Spinocerebellar Ataxia (Parson Russell Terrier)

Body function:

Muscle & Neurological

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Spinocerebellar ataxia (SCA), or late-onset ataxia (LOA), is an inherited neurological disorder first identified in the Parson Russell Terrier. This condition results from a mutation in the CAPN1 gene. The disorder is characterized by a progressive loss of coordination and balance, typically beginning between six and twelve months of age. Affected dogs may experience worsening symptoms over time, and the disease often progresses to a point where euthanasia is considered due to severe impairment of mobility and overall quality of life.

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List of affected breeds:

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

Parson Russell Terrier

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What does this mean for a dog with this trait?

Managing spinocerebellar ataxia focuses on providing supportive care to maintain the dog’s quality of life as long as possible. Since there is no cure for the disorder, treatment primarily aims to alleviate symptoms and manage complications. This may involve medications to control tremors or other symptoms and environmental modifications to ensure the dog's safety and comfort. Regular monitoring by a veterinarian is essential to adjust care as the disease progresses. Given the progressive nature of the disorder and the significant decline in mobility, euthanasia is often considered when the dog's quality of life deteriorates to an unacceptable level.

Symptoms

The primary symptoms of spinocerebellar ataxia/ late-onset ataxia include uncoordinated gait characterized by stilted, "toy soldier" leg movements. Dogs may also display intention tremors, where a limb or head shakes more violently as it reaches its target. At rest, affected dogs may exhibit truncal sway, where the spine and associated muscles struggle to maintain balance, leading to a noticeable swaying motion. Additionally, dogs may experience nystagmus, a rhythmic flicking of the eyes, due to impaired muscle control. As the disease progresses, mobility becomes increasingly difficult, and dogs may develop severe motor dysfunction, eventually necessitating euthanasia.

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