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Behaviour problems in Angelman syndrome

J A Summers1, D B Allison, P S Lynch

  • 1Kennedy Krieger Institute, Baltimore, MD, USA.

Journal of Intellectual Disability Research : JIDR
|April 1, 1995
PubMed
Summary
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Behavioral issues are common in Angelman syndrome (AS), a genetic disorder affecting neurological and intellectual function. Early identification and treatment of these problems can improve adaptive functioning in individuals with AS.

Area of Science:

  • Genetics
  • Neurodevelopmental Disorders
  • Behavioral Science

Background:

  • Angelman syndrome (AS) is a rare genetic disorder caused by a deletion on chromosome 15.
  • AS is characterized by significant neurological, motor, and intellectual impairments.
  • While behavioral problems are noted in AS, their developmental trajectory and outcomes require further investigation.

Purpose of the Study:

  • To investigate the nature and prevalence of behavioral problems in individuals with Angelman syndrome.
  • To understand the developmental course and impact of these behaviors.

Main Methods:

  • A comprehensive review of published case reports on Angelman syndrome.
  • A survey administered to parents of a small cohort (n=11) of children diagnosed with AS.

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Main Results:

  • Behavioral problems were consistently observed across all ages and genders in individuals with AS.
  • Common issues included language deficits, excessive laughter, hyperactivity, attention deficits, feeding and sleep disturbances, aggression, noncompliance, object mouthing, tantrums, and stereotyped behaviors.
  • Data from case reports and parent surveys corroborated the high prevalence of these behavioral challenges.

Conclusions:

  • Behavioral problems are a significant and pervasive feature of Angelman syndrome.
  • Identifying and addressing severe behavioral issues may lead to improved adaptive functioning and quality of life for individuals with AS.