Incontinence and psychological symptoms in individuals with Mowat-Wilson Syndrome

Justine Niemczyk*, Stewart Einfeld, David Mowat, Monika Equit, Catharina Wagner, Leopold Curfs, Alexander von Gontard

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

6 Citations (Web of Science)

Abstract

Background: Mowat-Wilson Syndrome (MWS) is caused by deletion/mutation of the ZEB2 gene on chromosome 2q22. MWS is characterized by a distinctive facial appearance, severe intellectual disability and other anomalies, e.g. seizures and/or Hirschsprung disease (HSCR). Most individuals have a sociable demeanor, but one third show psychological problems.

Aims: The aim was to investigate incontinence and psychological problems in MWS.

Methods and procedures: 26 children (4-12 years), 13 teens (13-17 years) and 8 adults (>18 years) were recruited through a MWS support group. The Parental Questionnaire: Enuresis/Urinary Incontinence, as well as the Developmental Behaviour Checklist (DBC) were completed by parents or care-givers.

Outcomes and results: 97.7% of persons with MWS had incontinence (nocturnal enuresis 74.4%; daytime urinary incontinence 76.2%; fecal incontinence 81.4%). Incontinence remained high over age groups (children 95.8%, teens 100%, adults 100%). 46.2% of children, 25% of teens and 37.5% of adults exceeded the clinical cut-off on the DBC. The ability to use the toilet for micturition improved with age.

Conclusions and implications: MWS incontinence rates are very high. All had physical disabilities including anomalies of the genitourinary and gastrointestinal tract. Due to the high prevalence rates, a screening for incontinence and psychological problems in MWS is recommended. (C) 2017 Elsevier Ltd. All rights reserved.

Original languageEnglish
Pages (from-to)230-237
Number of pages8
JournalResearch in Developmental Disabilities
Volume62
DOIs
Publication statusPublished - Mar 2017

Keywords

  • Mowat-Wilson Syndrome
  • Urinary incontinence
  • Fecal incontinence
  • Enuresis
  • Psychopathology
  • ANGELMAN-SYNDROME
  • HIRSCHSPRUNGS-DISEASE
  • URINARY-INCONTINENCE
  • CHILDREN
  • PHENOTYPE
  • FEATURES

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