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

Title

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

Creator

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

Publisher

Developmental Medicine and Child Neurology

Date

2003

Subject

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

Description

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.

Rights

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).

Citation

Robey K L; Reck J F; Giacomini K D; Barabas G; Eddey G E, “Modes and patterns of self-mutilation in persons with Lesch-Nyhan disease,” Pediatric Palliative Care Library, accessed October 19, 2021, https://pedpalascnetlibrary.omeka.net/items/show/16791.

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