Disease Directory Sturge-Weber Syndrome
Neurological

Sturge-Weber Syndrome

Also known as: SWS, encephalotrigeminal angiomatosis, GNAQ somatic mosaic mutation

Prevalence

1-9 per 100,000 (Orphanet)

Onset

Neonatal, Infantile

Type

Somatic mosaic mutation (non-inherited in most cases)

Gene

GNAQ

About Sturge-Weber Syndrome

Sturge-Weber syndrome is caused by a somatic mosaic gain-of-function mutation in GNAQ (p.R183Q), which arises during early embryogenesis and leads to abnormal vascular development. The hallmark is a port-wine birthmark (capillary malformation) on the face, ipsilateral leptomeningeal angiomatosis (brain vascular malformation), and glaucoma. Neurological complications include seizures, stroke-like episodes, focal weakness, and progressive intellectual disability due to brain damage from chronic venous hypertension.

Common Clinical Features

Port-wine birthmark on face (trigeminal distribution) Seizures Glaucoma Hemiplegia Headaches Visual field defects Intellectual disability

Clinical Trial Eligibility Tips

What to know before applying to Sturge-Weber Syndrome trials.

Brain MRI with gadolinium contrast demonstrating leptomeningeal enhancement is required for neurological SWS diagnosis

Seizure onset age and frequency are key baseline measures — prophylactic aspirin use should be documented

Sirolimus and other mTOR/MEK pathway inhibitor trials are emerging — document prior targeted therapy use

Port-wine birthmark distribution (ophthalmic V1 vs. maxillary V2 dermatomes) and glaucoma status affect trial stratification

Patient Resources

Patient Organization

Sturge-Weber Foundation

Visit website ↗

Natural History Registry

Sturge-Weber Foundation Patient Registry

Join registry ↗

Orphanet

European reference resource for rare diseases (ORPHA:3205)

View on Orphanet ↗

NORD

National Organization for Rare Disorders

Search NORD ↗

Find recruiting Sturge-Weber Syndrome trials

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

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