Degenerative myelopathy

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Degenerative myelopathy (DM) is an autosomal-recessive genetic neurological disease characterized by progressive ataxia and weakness.

This disease is an adult-onset, fatal neurodegenerative disease commonly reported in the Pembroke Welsh Corgi, Boxer and Bernese Mountain Dog, but can occur in many other breeds[1].

It is caused by missense mutations in the superoxide dismutase (SOD1) gene, which leads to muscle atrophy due to progressive denervation as a result of axonal degeneration and demyelination[2].

In the German Shepherd, this disease is associated with concurrent pituitary dwarfism, congenital megaesophagus and pancreatic acinar atrophy[3].

Clinically affected dogs present with progressive ataxia, inconctinence and hemiparesis and paralysis.

A differential diagnosis would include spinal cord injuries, vertebral osteomyelitis, syringomyelia, osteochondromatosis, various causes of peripheral neuropathy, meningoencephalitis and [osteochondroma]].

A definitive diagnosis requires PCR-based gene testing.

There is no known treatment for this condition.

References

  1. Wininger FA et al (2011) Degenerative myelopathy in a Bernese Mountain Dog with a novel SOD1 missense mutation. J Vet Intern Med 25(5):1166-1170
  2. Shelton GD et al (2012) Degenerative myelopathy associated with a missense mutation in the superoxide dismutase 1 (SOD1) gene progresses to peripheral neuropathy in Pembroke Welsh Corgis and Boxers. J Neurol Sci 318(1-2):55-64
  3. Tsai KL et al (2012) Genome-wide association studies for multiple diseases of the German Shepherd Dog. Mamm Genome 23(1-2):203-211