Modes and patterns of self-mutilation in persons with Lesch-Nyhan disease

Modes and patterns of self-mutilation in persons with Lesch-Nyhan disease

Robey K L; Reck J F; Giacomini K D; Barabas G; Eddey G E

Developmental Medicine and Child Neurology

2003

Male; Surveys and Questionnaires; Child; Humans; Adult; Adolescent; Female; Child Preschool; Infant; Anticonvulsants/therapeutic use; Carbamazepine/therapeutic use; Lesch-Nyhan Syndrome/genetics; Chromosomes Human X/genetics; Diazepam/therapeutic use; Self-Injurious Behavior/diagnosis/drug therapy; behavioral problems; Lesch-Nyhan syndrome; trajectory; characteristics; self-injury

Lesch-Nyhan disease (LND) is a rare X-linked recessive genetic disorder associated with cognitive impairment, choreoathetosis, hyperuricemia, and the hallmark symptom of severe and involuntary self-mutilation. This study examines data gathered from a survey of 64 families in the USA and abroad regarding the self-injury of their family members who have LND. The individuals with LND ranged in age from 1 to 40 years (mean 16 years 7 months, SD 11 years 2 months) and, with the exception of one, were males. The most common initial mode of self-mutilation, and the most frequently cited past or current behavior, was biting of lips and/or fingers. Other behaviors, in order of frequency, included head banging, extension of arms when being wheeled through doorways, tipping of wheelchairs, eye-poking, fingers in wheelchair spokes, and rubbing behaviors. Hierarchical cluster analysis identified patterns of association among the types of self-mutilation. Modes of self-mutilation in which external surfaces (such as a wheelchair component) served as instruments of self-injury tended to co-occur, as did biting of lips and fingers.

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