Cerebellar Abiotrophy, Neonatal Cerebellar Cortical Degeneration, NCCD

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 and worsen over time.

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 cerebellar abiotrophy, NCCD result so you can plan ongoing care and monitoring.

Watch for early signs such as unsteady movement, head tremors, or difficulty walking, 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.

Dogs with severe symptoms may have a shortened lifespan, but supportive care can help keep them comfortable.

Neonatal cerebellar cortical degeneration (NCCD) is a progressive neurological disease. Puppies with NCCD are born with a normal cerebellum, but degeneration begins soon after birth. As soon as pups start to walk, they display signs of cerebellar dysfunction. Once NCCD stabilizes, it typically does not worsen. However, NCCD dogs will be never be able to walk or maintain their balance. They will likely require help eating and going to the bathroom as well as regular nursing care to reduce pressure sores or scrapes. In Beagles, NCCD has been mapped to a mutation in the SPTBN2 gene.

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

SPTBN2

Inheritance type

recessive

Clinical category

Neurologic

Citations

1. Forman et al 2012
2. Gao et al 2011

• Beagle