Hereditary Ataxia, Cerebellar Degeneration

Brain and Spinal Cord

Known as the "oldest" (even reptiles and more ancient species have them!) part of the brain, the cerebellum fine-tunes motor signals from the brain to the muscles, allowing for balance and coordination. When the cerebellum does not function properly, dogs become uncoordinated and do not have the ability to perform fine motor skills.

  • Signs and symptoms

    Signs of cerebellar disease include: dogs have an uncoordinated gait (ataxia) with stilted "toy soldier" leg movements. They can display "intention tremors," where a stretched out limb or head shakes more and more violently as it approaches its target. At rest, dogs sometimes display a "truncal sway;" their spinal muscles have lost their fine-tuning, pulling the spine (and everything attached to it) back and forth in a struggle to maintain balance. The muscles of the eyes have also lost this fine-tuning, leading to a rhythmic eye flicking (nystagmus).

    Signs develop in puppies.

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  • Diagnosis

    Unless a genetic basis is suspected due to the age, breed, or history of the dog, diagnostics must be performed to rule out infectious, inflammatory, or neoplastic causes.

  • Treatment

    There is no treatment for this disorder. Certain medications may be prescribed to control symptoms.

  • What to do if your dog is at risk

    Actions

    • Keeping your affected puppy comfortable and giving them the best quality of life you can are the only actions you can take at home.

  • Genetic Information

    A mutation in the RAB24 gene has been associated with a juvenile-onset form of hereditary ataxia in the Old English Sheepdog and the Gordon Setter. RAB24 is a known component of the autophagy pathway, which recycles old or dysfunctional cell parts.

    This disease is inherited in an autosomal recessive manner, meaning that affected dogs must have two copies of the mutation to show clinical signs.

    Gene names:

    RAB24 (Exon 1) ‐ chr

    Inheritance type:

    recessive

  • Breeds affected

    This health condition affects the following breeds

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