutilation, and mental retardation. We report on a patient in whom self-mutilation of the lower lip was suppressed with the help of a mouth guard made from soft resin.

Publication types

  • Case Reports

MeSH terms

  • Humans
  • Infant
  • Lesch-Nyhan Syndrome / therapy*
  • Lip / injuries*
  • Male
  • Mouth Protectors*
  • Self Mutilation / prevention & control*