Degenerative Myelopathy (DM)

Quick Summary

Degenerative myelopathy (DM) is an inherited neurologic disorder of dogs characterized by gradual muscle wasting and loss of coordination typically beginning in the hind limbs. Testing is most appropriate for those breeds in which the clinical disease has been associated with the SOD1 allele.
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Dog with degenerative myelopathy. Photo Credit: Forrest Tanaka

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Phenotype: Affected dogs usually present clinical signs of disease in adulthood (at least 8 years of age) with gradual muscle wasting and loss of coordination typically beginning in the hind limbs. Disease progression continues until the dog is unable to walk. Small breed dogs tend to progress more slowly. In late stages of the disease, dogs may become incontinent and the forelimbs may be affected. Affected dogs may fully lose the ability to walk 6 months to 2 years after the onset of signs.

Mode of Inheritance: Autosomal recessive, incomplete penetrance

Alleles: N = Normal/Unaffected, DM = Degenerative myelopathy

Breeds appropriate for testing: Many breeds carry the SOD1 allele, but the following breeds have been reported as clinically-affected breeds: American Eskimo Dog, Australian Shepherd, Bernese Mountain Dog, Bloodhound, Borzoi, Boxer, Cardigan Welsh Corgi, Cavalier King Charles Spaniel, Chesapeake Bay Retriever, Czech Wolfdog, English Springer Spaniel, German Shepherd, Golden Retriever, Hovawart, Kerry Blue Terrier, Labrador Retriever, Pembroke Welsh Corgi, Pug, Rhodesian Ridgeback, Rough Coated Collie, Soft Coated Wheaten Terrier, Standard Poodle, and Wire Fox Terrier. Crossbred dogs have also been reported as clinically affected by SOD1-associated DM.

Explanation of Results:

  • Dogs with N/N genotype will not have degenerative myelopathy and cannot transmit this allele to their offspring.
  • Dogs with N/DM genotypes will not have degenerative myelopathy as a result of this allele, but are carriers. They will transmit this allele to 50% of their offspring. Matings between two carriers are predicted to produce 25% of puppies at risk for developing degenerative myelopathy.
  • Dogs with DM/DM may have degenerative myelopathy, a disabling condition, and will transmit this allele to all of their offspring.

Results of this test can be submitted to the OFA (Orthopedic Foundation for Animals)

Price

$55 single test per animal ($5 discount on 3 or more dogs)
$25 as additional test on same animal

Panels Available
Turnaround Time
At least 15 business days; may be delayed beyond 15 business days if sample requires additional testing, or a new sample is requested.
Additional Details

Degenerative myelopathy (DM) is an inherited neurological disorder of dogs, similar to Lou Gehrig’s disease in humans, that can be fatal. Affected dogs usually begin showing clinical signs of disease in adulthood (at least 8 years of age) with gradual muscle wasting and loss of coordination that typically begins in the hind limbs because of nerve degeneration. Disease progression continues until the dog is unable to walk. Small breed dogs tend to progress more slowly. In late stages of the disease, dogs may become incontinent, and the forelimbs may be affected. Affected dogs may fully lose the ability to walk 6 months to 2 years after the onset of clinical signs. It is important to note that various types of compressive spinal cord disorders can look like early signs of DM.

In clinically-affected breeds, the disease is inherited in an autosomal recessive fashion with incomplete penetrance, and is caused by a allele of the SOD1 gene (c.118G>A). Thus, two copies of the SOD1 allele (reported by the VGL as DM/DM) confer increased risk for DM. However, it is important to understand that not all DM/DM dogs will develop the disease. The variable presentation between breeds and incomplete penetrance supports that DM is not a simply inherited disease but rather has a complex mode of inheritance, meaning that other genetic and environmental factors likely play a role in disease risk and development. There is ongoing research to identify other genetic factors that modify risk for DM in different breeds. A single study has been published to date where Ivansson and colleagues (2016) describe an allele of the P110 nuclear body protein (SP110) gene as a possible modifier of the SOD1 allele in Pembroke Welsh Corgis. Although results showed that DM/DM dogs that also had the SP110 allele were at higher risk than those that were DM/DM only, researchers called for additional studies to further clarify the function of this SP110 allele in disease development.

A 2014 study by Zeng and colleagues revealed the presence of the SOD1 allele (c.118G>A) in crossbred dogs and dogs from 124 breeds. However, breed-specific clinical investigations have not been performed for all breeds that carry this SOD1 allele. The same 2014 study revealed an association between the homozygous DM/DM genotype and histopathology-confirmed clinical DM in mixed breed dogs and dogs from the following breeds: American Eskimo Dog, Australian Shepherd, Bernese Mountain Dog, Bloodhound, Borzoi, Boxer, Cardigan Welsh Corgi, Cavalier King Charles Spaniel, Chesapeake Bay Retriever, Czech Wolfdog, English Springer Spaniel, German Shepherd, Golden Retriever, Hovawart, Kerry Blue Terrier, Labrador Retriever, Pembroke Welsh Corgi, Pug, Rhodesian Ridgeback, Rough Coated Collie, Soft Coated Wheaten Terrier, Standard Poodle, and Wire Fox Terrier.

Genetic testing for the SOD1 allele (c.118G>A), reported by the VGL as DM, helps breeders establish the genetic status of breeding stock and select mating pairs appropriately to reduce the risk of producing DM-affected offspring. Breeding two carriers of the SOD1 allele together is predicted to produce 25% pups that may develop DM.

It is important to note that similar disease presentation has been observed in animals that do not have the SOD1 allele and not all dogs that are DM/DM will develop signs of disease. Testing is most appropriate for those breeds in which the clinical disease has been associated with the SOD1 allele. Despite the high frequency of the SOD1 allele in the canine population, other more common and treatable causes of progressive hind end weaknesses should still be considered even in homozygous DM/DM dogs.

 

NOTE: A second allele in the SOD1 gene (c.52A>T) has been associated with DM in the Bernese Mountain Dog only. The allele was found in a very low frequency in the breed and the VGL does not currently test for this allele.
Type of Sample
Interdental/GUM brushes - supplied by owner
Cytology Brush - supplied by VGL at no additional charge

Species

Dog

Breed

AMB/AMBOR Affenpinscher Afghan Hound Africanis Aidi Ainu Dog Airedale Terrier Akbash Dog Akita: American type Akita: Blend, Other Akita: Japanese type Akita: Unregistered Alaskan Husky Alaskan Klee Kai Alaskan Malamute Alaskan Sled Dog Alpine Dachsbracke American Bandogge Mastiff American Blue Gascon Hound American Bulldog American Bully American Eskimo, Miniature American Eskimo, Standard American Eskimo, Toy American Foxhound American Hairless Terrier American Pit Bull Terrier American Staffordshire Terrier American Water Spaniel Anatolian Shepherd Dog Anglo-Francais de Moyen Venerie Anglo-Francais de Petite Venerie Appenzeller Ariegeois Armant Aryan Molossus Aussiedoodle Australian Cattle Dog Australian Cobberdog Australian Domestic Dog Australian Kelpie Australian Labradoodle Australian Shepherd Australian Shepherd, Miniature Australian Terrier Austrian Black & Tan Hound Austrian Pinscher Austrian Shorthaired Pinscher Austrian Smoothhaired Hound Azawakh Bali Street Dog Barbet Basenji Basque Sheepdog Basset Artesian Normand Basset Bleu de Gascogne Basset Fauve de Bretagne Basset Hound Bavarian Mountain Hound Beagle Beagle Harrier Bearded Collie Beauceron Bedlington Terrier Belgian Griffon Belgian Laekenois Belgian Malinois Belgian Mastiff Belgian Sheepdog Belgian Tervuren Bergamasco Berger Picard Berger de Pyrenees, Smooth Muzzled Berger du Languedoc Berner Laufhund Berner Neiderlaufhund Bernese Mountain Dog Bichon Frise Biewer Biewer Terrier Biewer Yorkshire Terrier Biewer, Biro Billy Black & Tan Coonhound Black Forest Hound Black Mouth Cur Black Russian Terrier Bloodhound Blue Lacy Bluetick Coonhound Bolognese Border Collie Border Collie Cross Border Terrier Borzoi Bosnian Roughhaired Hound Boston Terrier Bouvier Des Ardennes Bouvier Des Flandres Boxer Boykin Spaniel Bracco Italiano Braque Dupuy Braque Francais, de Grande Taille Braque Francais, de Petite Taille Braque Saint-Germain Braque d'Auvergne Braque de l'Ariège Braque du Bourbonnais Brazilian Terrier Briard Briquet Griffon Vendeen Brittany Broholmer Bruno Jura Laufhund Brussels Griffon Bull Terrier Bulldog Bullmastiff CB Romanesc Carpatin Ca De Bestier Cairn Terrier Canaan Dog Canadian Cur Canadian Eskimo Dog Cane Corso Canis lupus Cardigan Welsh Corgi Carolina Dog Catahoula Leopard Dog Catalan Sheepdog Caucasian Ovcharka Cavalier King Charles Spaniel Central Asian Shepherd Cesky Fousek Cesky Terrier Chart Polski Chesapeake Bay Retriever Chien Francais Blanc et Noir Chien Francais Blanc et Orange Chien Francais Blanc et Tricolore Chien d'Artois Chihuahua Chinese Crested Chinese Foo Dog Chinook Chortaj Chow Chow Cimarron Uruguayo Cirneco dell'Etna Clumber Spaniel Cockapoo Cocker Spaniel, American Cocker Spaniel, Unknown Variety Collie Cook Island Feral Dog Coton de Tulear Coyote Croatian Sheepdog Crossbred Curly-Coated Retriever Czech Wolfdog Czechoslovakian Vlcak Dachshund Dalmatian Dandie Dinmont Terrier Danish Broholmer Danish/Swedish Farmdog Denmark Feist Deutsch Kooiker Deutsch-Drahthaar Deutsche Bracke Deutscher Wachtelhund Dingo Doberman Pinscher Dogo Argentino Dogue de Bordeaux Drentse Patrijshond Drever Dunker Dutch Malinois Dutch Shepherd, Longhaired Dutch Shepherd, Roughhaired Dutch Shepherd, Shorthaired Dutch Smoushond East European Shepherd East Siberian Laika English Bulldog English Cocker Spaniel English Coonhound English Foxhound English Mastiff English Setter English Shepherd English Springer Spaniel English Toy Spaniel English Toy Terrier Entlebucher Epagneul Bleu de Picardie Epagneul Breton Epagneul Francais Epagneul Picard Epagneul Pont-Audemer Estonian Hound Estrela Mountain Dog Eurasian Farm Collie Fell Hound Fell Sheepdog Field Spaniel Fila Brasileiro Finnish Hound Finnish Lapphund Finnish Spitz Flat-Coated Retriever Fox Fox Terrier, Smooth Fox Terrier, Wire French Bulldog Galgo Espanol German Drahthaar German Hunt Terrier German Longhaired Pointer German Pinscher German Sheeppoodle German Shepherd German Shorthaired Pointer German Wirehaired Pointer German Wolfspitz Giant German Spitz Giant Schnauzer Glen of Imaal Terrier Golden Retriever Goldendoodle Gordon Setter Gr Ang-Fr Tricolor Hound Gr Ang-Fr W & B Hound Gr Ang-Fr W & O Hound Grand Anglo-Francais Grand Basset Griffon Vendeen Grand Bleu de Gascogne Grand Gascon-Saintongeois Grand Griffon Vendeen Great Dane Great Pyrenees Greater Swiss Mountain Dog Greek Harehound Greek Sheepdog Greenland Dog Greyhound Griffon Bleu De Gascogne Griffon Fauve de Bretagne Griffon Nivernais Haldenstovare Hamiltonstovare Hanoverian Hound Harlequin Pinscher Harrier Havana Silk Havanese Hawaiian Poi Dog Heading Dog Hertha Pointer Hovawart Huntaway Hygenhund Ibizan Hound Iceland Dog Inca Hairless Dog Inuit Sled Dog Inuit Sled Dog Mix Irish Red & White Setter Irish Setter Irish Terrier Irish Water Spaniel Irish Wolfhound Islandsk Farehund Istrian Hound, Smoothhaired Istrian Hound, Wirehaired Italian Greyhound Italian Hound Jack Russell Terrier Japanese Chin Japanese Spitz Japanese Terrier Jindo Dog Jura Neiderlaufhund Kai Dog Kangal Dog Kangaroo Dog Karakachan Karelian Bear Dog Karelo-Finnish Laika Kayo Inu Keeshond Kerry Beagle Kerry Blue Terrier King Shepherd Kintamani Dogs Kishu Komondor Kooikerhondje Koolie Korean German Shepherd Kraski Ovcar Kromfohrlander Kuvasz Kyi Leo Labradoodle Labrador Retriever LabxGolden Laekense Lagotto Romagnolo Lakeland Terrier Lamalese Lancashire Heeler Landseer Lapinporokoira Lapponian Herder Large Munsterlander Large Spanish Hound Latvian Hound Leonberger Leopard Cur Leopard Hound Levesque Lhasa Apso Lithuanian Hound Llewellin Setter Lowchen Lundehund Lurcher Luzerner Laufhund Luzerner Neiderlaufhund Magyar Agar Majestic Tree Hound Maltese Manchester Terrier Manchester Terrier, Toy Maremma Sheepdog Mastiff Matthes Lion Hound McNab Mi-Ki Middle Asian Owtcharka Miniature American Shepherd Miniature Bull Terrier Miniature Goldendoodle Miniature Pinscher Montenegrin Mountain Hound Moscow Longhaired Toy Terrier Moscow Watchdog Mountain Cur Mudi Mullins Feist Native American Indian Dog Neapolitan Mastiff New Guinea Singing Dog Newfoundland Norfolk Terrier Norrbottenspets North American Shepherd Northeasterly Hauling Laika Norwegian Buhund Norwegian Elkhound Norwegian Elkhound, Black Norwegian Lundehund Norwich Terrier Nova Scotia Duck Tolling Retriever Old Danish Bird Dog Old English Sheepdog Olde English Bulldogge Otterhound Owczarek Podhalanski Papillon Parson Russell Terrier Patterdale Terrier Pekingese Pembroke Welsh Corgi Perdiguero Navarro Perdiguero Portugueso Perdiguero de Burgos Perro de Pastor Mallorquin Perro de Presa Canario Perro de Presa Mallorquin Peruvian Hairless Dog Peruvian Inca Orchid Petit Basset Griffon Vendeen Petit Bleu de Gascogne Petit Brabancon Petit Gascon-Saintongeois Petit Griffon Bleu de Gascogne Phalene Pharaoh Hound Pit Bull Plott Hound Podenco Canario Podengo Portugueso Grande Podengo Portugueso Medio Podengo Portugueso Pequeno, Smooth Podengo Portugueso Pequeno, Wirehaired Pointer Poitevin Polish Hound Polish Lowland Sheepdog Polish Owczarek Podhalanski Polski Owczarek Nizinny Pomeranian Pomsky Poodle, Miniature Poodle, Standard Poodle, Toy Poongsan Dog Porcelaine Portuguese Cattle Dog Portuguese Pointer Portuguese Sheepdog Portuguese Water Dog Posavac Hound Prager Ratter Pudelpointer Pug Puli Pumi Pyrenean Mastiff Pyrenean Shepherd Rafeiro do Alentejo Rat Terrier Red & White Setter Redbone Hound Rhodesian Ridgeback Ridgeback Cross Rottweiler Rumanian Sheepdog Russell Terrier Russian Harlequin Hound Russian Hound Russian Samoyed Laika Russian Spaniel Russkiy Toy Russo-European Laika Saarlooswolfhond Sabuesos Espanol Lebrero Sabuesos Espanol de Monte Saint Bernard Saint Hubert Jura Laufhund Saint Usage Spaniel Saluki Samoyed Sanshu Dog Santal Hound Sapsaree Sarplaninac Schapendoes Schillerstovare Schipperke Schnauzer, Miniature Schnauzer, Standard Schweizer Laufhund Schweizer Neiderlaufhund Scottish Collie Scottish Deerhound Scottish Terrier Sealyham Terrier Segugio Italiano a Pelo Forte Segugio Italiano a Pelo Raso Seppala Siberian Sleddog Serbian Hound Serbian Tricolor Hound Shar Pei Shetland Sheepdog Shiba Inu Shih Tzu Shikoku Shiloh Shepherd Dog Shiloh Shepherd, ISSA Siberian Husky Silky Terrier Skye Terrier Sloughi Slovak Cuvac Slovakian WH Pointing Dog Smalandsstovare Small German Spitz Small Munsterlander Soft Coated Wheaten Terrier South African Boerboel Mastiff South Russian Ovcharka South Russian Steppe Hound Spanish Alano Spanish Mastiff Spanish Water Dog Spinone Italiano Sporting Lucas Terrier Springer Spaniel Sri Lankan Dog Stabyhoun Staffordshire Bull Terrier Standard German Spitz Stephens Stock Stichelhaar Strellufstover Stumpy-Tail Cattle Dog Styrian Roughhaired Mountain Hound Sudanese Dog Sussex Spaniel Swedish Elkhound Swedish Lapphund Swedish Vallhund Tahltan Bear Dog Taigan Tamaskan Tasy Teddy Roosevelt Terrier Telomian Tengger Dog Tenterfield Terrier Thai Ridgeback Thornburg Feist Tibetan Mastiff Tibetan Spaniel Tibetan Terrier Tosa Inu Tosa Ken Toy Fox Terrier Toy German Spitz Transylvanian Hound, Long Transylvanian Hound, Short Treeing Feist Treeing Tennessee Brindle Treeing Walker Coonhound Turkish Feral Dog Turkish Greyhound Tyrolean Hound Tyroler Bracke Unknown Vasgotaspets Vizsla Volpino Italiano Walker Coonhound Weimaraner Welsh Hound Welsh Sheepdog Welsh Springer Spaniel Welsh Terrier West Highland White Terrier West Siberian Laika Westphalian Dachsbracke Wetterhoun Whippet Whippet, Longhaired Cross White Shepherd Windsprite (Longhaired Whippet) Wirehaired Pointing Griffon Wirehaired Vizsla Wolf Wolf Hybrid Working Dog X-Bred Coonhound Xoloitzcuintli Xoloitzcuintli, Toy Yorkshire Terrier
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Type of Test
Health
Results Reported As
Test Result Degenerative Myelopathy (DM)
N/N No copies of the DM mutation.
N/DM 1 copy of the DM mutation.
DM/DM 2 copies of the DM mutation. Dog may develop DM disease.
References

Coates, J. R., March, P. A., Oglesbee, M., Ruaux, C. G., Olby, N. J., Berghaus, R. D., O'Brien, D. P., Keating, J. H., Johnson, G. S., & Williams, D. A. (2007). Clinical characterization of a familial degenerative myelopathy in Pembroke Welsh Corgi dogs. Journal of Veterinary Internal Medicine, 21(6), 1323. doi: 10.1892/07-059.1

Awano, T., Johnson, G. S., Wade, C. M., Katz, M. L., Johnson, G. C., Taylor, J. F., Perloski, M., Biagi, T., Baranowska, I., Long, S., March, P. A., Olby, N. J., Shelton, G. D., Khan, S., O'Brien, D. P., Lindblad-Toh, K., & Coates, J. R. (2009). Genome-wide association analysis reveals a SOD1 mutation in canine degenerative myelopathy that resembles amyotrophic lateral sclerosis. Proceedings of the National Academy of Sciences, 106(8), 2794-2799. doi: 10.1073/pnas.0812297106

Shelton, G. D., Johnson, G. C., O’Brien, D. P., Katz, M. L., Pesayco, J. P., Chang, B. J., Mizisin, A. P., & Coates, J. R. (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. Journal of the Neurological Sciences, 318(1-2), 55-64. doi: 10.1016/j.jns.2012.04.003

Zeng, R., Coates, J. R., Johnson, G. C., Hansen, L., Awano, T., Kolicheski, A., Ivansson, E., Perloski, M., Lindblad-Toh, K., O'Brien, D. P., Guo, J, Katz, M. L., & Johnson, G. S. (2014). Breed Distribution of SOD1 Alleles Previously Associated with Canine Degenerative Myelopathy. Journal of Veterinary Internal Medicine, 28(2), 515-521. doi: 10.1111/jvim.12317

Ivansson, E. L., Megquier, K., Kozyrev, S. V., Murén, E., Körberg, I. B., Swofford, R., Koltookian, M., Tonomura, N., Zeng, R., Kolicheski, A. L., Hansen, L., Katz, M. L., Johnson, G. C., Johnson, G. S., Coates, J. R., & Lindblad-Toh, K. (2016). Alleles within the SP110 nuclear body protein modify risk of canine degenerative myelopathy. Proceedings of the National Academy of Sciences of the United States of America, 113(22), E3091–E3100. doi: 10.1073/pnas.1600084113

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