Self-injury in Lesch-Nyhan disease


Self-injury in Lesch-Nyhan disease


Anderson L T; Ernst M



Journal of Autism and Developmental Disorders




Adolescent; Adult; Aggression/px [Psychology]; Arousal; Child; behavioral problems; Lesch-Nyhan syndrome; trajectory; characteristics; self-injury


UNLABELLED: 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.


Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).


Anderson L T; Ernst M, “Self-injury in Lesch-Nyhan disease,” Pediatric Palliative Care Library, accessed December 9, 2023,