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Brain, Vol. 112, No. 5, 1261-1276, 1989
© 1989 Guarantors of Brain

research-article

MYOCLONUS EPILEPSY AND RAGGED-RED FIBRES (MERRF)

2ELECTROPHYSIOLOGICAL STUDIES AND COMPARISON WITH OTHER PROGRESSIVE MYOCLONUS EPILEPSIES

NORMAN SO, SAMUEL BERKOVIC, FREDERICK ANDERMANN, RUBEN KUZNIECKY, DANIEL GENDRON and LUIS FELIPE QUESNEY

Montreal Neurological Institute, and the Neurology and Neurosurgery Department, McGill University Montreal, Canada

Correspondence to: Correspondence to: Dr Frederick Andermann, Montreal Neurological Institute, 3801 University Street, Montreal, Quebec, Canada H3A 2B4

Electrophysiological studies (EEG, evoked potentials, nerve conduction and EMG) in 13 patients with myoclonus epilepsy with ragged red fibres (MERRF) are presented The most notable findings are the presence of atypical irregular generalized spike and wave discharges arising from an abnormal EEG background (9 patients), focal epileptiform abnormalities (6 patients) most commonly over the occipital regions and giant cortical SEPs (4 of 6 patients tested). Similar findings of disturbed EEG background activity, generalized spike and wave discharges and giant cortical SEPs appear to be shared by the group of diseases characterized by progressive myoclonus epilepsy.

Received October 20, 1987. Revised November 8, 1988. Accepted December 7, 1988.


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