The term is used to describe premature degeneration of fully formed cerebellar neurons caused by an intrinsic metabolic defect (literally the loss of a vital nutritive factor). Cerebellar abiotrophies typically involve a primary degeneration or loss of Purkinje neurons, variable loss of granule cells, and cortical astrogliosis.
The disease, which has been observed in cats, sheep, cattle, pigs, horses, and alpacas, has also been reported in a number of dog breeds.
Breeds of dogs with a predisposition to cerebellar abiotrophy
|Breed||Age of onset||Neurological signs||Associated condition|
|Airedale Terrier||< 6 months||Progressive|
|American Staffordshire Terrier||1.5 - 9 years||Slowly progressive|
|Australian Kelpie||6 - 12 years||Progressive|
|Bavarian Mountain Dog||3 - 7 months||Slowly progressive||cerebellar Purkinje cell degeneration|
|Bernese Mountain Dog||4 - 16 weeks||Progressive||hepatocerebellar degeneration|
|Border Collie||6 - 16 weeks||Progressive|
|Brittany Spaniel||7 - 13 years||Slowly progressive|
|Bullmastiff||4 - 28 weeks||Progressive|
|Chinese Crested||3 - 6 months||Slowly progressive|
|Coton de Tulear||8/2 weeks||Progressive/Non-progressive|
|English Bulldog||8 - 12 weeks||Slowly progressive|
|Finnish Harrier||< 6 months||Progressive||cerebellar Purkinje cell degeneration|
|Gordon Setter||6 - 10 months||Slowly progressive|
|Irish Setter||3 - 10 days||Progressive|
|Jack Russell Terrier||2 weeks||Progressive|
|Kerry Blue Terrier||8 - 16 weeks||Progressive|
|Labrador Retriever||12 weeks||Rapidly progressive|
|Lagotto Romagnolo||10 - 15 weeks||Rapidly progressive|
|Miniature Poodle||3 - 4 weeks||Unknown|
|Miniature Schnauzer||6 - 16 weeks||Progressive|
|Papillon||8 - 12 weeks||Progressive|
|Rhodesian Ridgeback||Birth||Progressive||Color dilution alopecia|
|Rough Coated Collie||4 - 8 weeks||May stabilize|
|Samoyed||Birth - 6 months||Slowly progressive|
Clinically affected dogs are normal at birth and present at an older age with hindlimb ataxia, head tilt, head pressing, intentional tremors, seizures and progressive proprioceptive deficits resulting in collapse.
Blood tests, CSF analysis and computed tomography are usually unrewarding.
Culturing of CSF fluid is recommended to eliminate infectious causes such as canine distemper virus, Tick-borne encephalitis virus, Borrelia burgdorferi, Neospora caninum, Toxoplasma gondii, Anaplasma phagocytophilum, Ehrlichia canis, Rickettsia spp and Cryptococcus spp.
Definitive diagnosis usually requires histological examination of brain tissue, which typically shows primary degeneration or loss of Purkinje neurons, variable loss of granule cells, and cortical astrogliosis. PCR testing of brain tissue for canine parvovirus DNA is essential to exclude this as a cause of disease.
There is no known treatment for this disease and severely affected dog usually require euthanasia, however, this disease is not always fatal and mildly affected dogs can lead a relatively normal life.
- Gumber S et al (2010) Late onset of cerebellar abiotrophy in a boxer dog. Vet Med Int 2010:406275
- de Lahunta A (1990) Abiotrophy in domestic animals: a review. Can J Vet Res 54(1):65–76
- de Lahunta, A & Glass, E (2009) Cerebellum; in Veterinary Neuroanatomy and Clinical Neurology, A. De Lahunta and E. Glass, Eds., pp:343–388, Elsevier, St. Louis, Mo, USA
- Summers BA et al (1995) Degenerative diseases of the central nervous system. In: Summers BA, Cummings JF, De Lahunta A, editors. Veterinary Neuropathology. St. Louis, Mo, USA: Mosby-Year Book. pp:300–305
- Adapted from Dewey, CW (2008) A practical guide to canine and feline neurology. Iowa State University Press, Iowa. 2nd edition. pp:300
- Shearman JR et al (2011) Mapping cerebellar abiotrophy in Australian Kelpies. Anim Genet 42(6):675-678
- Carmichael KP et al (1996) Clinical, hematologic, and biochemical features of a syndrome in Bernese Mountain Dogs characterized by hepatocerebellar degeneration. J Am Vet Med Assoc 208:1277–1279
- Higgins RJ et al (1998) Late-onset progressive spinocerebellar degeneration in Brittany Spaniel dogs. Acta Neuropathol 96:97–101
- de Lahunta A et al (1980) Hereditary cerebellar cortical abiotrophy in the Gordon setter. J Am Vet Med Assoc 177:538–541
- deLahunta A & Averill DR (1976) Hereditary cerebellar cortical and extrapyramidal nuclear abiotrophy in Kerry Blue Terriers. J Am Vet Med Assoc 168:1119–1124
- Jokinen TS et al (2007) Cerebellar cortical abiotrophy in Lagotto Romagnolo dogs. J Small Anim Pract 48(8):470-473
- Berry ML & Blas-Machado U (2003) Cerebellar abiotrophy in a miniature schnauzer. Can Vet J 44(8):657-659
- Nibe K et al (2007) Clinicopathological features of canine neuroaxonal dystrophy and cerebellar cortical abiotrophy in Papillon and Papillon-related dogs. J Vet Med Sci 69(10):1047-1052
- Chieffo C et al (1994) Cerebellar Purkinje's cell degeneration and coat color dilution in a family of Rhodesian Ridgeback dogs. J Vet Intern Med 8:112–116
- De Lahunta A & Glass E (2009) Cerebellum. In: De Lahunta A, Glass E, editors. Veterinary Neuroanatomy and Clinical Neurology. St. Louis, Mo, USA: Elsevier. pp:343–388
- Nibe K et al (2010) Comparative study of cerebellar degeneration in canine neuroaxonal dystrophy, cerebellar cortical abiotrophy, and neuronal ceroid-lipofuscinosis. J Vet Med Sci 72(11):1495-1499