title: Incontinence and psychological symptoms in individuals with Mowat-Wilson Syndrome.
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.
  
  
  OBJECTIVE
  The aim was to investigate incontinence and psychological problems in MWS.
  
  
  METHODS
  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.
  
  
  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
  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.
authors.affiliation: Department of Child and Adolescent Psychiatry, Saarland University Hospital, Homburg 66421, Germany. Electronic address: justine.niemczyk@uks.eu.
authors.forename: Justine
authors.initials: J
authors.lastname: Niemczyk
authors.affiliation: Centre for Disability Research and Policy, Brain and Mind Research Institute, University of Sydney, Sydney, NSW, Australia.
authors.forename: Stewart
authors.initials: S
authors.lastname: Einfeld
authors.affiliation: Department of Medical Genetics, Sydney Children's Hospital, NSW, Australia.
authors.forename: David
authors.initials: D
authors.lastname: Mowat
authors.affiliation: Department of Clinical Psychology and Psychotherapy, Saarland University, Saarbruecken, Germany.
authors.forename: Monika
authors.initials: M
authors.lastname: Equit
authors.affiliation: Department of Child and Adolescent Psychiatry, Saarland University Hospital, Homburg 66421, Germany.
authors.forename: Catharina
authors.initials: C
authors.lastname: Wagner
authors.affiliation: Department of Clinical Genetics, Governor Kremers Centre, Maastricht University Medical Centre, Maastricht, The Netherlands.
authors.forename: Leopold
authors.initials: L
authors.lastname: Curfs
authors.affiliation: Department of Child and Adolescent Psychiatry, Saarland University Hospital, Homburg 66421, Germany.
authors.forename: Alexander
authors.initials: A
authors.lastname: von Gontard
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D015897:Comorbidity; D004775:Enuresis / Q000453:epidemiology* / Q000503:physiopathology; D019066:Facies; D005242:Fecal Incontinence / Q000453:epidemiology* / Q000503:physiopathology; D005260:Female; D006330:Heart Defects, Congenital / Q000453:epidemiology; D006627:Hirschsprung Disease / Q000453:epidemiology* / Q000503:physiopathology / Q000523:psychology; D006801:Humans; D008607:Intellectual Disability / Q000453:epidemiology* / Q000503:physiopathology / Q000523:psychology; D008297:Male; D001523:Mental Disorders / Q000453:epidemiology* / Q000523:psychology; D008831:Microcephaly / Q000453:epidemiology* / Q000503:physiopathology / Q000523:psychology; D015995:Prevalence; D012640:Seizures / Q000453:epidemiology; D011795:Surveys and Questionnaires; D014564:Urogenital Abnormalities / Q000453:epidemiology / Q000503:physiopathology; D055815:Young Adult
keywords: Enuresis; Fecal incontinence; Mowat-Wilson Syndrome; Psychopathology; Urinary incontinence