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Cerebellar Ataxia

CEREBELLAR ATAXIA is a degenerative or inflammatory disease of the cerebellum causing, among other things, incoordination of voluntary movements and balance disorders.

Age of onset

  • This disease begins in adulthood
  • The first symptoms appear between 3 and 5 years of age in the vast majority of cases
  • The disease can appear in dogs between 1 and 9 years of age
  • Males and females are affected in equal proportions
Important: It is crucial to screen dogs — ESPECIALLY breeding dogs — in order to prevent rather than cure.

Clinical signs

Initial manifestation

Hereditary cerebellar ataxia is a disease of insidious onset. It first manifests as "clumsiness", or a slight gait abnormality, particularly in certain challenging situations:

  • Going up or down stairs
  • Changing direction
  • Swimming (risk of drowning)

At this early stage, some dogs occasionally experience episodes of opisthotonus lasting a few seconds, or full-body muscle contractions, and may stiffen during sleep.

Disease progression

As the disease progresses, ataxia becomes permanent and increasingly pronounced:

Hypermetria

The dog walks with a "goose-stepping" gait — it lifts its limbs exaggeratedly, particularly the forelimbs.

Astasia

Balance disorder at rest. The dog has difficulty standing and displays a widened base of support.

Abasia

Difficulty or inability to walk, with possible falls to either side (symmetrical ataxia).

Swaying

Swaying of the body and head.

Other manifestations

  • Hindlimb stiffness: bunny-hopping during running (both hind limbs landing simultaneously), exaggerated jumps and falls when the dog is standing on its hindquarters.
  • Intention tremors: observed in some dogs, particularly after sudden movements or when the dog is excited.
  • Proprioception: generally normal, but may be reduced in the hindlimbs in rare cases.
  • Motor strength and spinal reflexes: normal.

Head abnormalities

  • Temporary head tilt to the right or left
  • Reduced or absent menace response (vision and facial motor function preserved)
  • Vertical, horizontal or rotatory nystagmus (often present during certain stimulations)
  • No cranial nerve deficits

Disease progression

Aggravating factors: Neurological signs are exacerbated by excitement, lifting of the head, rolling onto the back, sudden movements or challenging situations.

Duration of progression:

  • Hereditary cerebellar ataxia progresses over several months or even years
  • Dogs become unable to move after a period of 6 months to more than 8 years
  • For the majority of dogs: between 2 and 4 years
  • The disease sometimes progresses in episodes with stable periods in between

Mode of transmission

3 possible statuses:

Clear (normal homozygote)

Represented by the colour white

Carrier (heterozygote)

Represented by red and white

Affected (mutant homozygote)

Represented by the colour red

Breeding combinations:

  • Clear male + clear female = 100% clear puppies
  • Clear male + carrier female = 50% clear puppies, 50% carrier puppies
  • Clear male + affected female = 100% carrier puppies
  • Carrier male + carrier female = 25% clear puppies, 50% carrier puppies, 25% affected puppies
  • Carrier male + affected female = 50% carrier puppies, 50% affected puppies
  • Affected male + affected female = 100% affected puppies

In summary:

Strongly advised against

It is strongly advised against breeding a dog (male or female) that is affected by this disease — i.e. that carries a homozygous mutation.

Possible with precautions

It is perfectly possible to breed a healthy heterozygous carrier, but in this case it is imperative to choose a clear partner — i.e. a normal homozygote.

Mandatory testing

ALL puppies born from a pairing between a clear dog and a carrier must be genetically tested, as 50% of them will be healthy heterozygous carriers and will in turn pass on the defective copy of the ataxia gene.

At-risk pairings

For at-risk pairings (pairings that will produce 25% affected puppies), a club dispensation will be required!

We understand that it would be a shame to lose the physical qualities of a sire or dam that can contribute to the breed. However, the dog in question MUST be paired with a partner that is clear for ataxia!