Incontinence in individuals with Angelman syndrome: a comparative study.
Frequency and type of incontinence and variables associated with incontinence were assessed in individuals with Angelman syndrome (AS; n=71) and in a matched control group (n=69) consisting of individuals with non-specific intellectual disability (ID). A Dutch version of the "Parental Questionnaire: Enuresis/Urinary Incontinence" (Beetz, von Gontard, & Lettgen, 1994) was administered and information on primary caretakers' perspectives regarding each individual's incontinence was gathered. Results show that diurnal incontinence and fecal incontinence during the day more frequently occurred in the control group than in the AS group. In both groups, nocturnal enuresis was the most common form of incontinence. More incontinence was seen in individuals with AS who were younger, had a lower level of adaptive functioning and/or had epilepsy. Individuals with AS were able to stay dry for longer periods of time than the controls and often showed both in-toilet urination and urinary accidents during the day, whereas accidents and correct voids during the day were more set apart in the control group. Also, persons with AS had a lower micturition frequency implying possible voiding postponement. Both groups showed high rates of LUTS (lower urinary tract symptoms) possibly indicative of functional bladder disorders such as voiding postponement, dysfunctional voiding, or even an underactive bladder. In general, most primary caretakers reported severe intellectual disability as the main cause for urinary incontinence. Based on these results incontinence does not appear to be part of the behavioral phenotype of Angelman syndrome. Therefore, pediatric or urologic diagnostics and treatment are recommended for all persons with incontinence and intellectual disability. Further implications for practice and research are given.
Department of Special Education, Radboud University Nijmegen, The Netherlands; Daelzicht, Heel, The Netherlands. Electronic address: m.radstaake@pwo.ru.nl.
Maartje
M
Radstaake
Robert
R
Didden
Sanne
S
Giesbers
Hubert
H
Korzilius
Nienke
N
Peters-Scheffer
Russell
R
Lang
Alexander
A
von Gontard
Leopold M G
LM
Curfs
D000293:Adolescent; D000328:Adult; D000367:Age Factors; D017204:Angelman Syndrome / Q000150:complications*; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D004775:Enuresis / Q000150:complications*; D004827:Epilepsy / Q000150:complications; D005242:Fecal Incontinence / Q000150:complications*; D005260:Female; D006801:Humans; D008607:Intellectual Disability / Q000150:complications*; D059411:Lower Urinary Tract Symptoms / Q000150:complications; D008297:Male; D008875:Middle Aged; D053206:Nocturnal Enuresis / Q000150:complications; D011795:Surveys and Questionnaires; D014549:Urinary Incontinence / Q000150:complications*; D055815:Young Adult
Angelman syndrome; Behavioral phenotype; Incontinence; Intellectual disability