ICD-11

Childhood epileptic syndrome characterised by absence seizures associated with severe rhythmic bilateral myoclonic jerks. The EEG pattern shows rhythmic, bilateral, synchronous, symmetric 3-Hz spike and slow-waves discharges associated with EMG myoclonic bursts at 3 Hz, superimposed to a progressively increasing tonic contraction.

Two distinct forms may be identified: a form in which myoclonic absences are the sole or predominant seizure type and a form in which myoclonic absences are associated with other seizure types (particularly generalized tonic-clonic), the latter with poor seizure control and neuropsychological deterioration.