Subacute necrotising encephalopathy (SNE) in Yorkshire Terriers is a recessively inherited lethal necrotising brain disease affecting dogs of both sexes less than 1 year of age. Clinically, the disease is characterized by gait abnormalities, central visual deficits, and reduced mental state. Neuropathologically, SNE in Yorkshire Terriers is nearly identical to the Alaskan Husky form and very similar to human Leigh syndrome. We identified the genetic cause of SNE and developed a DNA test, which allows the unequivocal diagnosis of carriers.