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Brain, Vol. 113, No. 2, 447-462, 1990
© 1990 Oxford University Press

research-article

PERIPHERAL NEUROPATHY IN LEIGH'S DISEASE

J. M. JACOBS1,, B. N. HARDING1,2, B. D. LAKE2, J. PAYAN3 and J. WILSON

1Institute of Neurology, National Hospital Queen Square, London 2Institute of Child Health London 3Hospital for Sick Children London

Correspondence to: Correspondence to: Dr J. M. Jacobs, Department of Neuropathology, Institute of Neurology, Queen Square, London WCIN 3BG.

Sural nerves were examined in 3 childhood cases of Leigh's disease (from 2 families), each with electrophysiological documentation of peripheral neuropathy. Postmortem confirmation of Leigh's disease was made in 2 cases; the third had characteristic CT scan appearances. Myelinated fibre densities were within normal limits, with no evidence of degeneration or regeneration. The myelin sheaths, however, appeared thin and this was confirmed quantitatively. In teased fibres there was no demyelination in 2 cases but some in 1 case. The findings suggest that there is hypomyelination of peripheral nerves leading eventually to demyelination.

Received February 14, 1989. Revised April 21, 1989. Accepted May 22, 1989.


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