Maladaptive behaviors are challenging and a source of stress for caregivers of individuals with Angelman Syndrome (AS). There is limited information on how these maladaptive behaviors vary over time among individuals with AS due to different genetic etiologies. In this study, caregivers of 301 individuals with AS were asked questions about their child's behavior and completed the Aberrant Behavior Checklist-Community version (ABC-C). Developmental functioning was evaluated with either the Bayley Scales of Infant Development, Third Edition (Bayley-III) or the Mullen Scales of Early Learning (MSEL). Family functioning was assessed using the parent-completed Parenting Stress Index (PSI) and the Family Quality of Life questionnaire (FQoL). Approximately 70% of participants had AS due to a deletion on the maternally-inherited copy of chromosome 15q11q13. Results revealed that at baseline, individuals with AS had low scores in the domains of lethargy (mean: 2.6-4.2 depending on genotype) and stereotypy (mean: 2.3-4.2 depending on genotype). Higher cognitive functioning was associated with increased irritability (r = 0.32, p < .01). Hyperactivity (p < .05) and irritability (p < .05) increased with age across all genotypes and should be ongoing targets for both behavioral and pharmacological treatment. Concerns for short attention span were endorsed by more than 70% of caregivers at baseline. Maladaptive behaviors, particularly hyperactivity, irritability and aggression, adversely affected parental stress, and family quality of life. K E Y W O R D S behavior rating scale, behavioral symptoms, developmental disabilities, problem behavior Lynne M. Bird and Wen-Hann Tan contributed equally to the paper.
The preview performance of Julie Bovasso’sAngelo's Weddingon 11 May 1985 imploded after an altercation between the playwright and the staff of Marshall Mason's Off-Broadway Circle Repertory. Bovasso, then almost fifty-five years old, attended the performance against the explicit wishes of the production team; the rehearsal period had been fraught. Suspecting unauthorized cuts, Bovasso took a seat in the audience, but then, midshow, confronted the backstage crew and demanded the chance to give the actors notes. The staff refused. At the start of the third act, Bovasso changed tactics: she alighted the stage and instructed the audience to leave the theatre. Members of the crew blocked her access to the actors, leading to a physical altercation, a 911 call, and, eventually, her forced eviction from the theatre.
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