DISEASE SCANNER

Global Incurable Diseases Tracker

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Genetic Disorder

Sturge-Weber Syndrome

HIGH SEVERITY

A rare congenital neurological and skin disorder characterized by a facial port-wine birthmark, neurological abnormalities including seizures, and eye problems such as glaucoma. Caused by somatic mutation in GNAQ gene. Affects approximately 1 in 20,000-50,000 people.

Global Affected

200.0K

Countries

30

Symptoms

Port-wine stain (face)
Seizures
Developmental delays
Intellectual disability
Glaucoma
Increased pressure in eye
Buphthalmos (enlarged eye)
Stroke-like episodes
Hemiparesis (weakness)
Headaches
Behavioral problems
Visual field defects
Limb overgrowth

Treatment Options

Laser therapy (pulse dye laser)
Antiepileptic drugs
Low-dose aspirin
Glaucoma medications
Glaucoma surgery
Hemispherectomy (severe cases)
Physical therapy
Occupational therapy
Speech therapy
Special education
Eye drops
Surgical procedures
Behavioral therapy

Risk Factors

1Somatic GNAQ gene mutation
2Occurs sporadically
3Not inherited
4No racial predilection
5No gender predilection
6Occurs before birth

Diagnostic Methods

  • 1Clinical examination
  • 2MRI of brain (leptomeningeal enhancement)
  • 3CT scan (calcifications)
  • 4Eye pressure measurement
  • 5EEG
  • 6Genetic testing (rarely needed)
  • 7Neuropsychological testing

Prognosis

Variable. Seizures often refractory. Intellectual disability common. Glaucoma manageable with treatment. Quality of life affected by neurological issues. Life expectancy variable. Early intervention improves outcomes. Lifelong management required. Visual impairment possible.

Prevention

  • No prevention possible (somatic mutation)
  • Prenatal diagnosis not possible
  • Early diagnosis and treatment
  • Regular monitoring
  • Seizure management
  • Glaucoma screening
  • Multidisciplinary care

Research Status

Laser therapy for port-wine stain. Antiepileptic drugs for seizures. Low-dose aspirin may help. Glaucoma management. Hemispherectomy in severe cases. Neurological monitoring. Eye exams essential. Multidisciplinary care. Research into topical timolol and sirolimus for skin.

Sources

  • https://www.mayoclinic.org/diseases-conditions/sturge-weber-syndrome
  • https://www.hematology.org/education/patients
  • https://www.ncbi.nlm.nih.gov/books
  • https://rarediseases.org/rare-diseases

Medical Disclaimer

This information is for educational purposes only. Always consult healthcare professionals for medical advice, diagnosis, and treatment.