Issue Description The cerebellum is the part of the brain that
regulates the control and coordination of movement. In this condition,
cells in the cerebellum mature normally before birth, but then
deteriorate prematurely causing clinical signs associated with poor
coordination and lack of balance. The Purkinje cells in the cerebellum
are primarily involved; cells in other areas of the brain may also be
affected. Other Names CA, Cerebellar Abiotrophy, CCA, Neonatal CA, Cerebellar Cortical Abiotrophy, Cerebellar and Extrapyramidal Nuclear Abiotrophy
Causes CA is usually an autosomal recessive gene, but
in a few breeds, such as the English Pointer, the gene is sex-linked.
Symptoms The clinical signs of cerebellar dysfunction in
affected dogs may include poor balance, a wide-based stance (feet
planted far apart), stiff or high-stepping gait, apparent lack of
awareness of where the feet are (standing or walking with a foot
knuckled over), and head or body tremors. Affected dogs may become
unable to climb stairs or stand without support. They have normal
Where other regions of the brain are also
affected, you may see signs such as behavior change (loss of house
training, aggression), confusion, blindness, and seizures.
Diagnosis This is a rare disorder. The clinical signs are
suggestive of cerebellar disease, particularly if they are seen in a
breed in which abiotrophy is known to occur. Your veterinarian will do
tests to rule out other conditions that can cause similar signs.
Routine diagnostic tests are normal with this condition and a
definitive diagnosis can only be made by brain biopsy or post-mortem.
MRI may be helpful in dogs in which there is gross cerebellar
malformation; however generally with this condition, the cerebellum
appears grossly normal. Histopathologic abnormalities are often
minimal and do not seem to correlate with the severity of cerebellar
Treatment There is no treatment for this condition. Dogs
do not recover from this disorder and usually at some point (depending
on the rate of the progressive deterioration that occurs), euthanasia
becomes the best option.
Breeds Affected By CA Neonatal CA (very rare)
- Affected cells start to degenerate before birth, so that signs of
cerebellar dysfunction are present at birth or when the pup first
Postnatal CA - Cells in the cerebellum are
normal at birth and begin to degenerate at variable times thereafter.
Labrador Retriever - Clinical signs are first seen at 6 to 12
weeks, and the condition worsens quickly (over a few weeks).
Airedale - There is early onset (12 weeks of age) and a slow
progression of clinical signs.
Bern Running Dog
Bernese Mountain Dog
German Shepherd - Signs are seen by 6 months of age.
Gordon Setters - Clinical signs develop at 6 months to 2 years of
age, and the progression is slow (months to years).
Brittany spaniels - The onset of clinical signs is late (average
age 10 years), and the condition progresses slowly.
English pointers - Clinical signs develop around 8 to 12 weeks and
become pronounced by 16 months of age.
Cerebellar and extrapyramidal nuclear abiotrophy - Cells in
other regions of the brain deteriorate as well.
Kerry Blue Terrier - The first signs occur at 6 to 12 weeks and
dogs are unable to stand by 1 year of age.
Miniature Poodle - Clinical signs are seen at 4 to 12 weeks of
age, and there is rapid deterioration over a few weeks to months.