Myoclonus epilepsy and ragged-red fibres (MERRF). 1. A clinical, pathological, biochemical, magnetic resonance spectrographic and positron emission tomographic study.

@article{Berkovic1989MyoclonusEA,
  title={Myoclonus epilepsy and ragged-red fibres (MERRF). 1. A clinical, pathological, biochemical, magnetic resonance spectrographic and positron emission tomographic study.},
  author={Samuel F. Berkovic and Stirling Carpenter and Andrew Stephen Evans and George Karpati and Eric A Shoubridge and Frederick Andermann and E. F. Meyer and Jane L. Tyler and Mirko Diksic and Dorian Arnold},
  journal={Brain : a journal of neurology},
  year={1989},
  volume={112 ( Pt 5)},
  pages={1231-60}
}
Thirteen patients, including 6 from one family, with the syndrome of myoclonus epilepsy and ragged-red fibres (MERRF) were studied. There was considerable heterogeneity in the age of onset, severity and associated clinical features. Postmortem studies in 3 patients from the one family showed a particular system degeneration. In addition, the youngest and most severely affected family member showed the pathological changes of Leigh's syndrome. Cortical dysfunction is a prominent clinical feature… CONTINUE READING
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