The detail information of Leukodystrophies
Basic Information

Disease Description: Leukodystrophies in dogs, a rare condition, relates to loss of the fatty substance called myelin that coats nerve cells and protects the normal conduction of the nerve impulses.

Inherit Mode: Dalmatian leukodystrophy, Rottweiler leukodystrophy, hereditary ataxia, Labrador retriever central axonopathy, and Afghan myelomalacia are believed to be autosomal recessive.

Disease Symptom: Rottweiler leukodystrophy/ Leukoencephalomyelopathy: Affected dogs begin to lose muscle coordination (develop ataxia), somewhere between 11/2 to 4 years of age. They may also have an exaggerated gait. The condition worsens over 6 to 12 months until the dog is unable to rise. Dalmatian leukodystrophy:Clinical signs of this disorder begin at 3 to 6 months of age, and include difficulties with vision, poor coordination, and weakness. Miniature poodle leukodystrophy/ Demyelinating myelopathy:Signs of weakness begin at 2 to 4 months of age and rapidly worsen to paralysis. Hereditary ataxia (progressive ataxia): This is seen in smooth-haired fox terriers and Jack Russell terriers. A gradual loss of coordination begins by 2 to 6 months of age, and progresses to the point where the dog is unable to walk. These dogs have what is called intention tremor - that is the tremor worsens with effort (to move toward something for example) and subsides when the dog is at rest. Hound ataxia:This condition (seen in beagles, fox hounds, and harrier hounds) begins at 2 to 7 years of age with poor coordination in the hind limbs, which gradually worsens over the next 18 months or so. There is some evidence that this problem is due to environmental causes rather than of a hereditary nature. Labrador retriever central axonopathy:Signs are evident by 4 to 6 weeks of age, and include lack of coordination, weakness and an exaggerated gait. By 5 months of age, affected pups are unable to walk. Afghan myelomalacia (hereditary myelopathy of Afghan hounds):Signs of weakness and incoordination develop in affected dogs by 3 to 13 months of age and progress rapidly.

Disease Cause: -

Disease Diagnose: These conditions are rare. Your veterinarian will base the diagnosis on the clinical signs, a detailed neurological examination, and lack of abnormalities on other diagnostic tests. The specific changes in the nervous system can only be confirmed by examination after death (post-mortem examination).

Treat Method: Unfortunately there is no treatment for these disorders. Your veterinarian will help you decide when your dog's condition has deteriorated to the point where euthanasia is the best option.

Breeder Advice: Except in the rottweiler, the signs of these conditions are apparent before breeding age. Of course affected dogs should not be used for breeding; breeding of parents and siblings (suspect carriers) should be avoided as well.

Disease Description Source: Link

Associated Diseases
Disease Name Other Name Mode of inheritance Link ID Possible OMIM ID Gene
Leucodystrophy canine spongiform leukoencephalomyelopathy Mitochondrial - CYTB
Associated Breeds
iDog Breed Number Breed Name Personality Height Weight Breed Source
CB95 English Foxhound Affectionate, Gentle, Sociable 61 cm 27.2-34 kg United Kingdom (England)
CB147 Labrador Retriever Friendly and outgoing, Labs play well with others 57.2-62.2 cm (male), 54.6-59.7 cm (female) 29.5-36.3 kg (male), 24.9-31.8 kg (female) Canada, United Kingdom (England)
CB177 Parson Russell Terrier Bold, friendly, athletic, and clever 35.6 cm (male), 33 cm (female) 5.9-7.7 kg United Kingdom (England)
CB205 Rottweiler Reserved with strangers and affectionate and loyal with his family. 61-68.6 cm (male), 55.9-63.5 cm (female) 49.9-59 kg (male), 34.9-49.9 kg (female) Germany
CB210 Samoyed Gentle, adaptable, eager, friendly; conservative but never shy 53.3-59.7 cm (male), 48.3-53.3 cm (female) 20.4-29.5 kg (male), 15.9-22.7 kg (female) Russia
CB214 Scottish Terrier Independent, confident, and never shy; spirited but dignified 25.4 cm 8.6-10 kg (male), 8.2-9.5 kg (female) United Kingdom (Scotland)
CB221 Silky Terrier Friendly, vivacious, cheeky; in all ways a true terrier 22.9-25.4 cm around 4.5 kg Australia
References
2021 Quitt,P.R.,Brühschwein,A.,Matiasek,K.,Wielaender,F.,Karkamo,V.,Hytönen,M.K.,Meyer-Lindenberg,A.,Dengler,B.,Leeb,T.,Lohi,H.,Fischer,A.: :
A hypomyelinating leukodystrophy in German Shepherd dogs. J Vet Intern Med :, 2021.
Pubmed reference: 33734486 . DOI: 10.1111/jvim.16085 .
2019 Störk,T.,Nessler,J.,Anderegg,L.,Hünerfauth,E.,Schmutz,I.,Jagannathan,V.,Kyöstilä,K.,Lohi,H.,Baumgärtner,W.,Tipold,A.,Leeb,T.: :
TSEN54 missense variant in Standard Schnauzers with leukodystrophy. PLoS Genet 15:e1008411, 2019.
Pubmed reference: 31584937 . DOI: 10.1371/journal.pgen.1008411 .
1999 Ackerman, L. :
The Genetic Condition: A Guide to Health Problems in Purebred Dogs AAHA Press. Lakewood, Colorado. pp 137-138
1995 LeCouteur, R.A., Child, G. :
Diseases of the spinal cord W.B. Saunders Co., Toronto. S.J. Ettinger and E.C. Feldman (eds.) Textbook of Veterinary Internal Medicine, p. 629-696
1977 Bjerkås,I.: :
Hereditary "cavitating" leucodystrophy in Dalmation dogs. Light and electron microscopic studies. Acta Neuropathol 40:163-9, 1977.
Pubmed reference: 930564 . DOI: 10.1007/bf00688706 .