Studio di una popolazione con epilessia mioclonica giovanile con sensibilità alla chiusura degli occhi

Objectives: "Eye closure sensitivity"(ECS) is a temporary epileptiform change in EEG that immediately follows the closure of the eyes, and it manifest itself as a brief, usually lasting 1-4 sec EEG discharges mainly generalized, appearing within 2-4 sec after eye closure. ECS is found in different epileptic conditions. We aimed to study a population with Juvenile Myoclonic Epilepsy (JME) in order to determine incidence, correlation and electro-clinical features of this phenomenon. Materials and Methods: Patients monitored in the Epilepsy Centre of the Department of Neurosciences, University of Catania, from January 1999 to December 2008, with diagnosis of JME, were retrospectively screened for the presence of ECS. We identified two subgroups: patients with eye closure sensitivity (ECS+) and patient without this condition (ECS-). We compared family history, clinical and EEG data, treatment and outcome of both groups. Results: 139 JME patients were enrolled (92 women and 47 men; age 32 ±9.8 years, mean +/- SD). Thirty-seven patients (26.6%) had ECS (29 women, 8 men; age of 29.9 ± 9.7 years) and 102 patients had not ECS, (63 women, 39 men; age 33±9.7 years). Photoparoxysmal response to intermittent photic stimulation during EEG was detected in 54% of patients ECS+ and in 23.5% of patients ECS-. The frequency of seizures in patients ECS+ was higher (monthly in 32.4%) than that in patients ECS-(sporadic in 42.1%). Patients with ECS+ were responsive to monotherapy in only 54% of patients, whereas 80% of patients with ECS-were responsive to monotherapy. Discussion and Conclusions: Eye closure sensitivity was observed in 26.6% of JME patients and it was associated with epileptic events during daily activity. Patients with ECS also presented with higher frequency of seizures than patients without ECS and had a poorer response to monotherapy.