DISEASE SCANNER

Global Incurable Diseases Tracker

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Genetic Disorder

Congenital Muscular Dystrophy

HIGH SEVERITY

A group of muscular dystrophies presenting at birth or early infancy with hypotonia, muscle weakness, and contractures. Multiple subtypes including merosin-deficient, Ullrich, and LMNA-related. Variable severity and progression.

Global Affected

150.0K

Countries

15

Symptoms

Hypotonia (floppy infant)
Muscle weakness
Contractures
Joint laxity
Respiratory insufficiency
Feeding difficulties
Delayed motor milestones
Scoliosis

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 by subtype. Ullrich CMD: progressive, shortened lifespan. LMNA-related CMD: cardiac involvement common. Bethlem myopathy: milder, near normal lifespan. Respiratory failure major cause of mortality.

Prevention

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

Research Status

Supportive care: respiratory support, feeding assistance, physical therapy. Gene therapy trials for merosin-deficient CMD. No approved disease-modifying therapy. Multidisciplinary management essential. Prognosis varies by subtype.

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.