DISEASE SCANNER

Global Incurable Diseases Tracker

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Genetic Disorder

Spinal Muscular Atrophy (SMA)

HIGH SEVERITY

An autosomal recessive motor neuron disease caused by SMN1 gene mutations affecting survival motor neuron protein. Progressive muscle weakness and atrophy. SMA type 1 (infantile) is most severe; SMA type 4 (adult-onset) is mildest.

Global Affected

100.0K

Countries

15

Symptoms

Muscle weakness
Hypotonia
Feeding difficulties
Respiratory weakness
Absent reflexes
Tongue fasciculations
Scoliosis
Joint contractures

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

Transformed by gene therapy and SMN2 modulators. Type 1: previously death by age 2, now many survive with treatment. Type 2: improved survival and function. Type 3: near normal lifespan with treatment.

Prevention

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

Research Status

Disease-modifying therapies: Nusinersen (SMN2 splicing modifier), Onasemnogene abeparvovec (gene replacement), Risdiplam (oral SMN2 modifier). Newborn screening allows presymptomatic treatment. Transformed from fatal to treatable disease.

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.