Mitochondrial Cytopathy or Leigh's Syndrome?

J. Egger; C. J. E. Wynne-Williams; Magda Erdohazi

doi:10.1055/s-2008-1059627

Neuropediatrics, Inhaltsverzeichnis

Neuropediatrics 1982; 13(4): 219-224
DOI: 10.1055/s-2008-1059627

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

Abstract

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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

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