DISEASE SCANNER

Global Incurable Diseases Tracker

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Genetic Disorder

Friedreich Ataxia

HIGH SEVERITY

The most common inherited ataxia, caused by GAA triplet repeat expansion in FXN gene. Progressive gait ataxia, sensory neuropathy, cardiomyopathy, and diabetes. Autosomal recessive. Onset typically adolescence.

Global Affected

80.0K

Countries

15

Symptoms

Progressive ataxia
Dysarthria
Sensory neuropathy
Cardiomyopathy
Scoliosis
Diabetes mellitus
Vision/hearing loss
Foot deformities

Treatment Options

Genetic counseling
Supportive care
Symptom management
Enzyme replacement (if applicable)
Physical therapy
Regular monitoring
Multidisciplinary care

Risk Factors

1Family history
2Genetic mutations
3Consanguinity
4Advanced paternal age
5Ethnic predisposition

Diagnostic Methods

  • 1Genetic testing
  • 2Clinical evaluation
  • 3Family history assessment
  • 4Specialized laboratory tests
  • 5Imaging studies
  • 6Biopsy (if applicable)

Prognosis

Progressive neuromuscular degeneration. Loss of ambulation 10-15 years after onset. Cardiomyopathy in 60%. Death typically 35-50 years from cardiac failure or aspiration.

Prevention

  • Genetic counseling
  • Carrier screening
  • Prenatal diagnosis
  • Preimplantation genetic diagnosis
  • Family planning

Research Status

Omaveloxolone (Nrf2 activator) FDA-approved 2023 - first disease-modifying therapy. Supportive care for complications. Idebenone for cardiac symptoms. Physical and occupational therapy. Mean age of death ~35 years (often cardiac).

Sources

  • https://www.ncbi.nlm.nih.gov/books/NBK1116
  • https://medlineplus.gov/genetics
  • https://rarediseases.org

Medical Disclaimer

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