Epilepsy with myoclonic absences

Douglas R Nordli Jr MD (Dr. Nordli of University of Southern California, Keck School of Medicine has no relevant financial relationships to disclose.)
Jerome Engel Jr MD PhD, editor. (Dr. Engel of the David Geffen School of Medicine at the University of California, Los Angeles, has no relevant financial relationships to disclose.)
Originally released October 18, 1993; last updated April 20, 2016; expires April 20, 2019

Historical note and terminology

The first reports concerning absence seizures with severe clonic or myoclonic jerks appeared in 1966 (Gibberd 1966). Only a few years later, however, myoclonic absences were recognized as a specific seizure type (Tassinari et al 1969) and proposed as the essential feature of a distinct syndrome (Tassinari and Bureau 1985).

The 1989 revised international classification of epilepsies and epileptic syndromes placed this syndrome under "cryptogenic or symptomatic generalized epilepsies and syndromes" (Commission on Classification and Terminology of the International League Against Epilepsy 1989). A subsequent publication from the same commission tentatively placed this among the so-called idiopathic generalized epilepsies (Engel 2001). 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 (Gallentine and Mikati 2012).

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