Canine degenerative myelopathy
Canine degenerative myelopathy, also known as chronic degenerative radiculomyelopathy, is an incurable, progressive disease of the canine spinal cord that is similar in many ways to amyotrophic lateral sclerosis. Onset is typically after the age of 7 years and it is seen most frequently in the German shepherd dog, Pembroke Welsh corgi, and boxer dog, though the disorder is strongly associated with a gene mutation in SOD1 that has been found in 43 breeds as of 2008, including the wire fox terrier, Chesapeake Bay retriever, Rhodesian ridgeback, and Cardigan Welsh corgi. Progressive weakness and incoordination of the rear limbs are often the first signs seen in affected dogs, with progression over time to complete paralysis. Myelin is an insulating sheath around neurons in the spinal cord. One proposed cause of degenerative myelopathy is that the immune system attacks this sheath, breaking it down. This results in a loss of communication between nerves in lower body of the animal and the brain.
The Orthopedic Foundation for Animals has a DNA saliva test to screen for the mutated gene that has been seen in dogs with degenerative myelopathy. Now that a test is available the disease can be bred out of breeds with a high preponderance. The test is only recommended for predisposed breeds, but can be performed on DNA from any dog on samples collected through swabbing the inside of the animal's cheek with a sterile cotton swab or through venipuncture.
The test determines whether the mutated copy of SOD1 is present in the DNA sample submitted. It must be interpreted with caution by a veterinary clinician in combination with the animal's clinical signs and other lab test results.
The results reported are:
- Normal / Normal (N/N, or 'clear'): The dog does not have the mutation and is extremely unlikely to develop degenerative myelopathy. There have been cases in which dogs that tested clear were found to have DM upon necropsy. This information was given to Dr Keller from the OFA. Dr Coates performed necropsy. It is important to note the OFA statement on their website that states "Recent evidence suggest that there are other causes of DM in some breeds".
- Normal / Abnormal (N/A or 'carrier'): The dog has one mutated copy of the gene (is heterozygous) and is a carrier but will not have degenerative myelopathy though there has now been several cases of Carriers developing DM. It will be possible for it to pass the mutation to offspring. A thorough examination of the dog's pedigree and DNA testing should be undertaken prior to breeding a dog with this result.
- Abnormal / Abnormal (A/A or 'At Risk'): The dog has two copies (is homozygous) for the mutation and is at risk for degenerative myelopathy.
There is a considerable amount of controversy as to the validity of the OFA DM DNA Test in relation to the German Shepherd Dog. All breeds can develop a myelopathy which is both chronic and progressive, however, the diagnostic test results between the German Shepherd Dog and other breeds are 180 degrees apart. For example:
DM Corgis, Boxers, : motor unit disease
DM GSD: Auto-immune disease
DM Corgis, Boxers : Protein is normal in the AO CSF
DM GSD: Protein is normal in the AO CSF but Protein is elevated in the Lumbar CSF
DM Corgis, Boxers: Oligoclonal bands of IgG are uncommon
DM GSDS: Oligoclonal bands of IgG are common in MS
DM Corgis, Boxers: affects cell bodies of neurons
DM GSDS: Does not affect cell bodies of neurons
DM Corgis. Boxers: muscle spams
DM GSDS: no muscle spasms
DM Corgis, Boxers: EMG is affected early in the disease
DM GSDS: EMG is normal
The fact that the diagnostic test results are so dramatically different suggest that German Shepherd Dogs develop their own unique type of Degenerative Myelopathy.
Breeding risks for degenerative myelopathy can be calculated using the Punnett Square:
- If both parents are clear (N/N) then all of the puppies will be clear
- If one parent is a carrier (N/A) and one is clear (N/N) then roughly 50% of the puppies will be clear and 50% will be carriers
- If both parents are carriers (N/A) then roughly 25% will be clear (N/N), 50% will be carriers (N/A), and 25% will be At risk (A/A)
- If one parent is clear (N/N) and one parent is affected (A/A) then all puppies will be carriers (N/A)
- If one parent is a carrier (N/A) and one is at risk (A/A) then roughly 50% of the puppies will be carriers (N/A) and 50% will be At risk (A/A)
- If both parents are At risk (A/A) then all puppies will be At risk (A/A)
Degenerative myelopathy initially affects the back legs and causes muscle weakness and loss, and lack of coordination. These cause a staggering affect that may appear to be arthritis. The dog may drag one or both rear paws when it walks. This dragging can cause the nails of one foot to be worn down. The condition may lead to extensive paralysis of the back legs. As the disease progresses, the animal may display symptoms such as incontinence and has considerable difficulties with both balance and walking. If allowed to progress, the animal will show front limb involvement and extensive muscle atrophy and paralysis. Eventually cranial nerve or respiratory muscle involvement necessitates euthanasia or long term palliative care.
Progression of the disease is generally slow but highly variable. The animal could be crippled within a few months, or may survive as long as three years or more.
The etiology of this disease is unknown. Recent research has shown that a mutation in the SOD1 gene is a risk factor for developing degnerative myelopathy in several breeds. Mutations in SOD1 are also associated with familial amyotrophic lateral sclerosis (Lou Gehrig's disease) in people. Known causes of spinal cord dysfunction should be excluded before accepting the diagnosis of degenerative myelopathy; disc disease (protrusions) or spinal cord tumors can cause compression of the spinal cord with similar signs to degenerative myelopathy.
Degenerative myelopathy is an irreversible, progressive disease that cannot be cured. There are no treatments that have been clearly shown to stop or slow progression of DM.
Exercise has been recommended to maintain the dog's ability to walk. Physiotherapy may prolong the length of time that the dog remains mobile and increase survival time. Canine hydrotherapy (swimming) may be more useful than walking. Use of a belly sling or hand-held harness allows the handler the ability to support the dog's hind legs for exercising or going up and down stairs. A 2-wheel dog cart, or "dog wheelchair" can allow the dog to remain active and maintain its quality of life once signs of weakness or paralysis of the hind limbs is detected.
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