Epilepsy With Myoclonic Absences

A rare childhood-onset epilepsy syndrome characterized by sudden onset of staring and unresponsiveness, in association with rhythmical myoclonic jerks predominantly involving the superior upper limbs and leading to typical raising of the arms and shoulders. Ictal EEG shows bilateral, synchronous, symmetric 3-Hz spike-wave discharges. Other types of seizures (e. g. generalized tonic-clonic seizures) are often associated. Additional symptoms may include intellectual disability.