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Angelman syndrome

Psychological and behavioural characteristics

The information below has been updated September 2007 by Dr O Udwin, Consultant Clinical Child Psychologist, West London Mental Health NHS Trust, London UK and Dr A Kuczynski, Child Clinical Psychologist, South London & Maudsley NHS Trust, London, UK.

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All individuals with Angelman syndrome have severe or profound learning disabilities. Furthermore, they have marked difficulties in their speech and language development. Early prelinguistic babbling and vocal play is often absent. Children typically acquire no more than a few words, and approximately one-third do not talk at all. Their understanding of language may be meaningfully better than their speech, and most use some nonverbal means of communication, including gestures, signs and picture boards.

Hyperactivity and "overexcitability" is common in childhood. Many affected children find it difficult to concentrate during the day and to settle and stay asleep at night. Bedtime may provoke tantrums. The overactivity and sleep problems may decrease with age.

Children and adults are typically described as happy, sociable, and affectionate, and as enjoying physical contact. They display frequent bouts of giggling laughter and hand-clapping or flapping. Although these are often a response to what is going on around them, the humour may at times be inappropriate. Still, individuals with Angelman syndrome may be less prone to irritability and social withdrawal than others with comparable general developmental difficulties. Many of them love music and water, and are fascinated by mirrors and other reflective surfaces.

Many children with Angelman syndrome are able to learn important self-care skills, including feeding and toileting, and as they grow older they may undertake other domestic tasks, such as dusting and setting the table with assistance. However, they all require supervision in their daily lives.

View What are the symptoms? What are the symptoms?  |  Inheritance patterns and prenatal diagnosis View Inheritance patterns and prenatal diagnosis

Medical text written November 1991 by Contact a Family. Approved November 1991 by Professor M Patton, Professor of Medical Genetics, St Georges Hospital Medical School, London, UK and Dr J E Wraith, Consultant Paediatrician, Royal Manchester Children's Hospital, Manchester, UK. Last updated August 2007 by Jill Clayton-Smith, Consultant Clinical Geneticist, St Mary's Hospital, Manchester, UK.

 

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