Diagnosis and Definition of MAE

Myoclonic-Astatic Epilepsy (MAE) was first described and identified in the late 1960s by Dr. Hermann Doose as a unique epilepsy syndrome, hence its original label, Doose Syndrome.In 1989, the International League Against Epilepsy classified it formally as a “symptomatic generalized epilepsy”, and 20 years later it was renamed “epilepsy with myoclonic-atonic seizures.” Before the delineation of MAE by Dr. Doose and its final recognition by the ILAE, it is evident that basically all MAE cases were classified as LGS, which still represents the most important differential diagnosis.