Hereditary Ataxia, Cerebellar Degeneration

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.

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.

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

Talk to your vet about your dog’s hereditary ataxia result so you can plan ongoing care and monitoring.

Watch for signs such as unsteady movement, tremors, or changes in balance and coordination, and contact your vet if you notice any of these.

Keep your dog’s environment calm and consistent. Use rugs or mats to prevent slipping and help them move safely around the home.

As this condition can progress over time, supportive care and a predictable routine can help keep your dog comfortable and engaged with their family.

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.

Penetrance: 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

RAB24 (Exon 1)

Inheritance type

recessive

Clinical category

Neurologic

Citations

1. Agler et al 2014

• Gordon Setter
• Old English Sheepdog