Cerebellar spongy degeneration

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Cerebellar spongy degeneration characterized by vacuolation and loss of myelin[1]

Cerebellar spongy degeneration (Shaker dog syndrome; spongiform leucoencephalopathy) is an autosomal-recessive genetic neurological disease reported in the Malinois (Belgian Shepherd) and Labrador Retriever breeds characterized by cerebellar degeneration and ataxia<[2].

This degenerative neuronal disease affects very young pups under 6 months of age and affected pups appear underweight and showing signs of severe ataxia, proprioceptive deficits, hypermetria, intention tremors which usually progress to non-ambulatory tetraparesis[3].

Postmortem examination of the brain in affected pups has revealed marked bilateral spongy degeneration of the cerebellar nuclei and vacuoles in the granular cell layer and foliate white matter of the cerebellum. In some puppies, discrete vacuoles in gray and white matter were present in other parts of the brain with associated spheroids and dilated myelin sheaths[4].

A differential diagnosis would include cerebellar abiotrophy and canine parvovirus-associated leucoencephalopathy.

There is no known treatment for this condition and bitches should be sterilized to prevent transmission of this condition.

References

  1. O'Brien DP & Zachary JF (1985) Clinical features of spongy degeneration of the central nervous system in two Labrador retriever littermates. J Am Vet Med Assoc 186(11):1207-1210
  2. Richards, B & Kakulasb, A (1978) Spongiform leucoencephalopathy associated with congenital myoclonia syndrome in the dog. J Comp Pathol 88:317-320
  3. Mariani CL et al (2001) Magnetic resonance imaging of spongy degeneration of the central nervous system in a Labrador Retriever. Vet Radiol Ultrasound 42(4):285-290
  4. Kleiter M et al (2011) Spongy degeneration with cerebellar ataxia in Malinois puppies: a hereditary autosomal recessive disorder? J Vet Intern Med 25(3):490-496