ISSN 2398-2969      

Muscular dystrophy

icanis

Introduction

  • Cause: heterogenous group of inherited, degenerative, mostly non-inflammatory muscle diseases.
  • Signs: progressive muscle weakness, wasting and contracture, cardiomyopathy.
  • Diagnosis: high serum creatinine kinase (CK), muscle biopsy, genetic testing.
  • Treatment: no specific treatment.
  • Prognosis: poor prognosis, progressively fatal disease.
    Print off the owner factsheet on Muscular dystrophies Muscular dystrophies to give to your client.

Pathogenesis

Etiology

  • Heterogenous group of more than 30 hereditary degenerative myopathies.
  • Dystrophin deficiency is the most common of muscular dystrophy and is associated with genetic mutations of the dystrophin gene.
  • Dystrophin gene is located on X-chromosome => dystrophin muscular dystrophy is an X-linked recessive trait transmitted by a female carrier.
  • Other less common forms of muscular dystrophy in dogs include sarcoglycan deficiency, dystroglycan deficiency and merosin (laminin alpha2) deficiency.

Pathophysiology

  • Dystrophin links the myofiber cytoskeleton to the extracellular matrix and is essential in stabilization of the muscle fiber membranes during contraction.
  • Mutations of genes coding for dystrophin or its associated proteins => disruption of the dystrophin-glycoprotein complex => membrane instability => leakage of intracellular contents into the extracellular fluid space and skeletal and cardiac muscle fiber damage.
  • Laminin alpha2 is the major component of the basal lamina that surrounds each muscle fiber and one of the extracellular ligands for the dystrophin-associated glycoprotein complex => links dystrophin to the extracellular matrix and contributes to the stability of the muscle basement membrane.

Timecourse

  • Signs usually develop in first 6 months of life and are most often rapidly progressive.

Diagnosis

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Treatment

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Prevention

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Outcomes

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Further Reading

Publications

Refereed papers

  • Recent references from PubMed and VetMedResource.
  • Brumitt J, Essman S C et al (2006) Radiographic features of Golden retriever muscular dystrophy. Vet Rad Ultrasound 47 (6), 574-580 PubMed.
  • Wieczorek L A, Garosi L S, Shelton G D (2006) Dystrophin-deficient muscular dystrophy in an old English sheepdog. Vet Rec 158 (8), 270-273 PubMed.
  • Schatzberg S J, Shelton G D (2004) Newly identified neuromuscular disorders. Vet Clin North Am Small Anim Pract 34 (6), 1497-1524 PubMed.
  • Shelton G D, Engvall E (2002) Muscular dystrophies and other inherited myopathies. Vet Clin North Am Small Anim Pract 32 (1), 103-123 PubMed.
  • Shelton G D, Liu L A et al (2001) Muscular dystrophy in female dogs. J Vet Intern Med 15 (3), 240-244 PubMed.
  • Wetterman C A, Harkin K R et al (2000) Hypertrophic muscular dystrophy in a young dog. J Am Vet Med Assoc 216 (6), 878-881 PubMed.

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