SLPCohortJournal of autism and developmental disorders2019

Brief Report: Challenging Behaviors in Toddlers and Preschoolers with Angelman, Prader-Willi, and Williams Syndromes.

Wei Siong Neo, Bridgette L Tonnsen

PMID 30542941

WHAT IT FOUND

Parents reported toddlers and preschoolers with Angelman or Williams syndromes had more overall behavior concerns than comparison children, especially attention, withdrawal, and autism spectrum problems.

Prader-Willi toddlers looked similar to comparison children overall.

Key findings

01Children with Angelman syndrome and children with Williams syndrome had greater total behavior problems than comparison children, with elevated somatic complaints, withdrawn behavior, attention problems, depressive problems, autism spectrum problems, and attention deficit/hyperactivity problems; Angelman also showed fewer oppositional defiant problems.

02Children with Prader-Willi syndrome generally had total behavior problems similar to comparison children, except greater withdrawn behavior and autism spectrum problems and fewer sleep and anxiety problems.

03Across syndromes, parents frequently reported acts too young for age and a speech problem, which were uncommon in comparison children; chewing non-edible items was specific to Angelman and Williams syndromes.

STILL TO COME

How it was doneWhat they foundWhat it means for SLPs

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What it does not show

The data are cross-sectional and parent-reported, so they cannot show how these behavior profiles change over time or what causes them. All reports came from mothers, and internalizing behaviors may be harder for parents to rate than externalizing behaviors. The sample was small, especially within each syndrome, and genetic or medical records verified diagnoses for only a majority of children with syndromes. Some behaviors known in older children with these syndromes, such as anxiety in Williams syndrome and compulsive behaviors, sleep problems, or temper tantrums in Prader-Willi syndrome, may not have appeared yet. The CBCL clinical ranges were not developed for these syndromes, so borderline or clinical scores may not capture syndrome-specific patterns. Cognitive functioning was not measured, so differences between syndromes could partly reflect developmental delay or cognition rather than behavior alone.

Declared interests

The authors declared no conflict of interest. The supplied text does not report funding.

The easy way to misread this

Do not use the Child Behavior Checklist scores as syndrome-specific clinical thresholds. The paper says many challenging behaviors were below clinical ranges and clinical norms have not been developed for Angelman, Prader-Willi, or Williams syndrome.

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