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15:58 - 16:02
ICCS S6-6
(SO)
INCONTINENCE IN PERSONS WITH ANGELMAN SYNDROME
Catharina WAGNER, Justine NIEMCZYK, Monika EQUIT and Alexander VON GONTARD
Saarland University Hospital, Department of Child and Adolescent Psychiatry, Homburg/saar, GERMANY
PURPOSE
Angelman Syndrome (AS) is a congenital syndrome caused by a microdeletion or uniparental disomy on chromosome
15q11-13 with a prevalence of 1:10-20,000. AS is characterized by impairments in intellectual, neurological and motor
functioning, as well as psychological problems. Individuals with AS often have severe intellectual disability, ataxia,
seizures, dysmorphic facies, an inability to speak and a happy, sociable disposition with inappropriate laughter. The aim
of the study was to investigate the rate of incontinence and associated psychological problems in AS.
MATERIAL AND METHODS
90 children (4-18 years) and 54 adults (18-31 years) with AS were recruited through a parent support group (55.6%
male, mean age 15.1 years). The Parental Questionnaire: Enuresis/Urinary Incontinence, the Incontinence
Questionnaire-Pediatric Lower Urinary Tract Symptoms (ICIQ-CLUTS), as well as the Developmental Behavior Checklist
for parents (DBC-P) or for adults (DBC-A) were filled out by parents or care-givers.
RESULTS
85.6% of individuals with AS were affected by at least one subtype of incontinence. 81% had nocturnal enuresis (NE),
61.4% daytime urinary incontinence (DUI) and 53.9% fecal incontinence (FI). The rate of incontinence declined in
adults (74.0% vs. 95.3% in children). 50.6% of the children and 34.1% of adults had a clinically relevant DBC score.
Incontinence was not associated with psychological symptoms.
CONCLUSIONS
Children with AS have high rates of incontinence, which decrease with age. Many adults are still affected by NE, DUI or
even FI. Screening, assessment and treatment of incontinence in children with AS is recommended.