Late Onset Spinocerebellar Ataxia

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).

    Unlike other SCAs in the Russell Terriers, loSCA begins between six and twelve months of age.

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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

    During the first few months of the disease, symptoms can become progressively more severe. Though the disease can stabilize to some degree with time, episodes of worsening have been reported. As walking becomes extremely difficult towards the end stages of the disease, euthanasia is usually elected.

  • What to do if your dog is at risk

    Actions

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

  • Genetic Information

    Late onset spinocerebellar ataxia (loSCA) is an inherited disease first identified in the Parson Russell Terrier. LoSCA has been mapped to a mutation in the CAPN1 gene.

    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:

    CAPN1 ‐ chr18

    Inheritance type:

    recessive

  • Breeds affected

    This health condition affects the following breeds

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