Frequency and type of incontinence and its association with other variables were assessed in females with Rett Syndrome (RS) (n = 63), using an adapted Dutch version of the ‘Parental Questionnaire: Enuresis/Urinary Incontinence’ (Beetz et al. 1994). Also, incontinence in RS was compared to a control group consisting of females with non-specific (mixed) intellectual disability (n = 26). Urinary incontinence (UI) (i.e., daytime incontinence and nocturnal enuresis) and faecal incontinence (FI) were found to be common problems among females with RS that occur in a high frequency of days/nights. UI and FI were mostly primary in nature and occur independent of participants’ age and level of adaptive functioning. Solid stool, lower urinary tract symptoms and urinary tract infections (UTI’s) were also common problems in females with RS. No differences in incontinence between RS and the control group were found, except for solid stool that was more common in RS than in the control group. It is concluded that incontinence is not part of the behavioural phenotype of RS, but that there is an increased risk for solid stool in females with RS.

Despite their intellectual and behavioral challenges, urinary continence can be acquired in AS. Several indications of voiding dysfunctions were found; further research is indicated.

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Skin picking is common in individuals with Prader-Willi Syndrome (PWS) but few treatment studies exist. This study reports the successful functional behavioral assessment (FBA) and treatment of skin picking in a 16-year-old female with PWS. A treatment package based on FBA results consisted of differential reinforcement for alternative and incompatible behavior, selfevaluation of progress, and reinforcement for weekly progress and participation in treatment. Pictures of wounds resulting from skin picking were taken during treatment. A total of 30 pictures were randomly selected and shown in a random order to 16 individuals unfamiliar with the study. The individuals then rated severity of the wounds displayed in the pictures. Pearson's c 2 was used to assess changes in the ratings of severity during the course of treatment, and significant results indicate improvement over the course of intervention, c 2 (1) = 246.6, p < .001. This study adds to the very small literature on treatment of skin picking in people with PWS.

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