Severe Subacute Necrotizing Encephalopathy (Leigh-like Syndrome) in American Staffordshire Bull Terrier Dogs

SummarySeventeen American Staffordshire bull terrier puppies, 6–8 weeks of age, from seven closely related litters, presented with rapidly progressive central vestibular neurological signs. Previously reported hereditary ataxias in the breed, including l-2 hydroxyglutaric aciduria and cerebellar cortical degeneration, as well as thiamine deficiency, were excluded. Elevated lactate levels and lactate:pyruvate ratios gave supporting evidence of a defect of the respiratory chain or Leigh-like syndrome. Histopathology in all cases showed a bilaterally symmetrical necrotizing encephalopathy, with malacia of the neuropil centred on the vestibular and olivary nuclei of the brainstem. This is the first documentation of a heritable rapidly progressive lethal necrotizing encephalopathy consistent with Leigh-like syndrome, in American Staffordshire bull terrier dogs.