Adaptive behaviour in Angelman syndrome: its profile and relationship to age.
BACKGROUND
Angelman syndrome (AS) is a neurodevelopmental disorder usually caused by an anomaly in the maternally inherited chromosome 15. The main features are severe intellectual disability, speech impairment, ataxia, epilepsy, sleep disorder and a behavioural phenotype that reportedly includes happy disposition, attraction to/fascination with water and hypermotoric behaviour.


METHODS
We studied the level of adaptive behaviour and the adaptive behavioural profile in the areas of 'motor skills', 'language and communication', 'personal life skills' and 'community life skills' in a group of 25 individuals with genetically confirmed AS, to determine whether there is a specific adaptive behaviour profile.


CONCLUSIONS
None of the individuals, whatever their chronological age, had reached a developmental age of 3 years. A specific adaptive behaviour profile was found, with 'personal life skills' emerging as relative strengths and 'social and communication skills' as weaknesses.
Brun Gasca
C
C
Universitat Autonoma de Barcelona, Facultat de Psicologia, Bellaterra, Spain. carme.brun@uab.cat
Obiols
J E
JE
Bonillo
A
A
Artigas
J
J
Lorente
I
I
Gabau
E
E
Guitart
M
M
Turk
J
J
D000223:Adaptation, Psychological; D000293:Adolescent; D000367:Age Factors; D017204:Angelman Syndrome / Q000523:psychology*; D002648:Child; D002675:Child, Preschool; D015331:Cohort Studies; D005260:Female; D006801:Humans; D007223:Infant; D008607:Intellectual Disability / Q000523:psychology*; D008297:Male; D010555:Personality Inventory; D012044:Regression Analysis; D012917:Social Adjustment