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Neuropediatrics 1982; 13(4): 219-224
DOI: 10.1055/s-2008-1059627
© Georg Thieme Verlag KG Stuttgart · New York

Mitochondrial Cytopathy or Leigh's Syndrome?

Mitochondrial Abnormalities in Spongiform EncephalopathiesJ.  Egger1 , C. J. E. Wynne-Williams2 , Magda  Erdohazi3
  • 1Department of Neurology and Immunology, The Hospital for Sick Children, Great Ormond Street, and Institute of Child Health, Guildford Street, London, England
  • 2Department of Paediatrics, General Hospital, Northampton, England
  • 3Department of Neuropathology, The Hospital for Sick Children, Great Ormond Street, and The National Hospital, Queen Square, London, England
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Publikationsdatum:
19. März 2008 (online)

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Abstract

A boy with mitochondrial cytopathy and neuropathological changes of subacute necrotizing encephalopathy is reported. Conditions with abnormal mitochondria in muscle and/or brain are reviewed and the role of mitochondrial abnormalities in spongiform encephalopathies is discussed. It is suggested that electronmicroscopical and histochemical investigations of muscle tissue would be of value in patients with Leigh's syndrome or other forms of spongiform encephalopathies.

Key words

Mitochondrial cytopathy - Leigh's syndrome - Spongiform encephalopathy