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. 2021 May;51(5):1748-1758.
doi: 10.1007/s10803-020-04617-x.

Repetitive and Self-injurious Behaviors in Children with Cornelia de Lange Syndrome

Siddharth Srivastava  1 , Bennett Clark  2 , Colleen Landy-Schmitt  3 , Elizabeth A Offermann  4 , Antonie D Kline  5 , Samuel T Wilkinson  6 , Marco A Grados  7
Affiliations

Repetitive and Self-injurious Behaviors in Children with Cornelia de Lange Syndrome

Siddharth Srivastava et al. J Autism Dev Disord. 2021 May.

Abstract

Cornelia de Lange syndrome (CdLS) is associated with repetitive and self-injurious behaviors (RBs, SIB). Evaluating children with CdLS, this study: (1) characterizes the spectrum of RBs; (2) characterizes the impact and severity of RBs including SIB; (3) describes how age and adaptive functioning relate to RBs including SIB. Fifty children (5-17 years) with CdLS were assessed with Children's Yale-Brown Obsessive Compulsive Scale Modified for PDD; Aberrant Behavior Checklist (ABC); Vineland Adaptive Behaviors Scales (VABS). All children had ≥ 1 type of RB; 44% had some form of SIB. 64% spent > 1 h/day displaying RBs. Lower VABS adaptive functioning was associated with higher stereotypy and SIB scores (ABC). In children with CdLS, RBs including SIB are common, impactful, and associated with lower adaptive functioning.

Keywords: Cornelia de Lange syndrome; Repetitive behaviors; Self-injurious behaviors; Stereotypies.

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