DISEASE SCANNER

Global Incurable Diseases Tracker

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Genetic Disorder

Spinocerebellar Ataxia (SCA)

HIGH SEVERITY

A group of autosomal dominant neurodegenerative disorders characterized by progressive cerebellar ataxia. Multiple subtypes (SCA1, SCA2, SCA3/Machado-Joseph, etc.) caused by CAG repeat expansions or other mutations. Variable features.

Global Affected

250.0K

Countries

15

Symptoms

Progressive gait ataxia
Limb ataxia
Dysarthria
Oculomotor abnormalities
Pyramidal signs
Neuropathy (some types)
Cognitive impairment (some)
Extrapyramidal features

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

Variable depending on specific subtype. SCA1, SCA2, SCA3: wheelchair dependence typically 10-15 years after onset, death 20-30 years after onset. SCA6: slower progression, 20-30 years to wheelchair, normal life span possible. SCA7: vision loss in addition to ataxia, progression over 20-30 years. All types progressive with no cure. Supportive care improves quality of life. Genetic anticipation with earlier onset in subsequent generations. Respiratory complications common cause of death.

Prevention

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

Research Status

No disease-modifying therapy. Supportive care: physical therapy, assistive devices, speech therapy. Managing complications. Genetic testing for diagnosis and family planning. Research on antisense oligonucleotides for specific types.

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.