DISEASE SCANNER

Global Incurable Diseases Tracker

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Genetic Disorder

Limb-Girdle Muscular Dystrophy

HIGH SEVERITY

A group of inherited muscular dystrophies affecting shoulder and pelvic girdle muscles. Multiple genetic subtypes with autosomal dominant (LGMD1) and recessive (LGMD2) inheritance. Variable age of onset and progression.

Global Affected

400.0K

Countries

15

Symptoms

Proximal muscle weakness
Difficulty climbing stairs
Difficulty rising from chair
Shoulder weakness
Scapular winging
Cardiac involvement (some types)
Respiratory weakness

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

Highly variable by subtype. Some forms (LGMD2B, 2L) have slow progression with preserved ambulation into 60s-70s. Other forms (LGMD2C-F, sarcoglycanopathies) cause loss of ambulation in teens-20s. Cardiac involvement in some subtypes requires monitoring. Respiratory weakness develops in advanced stages. Non-invasive ventilation extends survival and quality of life. Corticosteroids benefit some dystrophin-associated forms. Regular cardiac and pulmonary monitoring essential.

Prevention

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

Research Status

Gene therapy trials for LGMD2I, LGMD2E, LGMD2C. Supportive care: physical therapy, respiratory support, cardiac management. No approved disease-modifying therapy yet. Clinical trials of AAV-mediated gene replacement ongoing.

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.