Incontinence and psychological symptoms in individuals with Mowat-Wilson Syndrome

Res Dev Disabil. 2017 Mar:62:230-237. doi: 10.1016/j.ridd.2017.01.006. Epub 2017 Jan 13.


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 (>18years) 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.

Keywords: Enuresis; Fecal incontinence; Mowat-Wilson Syndrome; Psychopathology; Urinary incontinence.

MeSH terms

  • Adolescent
  • Adult
  • Child
  • Child, Preschool
  • Comorbidity
  • Enuresis / epidemiology*
  • Enuresis / physiopathology
  • Facies
  • Fecal Incontinence / epidemiology*
  • Fecal Incontinence / physiopathology
  • Female
  • Heart Defects, Congenital / epidemiology
  • Hirschsprung Disease / epidemiology*
  • Hirschsprung Disease / physiopathology
  • Hirschsprung Disease / psychology
  • Humans
  • Intellectual Disability / epidemiology*
  • Intellectual Disability / physiopathology
  • Intellectual Disability / psychology
  • Male
  • Mental Disorders / epidemiology*
  • Mental Disorders / psychology
  • Microcephaly / epidemiology*
  • Microcephaly / physiopathology
  • Microcephaly / psychology
  • Prevalence
  • Seizures / epidemiology
  • Surveys and Questionnaires
  • Urogenital Abnormalities / epidemiology
  • Urogenital Abnormalities / physiopathology
  • Young Adult

Supplementary concepts

  • Mowat-Wilson syndrome