Myoclonus epilepsy with ragged-red fibers
Jun. 10, 2021
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The first reports concerning absence seizures with severe clonic or myoclonic jerks appeared in 1966 (07). Only a few years later, however, myoclonic absences were recognized as a specific seizure type (12) and proposed as the essential feature of a distinct syndrome (10).
The 1989 revised international classification of epilepsies and epileptic syndromes placed this syndrome under "cryptogenic or symptomatic generalized epilepsies and syndromes" (02). A subsequent publication from the same commission tentatively placed this among the so-called idiopathic generalized epilepsies (05). Most recently, this syndrome has been included under the heading of genetic generalized epilepsy because of the increased incidence of family members with epilepsy, the normal initial development of many children, and the appearance of stereotyped or regular generalized spike-wave discharges superimposed on a normal EEG background (06).
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