Le mioclonie palpebrali con assenze alla chiusura degli occhi. Una sindrome sottostimata?

Purpose. To evaluate prevalence of Eyelid myoclonia with absence (EMA) among Idiopathic generalized Epilepsies (IGE), and its clinical and evolutive characteristics. Material and method. Retrospective analysis of 2780 epileptic pts. Inclusion criteria: Eyelid myoclonia and microabsences, related to EEG generalized paroxysmal activity, and triggered by eye closure and/or intermittent photic stimulation. Results. 7.46% of pts. with IGE are classified as EMA. Female/male ratio is 1.7:1. Positive familial history is present in about an half of pts, with two pair of identical twins. Rare generalized tonic-clonic seizures occurred in most pts. Conclusions. EMA is a not infrequent IGE syndrome, and, possibly, underdiagnosed because of the paucity of clinical semiology and of the modification of EEG findings induced by AEDs. Video-EEG analysis is sometimes necessary for diagnosis of EMA.