Angelman Disorder and Other Forms of Autism and Pervasive Developmental Disorder (PDD)

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angelman's disorder

Angelman's Disorder an 'Autistic-like' disorder that most
often has the following characteristics:

Developmental delay, functionally severe
speech impairment, none or minimal use of words
Receptive and non-verbal communication
skills higher than verbal ones
Movement or balance disorder, usually ataxia of gait
and/or tremulous movement of limbs
Behavioral uniqueness: any combination of frequent laughter/smiling; apparent happy demeanor; easily excitable personality, often with hand flapping movements; hypermotoric behavior; short attention span

angel

Frequently, children with Angelman's Disorder also have:

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

Some children with Angelman's Disorder have:

Hypopigmented skin and eyes
Tongue thrusting; suck/swallowing disorders
Overactive 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

Adapted from Williams CA, Angelman H, Clayton-Smith J, Driscoll DJ, Hendrickson JE, Knoll JHM, Magenis RE, Schinzel A, Wagstaff J, Whidden EM, Zori RT. Angelman Syndrome: Consensus for diagnostic criteria. Am J Med Genet. l995;56:237

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