Progressive Early-Onset Cerebellar Ataxia

Body function:

Muscle & Neurological

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Progressive early-onset cerebellar ataxia is a hereditary neurological disorder that primarily affects Finnish Hounds, though it has also been observed in Norrbottenspets. This condition is caused by a mutation in the SEL1L gene, which plays a role in the endoplasmic reticulum-associated protein degradation (ERAD) machinery. The mutation results in progressive degeneration of the cerebellum, a brain region crucial for motor control and coordination.

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List of affected breeds:

Genetic research has linked this gene and condition in the following breeds:

Finnish Hound

A black and white dog with a happy expression is looking up at the camera with its mouth open. The dog's ears are perked up, and it appears to be excited.

What does this mean for a dog with this trait?

There is no cure for this condition. Managing progressive early-onset cerebellar ataxia involves supportive care to maintain the affected dog's quality of life.

Symptoms

Dogs with progressive early-onset cerebellar ataxia exhibit significant motor impairment due to the degeneration of the cerebellum. The symptoms typically become evident by around three months of age and deteriorate quickly. Affected dogs struggle with controlling their leg movements and maintaining balance, leading to uncoordinated and erratic movements. As the disease progresses, it severely impairs the dog's ability to perform everyday activities, including eating. The progressive nature of the disease often results in affected dogs being euthanized before they reach adulthood, as their quality of life deteriorates significantly.

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