Progressive Retinal Atrophy with Neurodegeneration is an inherited syndromic disease affecting the Saarloos Wolfdog breed and comprising blindness and neurological deficits.
Clinical signs includes early adult onset retinal degeneration, ophthalmological findings are consistent with generalized progressive retinal atrophy (PRA), with first clinical signs being observable between 20 and 46 months of age. Symptoms include early adult-onset neurological deficits with gait abnormalities, hind limb weakness, tremors, ataxia, cognitive decline and behavioural changes such as aggression towards the owner.
Additionally, epileptic seizures were reported in some cases. Affected dogs often have to be euthanized because of progression of the neurological signs and impaired quality of life.
Histopathological examination of an affected dog demonstrated cataract, retinal degeneration, central and peripheral axonal degeneration, and severe astroglia hypertrophy and hyperplasia in the central nervous system.
