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.
Testing
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 : The dog does not have the mutation and is extremely unlikely to develop degenerative myelopathy. There have been cases, however, in which dogs that tested clear were found to have DM upon necropsy.
- Normal / Abnormal : The dog has one mutated copy of the gene and is a carrier but will not have degenerative myelopathy though there has now been some cases of heterozygous 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 : The dog has two copies for the mutation and is at risk for degenerative myelopathy.
Genetics
Breeding risks for degenerative myelopathy can be calculated using the Punnett Square:- If both parents are clear then all of the puppies will be clear.
- If one parent is a carrier and one is clear each puppy has a 50% chance of being clear and a 50% chance of being a carrier.
- If both parents are carriers each puppy has a 25% chance of being clear, 50% chance of being a carrier, and 25% chance of being affected and carrier
- If one parent is clear and one parent is affected then all puppies will be carriers
- If one parent is a carrier and one is at risk each puppy has a 50% chance of being a carrier and 50% chance of being affected and carrier
- If both parents are at risk then all puppies will be affected and carrier
Symptoms
Degenerative myelopathy initially affects the back legs and causes muscle weakness and loss, and lack of coordination. These cause a staggering effect 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.
Causes
The etiology of this disease is unknown. Recent research has shown that a mutation in the SOD1 gene is a risk factor for developing degenerative myelopathy in several breeds. Mutations in SOD1 are also associated with familial amyotrophic lateral sclerosis in people. More than 100 SOD1 gene mutations are involved in human familial amyotrophic lateral sclerosis, and the pathologic spinal lesions of ALS are similar to those of canine DM, making canine DM a potentially useful animal model of ALS.Known causes of spinal cord dysfunction should be excluded before accepting the diagnosis of degenerative myelopathy; disc disease or spinal cord tumors can cause compression of the spinal cord with similar signs to degenerative myelopathy.