Angelman Syndrome

Fact Sheet

What is Angelman Syndrome?

Angelman Syndrome is a complex genetic disorder that primarily affects the nervous system. Characteristic features of this condition include developmental delay, intellectual disability, severe speech impairment, and problems with movement and balance (ataxia). Most affected children also have recurrent seizures (epilepsy) and a small head size (microcephaly). Delayed development becomes noticeable by the age of 6 to 12 months, and other common signs and symptoms usually appear in early childhood.

Children with Angelman Syndrome typically have a happy, excitable demeanor with frequent smiling, laughter, and hand-flapping movements. Hyperactivity and a short attention span are common. Most affected children also have difficulty sleeping and need less sleep than usual. Some affected individuals have unusually fair skin and light-colored hair.

With age, people with Angelman Syndrome become less excitable, and the sleeping problems tend to improve. However, affected individuals continue to have intellectual disability, severe speech impairment, and seizures throughout their lives. Adults with Angelman Syndrome have distinctive facial features that are described as “coarse.” Some also develop an abnormal side-to-side curvature of the spine (scoliosis). The life expectancy of people with this condition appears to be nearly normal.

How is it manifested?

Consistent (100%)

Developmental delay, functionally severe
Speech impairment, no or minimal use of words; receptive and non-verbal communication skills higher than verbal ones
Movement or balance disorder, usually of gait and/or tremulous movement of limbs
Behavioural uniqueness: any combination of frequent laughter/smiling; apparent happy demeanour; easily excitable personality, often with hand flapping movements; hypermotoric behaviour; short attention span

Frequent (more than 80%)

Delayed, disproportionate growth in head circumference, usually resulting in microcephaly (absolute or relative) by age 2
Seizures, onset usually under 3 years of age
Abnormal EEG, characteristic pattern with large amplitude slow-spike waves

Associated (20 – 80%)

Hypopigmented skin and eyes
Tongue thrusting; suck/swallowing disorders
Hyperactive tendon reflexes
Feeding problems during infancy
Uplifted, flexed arms during walking
Prominent mandible
Increased sensitivity to heat
Wide mouth, wide-spaced teeth
Sleep disturbance
Frequent drooling, protruding tongue
Attraction to/fascination with water
Excessive chewing/mouthing behaviors
Flat back of head
Smooth palms

Diagnosis:

Diagnosis is made by noting the characteristic cluster of symptoms (listed below). Careful chromosomal study can reveal abnormalities on Chromosome 15 that are consistent with those identified in Angelman Syndrome.

A history of delayed motor milestones and then later a delay in general development, especially of speech
Unusual movements including fine tremors, jerky limb movements, hand flapping and a wide-based, stiff-legged gait.
A happy disposition with frequent laughter
A deletion or inactivity on chromosome 15
Characteristic facial appearance (but not in all cases)
A history of epilepsy and an abnormal tracing

Prevalance:

Angelman Syndrome affects an estimated 1 in 12,000 to 25, 000 people. Angelman Syndrome occurs in approximately 1 in 15,000 lives births and affects males and females equally.

Additional Resources:

Canadian Angelman Syndrome Society

Po Box 31092
Edmonton AB T5Z 3P3
Phone: 780-860-8603
Web Site: www.angelmancanada.org/
The Canadian Angelman Syndrome Society (CASS) is dedicated to educating parents and professionals about Angelman Syndrome by disseminating information and providing support for parents and caregivers of children with Angelman Syndrome

The Angelman Sydrome Foundation of the USA

414 Plaza Drive, Suite 209, Westmont, IL 60559
Phone: 800-IF-ANGEL (800-432-6435), International Calls: 630-734-9267
Web Site: www.angelman.org/
The Angelman Syndrome Foundation is a national organization of families, caregivers and medical professionals who care about those with Angelman Syndrome. Our mission is to advance the awareness and treatment of Angelman Syndrome through education and information, research, support and advocacy for individuals with Angelman Syndrome, their families, and other concerned parties.

Changing Angry Behaviour

When angry outbursts occur in your classroom, there are a variety of strategies that should be included in your program, such as:

Break the pattern.When possible, record incidents of the angry behavior to look for a pattern, a particular situation, and who the child targets. Break the generalized pattern by creating a structured activity plan (in your head at least) for the child who acts out using inappropriate anger strategies such as screaming, becoming aggressive, etc. Give the child a variety of helpful chores to do (e.g., help bring chairs, help set out activities, help set out snack, set the table, put out cots, wash tables, etc.) Praise the child for all successful activities.
Help the child learn appropriate outlets for anger. Help the child learn to recognize signs of anger or agitation, which lead the child to inappropriate actions. Then, help the child find appropriate outlets for these emotions:
- Use books and personal stories to teach practical anger management techniques
- Use games: circle games that teach impulse control, body management skills, for example, Red Light/Green Light, Freeze Dance, Head and Shoulders, Clapping Pattern Games and games and activities that teach appropriate anger responses, for example, yoga, anger bingo, relaxation techniques
- Role play using puppets or the children themselves
Have Clear Expectations. Use personal stories and visuals to help the child understand the appropriate expected behaviour and the consequences of their inappropriate behaviour. For example, a visual depicting “hands to yourself, no hitting.” Whatever the consequences are for the child, be consistent.
Use a reinforcement schedule. As well as clear consequences for inappropriate behaviour, use a reinforcement chart to reward the child for appropriate behaviour with others. Initially, begin with a short time expectation to promote success. Make sure the reinforcer is highly rewarding for the child. A token economy could also be used where the child receives a larger reward after accumulating a certain number of tokens.
Engage children in cooperative, nurturing games and activities. Make sure the child who has difficulties handling anger is involved in these activities. (Initially, the activities could be introduced at circle time or in large group activities with all the children and then, more of these activities could be used in a small group. The child who struggles with anger is always part of the small group.) An adult must always facilitate these activities. Build a puzzle or structure together. Make a mural or play Cooperative Simon Says. (See Link to “Cooperative Games” for additional strategies.)
Include activities and stories to build empathy in your curriculum. Once again, these activities can be done in the large group and also, emphasized individually with the child who has difficulties managing anger. An adult must facilitate these activities:
- Games to help a child build empathy. For example: emotions lotto, emotions dice, social lotto, Get to Know Your Friend Bingo, Same/Different Activity
- Books
- Worksheets. For example, Same/Different Worksheet, How Would You Feel Worksheet.
- Scripted Role Plays (can use puppets or the children themselves). Make sure the child who has difficulty controlling anger plays the role of a victim to help develop empathic understanding
Engage children in self-esteem building activities. Self-esteem activities are important because children who have issues handling anger appropriately may have low self-esteem. Do activities to help build self-esteem in the child. For example, Friendship Tree, Friendship Quilt, Superhero book and All About Me Activity Book. At group time or using worksheets, do activities to emphasize the strengths of all the children so the child also learns to value peers.

Understanding Person-Directed Planning

A DSTO Information Session Presented by Frances MacNeil, Honey Sherman, and Bill Sherman (Recorded May 2008)

Understanding Capacity, Competency & Consent 3: Property

A Shared Learning Forum Series Workshop with Elaine Atchison, Ministry of the Attorney General, Ontario (Recorded October 26, 2007)

Understanding Capacity, Competency & Consent 2: Personal Care

A Shared Learning Forum Series Workshop with Elaine Atchison, Ministry of the Attorney General, Ontario (Recorded October 26, 2007)

Understanding Capacity, Competency & Consent 1: Introduction

A Shared Learning Forum Series Workshop with Elaine Atchison, Ministry of the Attorney General, Ontario (Recorded October 26, 2007)

Sensory Indications for a Child with Special Needs Part 2

A workshop on Sensory Processing for a child with Special Needs Part 2 – Strategies by Jennifer Radonicich , Occupational Therapist, COTA (June 2008)

Sensory Indications for a Child With Special Needs Part 1

A workshop on Sensory Processing for a child with Special Needs Part 1 by Jennifer Radonicich , Occupational Therapist, COTA (June 2008)

Person Directed Planning

A DSTO Information Session Presented by Ana Vicente and Brian Woodman (May 2010)

Navigating Developmental Services in Toronto

A DSTO Information Session Presented by Karyn Farber, Jennifer Hassard, Craig Shields (Recorded May 2008)

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