Self-injury in Lesch-Nyhan disease

J Autism Dev Disord. 1994 Feb;24(1):67-81. doi: 10.1007/BF02172213.


Parents of 40 patients with Lesch-Nyhan disease completed a questionnaire detailing developmental history, life course, management, medication, factors influencing variability and topography of self-injury. Several conclusions were reached. Characteristics: Biting was the predominant form, perhaps only because of the difficulty of preventing it. There was considerable variability in self-injury which was strongly related to stress rather than to operant influences. Even though patients could not inhibit self-injury they could predict it and request restraints. Aggression against others was as prevalent as self-injury.

Management: Stress reduction, teeth extraction, and physical restraint were the most commonly used management techniques. Behavior modification was of limited efficacy. Benzodiazepines were the most commonly used medications for controlling self-injury.

Outcome: The severity of self-injury did not change over years. Age of onset was a predictor of outcome. The earlier the age of onset the worse the self-injury eventually became. The discussion describes research strategies, suggests dimensions along which self-injury can be classified, and highlights behavior not commonly described in patients with Lesch-Nyhan disease.

Publication types

  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Adolescent
  • Adult
  • Aggression / psychology
  • Arousal
  • Child
  • Child, Preschool
  • Humans
  • Lesch-Nyhan Syndrome / diagnosis
  • Lesch-Nyhan Syndrome / psychology*
  • Male
  • Parenting / psychology
  • Periodicity
  • Self-Injurious Behavior / classification
  • Self-Injurious Behavior / prevention & control
  • Self-Injurious Behavior / psychology*
  • Social Environment