The prevalence and phenomenology of repetitive behavior in genetic syndromes

Title

The prevalence and phenomenology of repetitive behavior in genetic syndromes

Creator

Moss J; Oliver C; Arron K; Burbidge C; Berg K

Publisher

Journal of Autism and Developmental Disorders

Date

2009

Subject

Middle Aged; Male; Young Adult; Surveys and Questionnaires; Child; Humans; Adult; Adolescent; Prevalence; Female; Child Preschool; Phenotype; Syndrome; London; Stereotyped Behavior; Angelman Syndrome/epidemiology/psychology; Cri-du-Chat Syndrome/epidemiology/psychology; De Lange Syndrome/epidemiology/psychology; Fragile X Syndrome/epidemiology/psychology; Intellectual Disability/epidemiology/genetics/psychology; Prader-Willi Syndrome/epidemiology/psychology; behavioral problems; tone and motor problems; cri-du-chat; De Lange syndrome; trajectory; characteristics; repetitive behavior

Description

We investigated the prevalence and phenomenology of repetitive behavior in genetic syndromes to detail profiles of behavior. The Repetitive Behaviour Questionnaire (RBQ) provides fine-grained identification of repetitive behaviors. The RBQ was employed to examine repetitive behavior in Angelman (N = 104), Cornelia de Lange (N = 101), Cri-du-Chat (N = 58), Fragile X (N = 191), Prader-Willi (N = 189), Lowe (N = 56) and Smith-Magenis (N = 42) syndromes and individuals with intellectual disability of heterogeneous aetiology (N = 56). Repetitive behavior was variable across syndromes. Fragile X syndrome scored highly on all subscales. Angelman syndrome demonstrated a significantly lowered probability for most behaviors. Prader-Willi, Cri-du-Chat and Smith-Magenis syndrome evidenced unique profiles of repetitive behavior. There is extreme heterogeneity of repetitive behavior across genetic syndromes, highlighting syndrome specific profiles.

Rights

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Citation

Moss J; Oliver C; Arron K; Burbidge C; Berg K, “The prevalence and phenomenology of repetitive behavior in genetic syndromes,” Pediatric Palliative Care Library, accessed March 29, 2024, https://pedpalascnetlibrary.omeka.net/items/show/16753.