Hereditary Ataxia (HA) is a neurodegenerative disease that affects the cerebellum causing progressive gait disturbance in both humans and dogs.
Old English Sheepdogs and Gordon Setters suffer from a juvenile onset, autosomal recessive form of canine hereditary ataxia primarily affecting the Purkinje neuron of the cerebellar cortex.
The cause of the disease is a mutation in the gene associated with the process of autophagy by which cell proteins and organelles are removed and recycled. The defect in the autophagy process results in neuronal death.
Symptoms often present in dogs aged 6 months - 4 years old. The characteristic symptom is pronounced hypermetria. Gait disturbances and intention tremor may also be seen.