Acta Neuropathologica

, 118:697 | Cite as

Leigh-like subacute necrotising encephalopathy in Yorkshire Terriers: neuropathological characterisation, respiratory chain activities and mitochondrial DNA

  • Kerstin Baiker
  • Sabine Hofmann
  • Andrea Fischer
  • Thomas Gödde
  • Susanne Medl
  • Wolfgang Schmahl
  • Matthias F. Bauer
  • Kaspar Matiasek
Original Paper


Our knowledge of molecular mechanisms underlying mitochondrial disorders in humans has increased considerably during the past two decades. Mitochondrial encephalomyopathies have sporadically been reported in dogs. However, molecular and biochemical data that would lend credence to the suspected mitochondrial origin are largely missing. This study was aimed to characterise a Leigh-like subacute necrotising encephalopathy (SNE) in Yorkshire Terriers and to shed light on its enzymatic and genetic background. The possible resemblance to SNE in Alaskan Huskies and to human Leigh syndrome (LS) was another focus of interest. Eleven terriers with imaging and/or gross evidence of V-shaped, non-contiguous, cyst-like cavitations in the striatum, thalamus and brain stem were included. Neuropathological examinations focussed on muscle, brain pathology and mitochondrial ultrastructure. Further investigations encompassed respiratory-chain activities and the mitochondrial DNA. In contrast to mild non-specific muscle findings, brain pathology featured the stereotypic triad of necrotising grey matter lesions with relative preservation of neurons in the aforementioned regions, multiple cerebral infarcts, and severe patchy Purkinje-cell degeneration in the cerebellar vermis. Two dogs revealed a reduced activity of respiratory-chain-complexes I and IV. Genetic analyses obtained a neutral tRNA-LeuUUR A-G-transition only. Neuropathologically, SNE in Yorkshire Terriers is nearly identical to the Alaskan Husky form and very similar to human LS. This study, for the first time, demonstrated that canine SNE can be associated with a combined respiratory chain defect. Mitochondrial tRNA mutations and large genetic rearrangements were excluded as underlying aetiology. Further studies, amongst relevant candidates, should focus on nuclear encoded transcription and translation factors.


Leigh syndrome Subacute necrotizing encephalopathy Mitochondrial Respiratory chain defect Canine Yorkshire Terrier 



We thank Bettina Treske and Karin Stingl for excellent technical assistance. This work was supported by the Deutsche Forschungsgesellschaft to M.F·B. (Ba1438/3 and 4) and to S·H. (Ho2374/1-1).


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Copyright information

© Springer-Verlag 2009

Authors and Affiliations

  • Kerstin Baiker
    • 1
  • Sabine Hofmann
    • 2
  • Andrea Fischer
    • 3
  • Thomas Gödde
    • 4
  • Susanne Medl
    • 5
  • Wolfgang Schmahl
    • 1
  • Matthias F. Bauer
    • 6
  • Kaspar Matiasek
    • 1
    • 7
  1. 1.Chair of General Pathology and Neuropathology, Institute of Veterinary PathologyLudwig-Maximilians University of MunichMunichGermany
  2. 2.Institute of Microbiology & Laboratory DiagnosticsGautingGermany
  3. 3.Section of Neurology, Small Animal Medical ClinicLudwig-Maximilians University of MunichMunichGermany
  4. 4.Small Animal Referral PracticePidingGermany
  5. 5.Small Animal Referral ClinicBabenhausenGermany
  6. 6.Institute of Clinical Chemistry and Molecular DiagnosticsLudwigshafen City HospitalLudwigshafenGermany
  7. 7.Neuropathology LaboratoryThe Animal Health TrustNewmarketUK

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