Disease Directory Mowat-Wilson Syndrome
Neurological

Mowat-Wilson Syndrome

Also known as: MWS, ZEB2 haploinsufficiency, Hirschsprung disease with intellectual disability

Prevalence

1-9 per 100,000 (Orphanet)

Onset

Infantile, Childhood

Type

Autosomal dominant genetic (de novo in most cases)

Gene

ZEB2

About Mowat-Wilson Syndrome

Mowat-Wilson syndrome is caused by de novo loss-of-function mutations or deletions of ZEB2, encoding Zinc finger E-box binding homeobox 2, a transcriptional repressor critical for neural crest development. Clinical features include distinctive facial appearance (deeply set eyes, prominent columella), moderate-to-severe intellectual disability, absent or severely limited speech, Hirschsprung disease (in ~50%), epilepsy, and structural brain abnormalities including corpus callosum hypoplasia.

Common Clinical Features

Moderate-to-severe intellectual disability Absent or limited speech Hirschsprung disease Epilepsy Distinctive facial features Corpus callosum hypoplasia Short stature

Clinical Trial Eligibility Tips

What to know before applying to Mowat-Wilson Syndrome trials.

ZEB2 pathogenic variant confirmed by sequence analysis and/or deletion/duplication testing is required for trial eligibility

Hirschsprung disease history and surgical intervention status should be documented — GI complications may affect trial eligibility

Seizure type and current antiseizure medication regimen must be stable for a defined period before enrollment

Adaptive behavior and communication assessments are the primary outcome measures in behavioral and pharmacological trials

Patient Resources

Patient Organization

Mowat-Wilson Syndrome Foundation

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Natural History Registry

Mowat-Wilson Syndrome International Registry

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Orphanet

European reference resource for rare diseases (ORPHA:261552)

View on Orphanet ↗

NORD

National Organization for Rare Disorders

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Find recruiting Mowat-Wilson Syndrome trials

Search 500,000+ studies from ClinicalTrials.gov, filtered for Mowat-Wilson Syndrome. Updated daily.

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