Review
doi: 10.1055/s-2008-1052480.
The syndrome of myoclonic epilepsy with ragged-red fibers. Report of a case and review of the literature
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- PMID: 3122068
- DOI: 10.1055/s-2008-1052480
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Review
The syndrome of myoclonic epilepsy with ragged-red fibers. Report of a case and review of the literature
Neuropediatrics.
1987 Nov.
Abstract
A patient with intention and action myoclonus, epilepsy, ataxia, and mental deterioration in association with ragged-red fibers in striated muscle is described. This patient demonstrated a unique form of erratic myoclonus with continuous EEG spike activity during eye closure. Both the myoclonus and the EEG spiking disappeared on opening the eyes. A defect in the activity of complex I in the respiratory chain was demonstrated.
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