title: Severe subacute necrotizing encephalopathy (Leigh-like syndrome) in American Staffordshire bull terrier dogs.
abstract: Seventeen 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.
authors.affiliation: Animal Referral Hospital, Homebush NSW 2140, Australia.
authors.forename: D
authors.initials: D
authors.lastname: Collins
authors.forename: J M
authors.initials: JM
authors.lastname: Angles
authors.forename: J
authors.initials: J
authors.lastname: Christodoulou
authors.forename: D
authors.initials: D
authors.lastname: Spielman
authors.forename: S A
authors.initials: SA
authors.lastname: Lindsay
authors.forename: J
authors.initials: J
authors.lastname: Boyd
authors.forename: M B
authors.initials: MB
authors.lastname: Krockenberger
mesh_terms: D000818:Animals; D001933:Brain Stem / Q000473:pathology*; D004283:Dog Diseases / Q000097:blood / Q000473:pathology*; D004285:Dogs; D019344:Lactic Acid / Q000097:blood; D007888:Leigh Disease / Q000097:blood / Q000473:pathology / Q000662:veterinary*; D019289:Pyruvic Acid / Q000097:blood
chemical_list: D019344:Lactic Acid; D019289:Pyruvic Acid