Rola stymulacji nerwu błędnego w leczeniu epilepsji z mioklonią powieki - Seria przypadków

OBJECTIVE: To study the effectiveness of vagus nerve stimulation (VNS) in patients with epilepsy with eyelid myoclonia (EEM, previously known as Jeavons Syndrome). BACKGROUND: EEM is an epilepsy syndrome characterized by eyelid myoclonia, eyelid closure-induced generalized EEG paroxysms or seizures, and photosensitivity. Many patients have drug-resistant epilepsy; however, the effectiveness of VNS in this epilepsy syndrome has not been well studied. METHODS: This is a single institution retrospective observational study of patients with EEM. Among a database of 134 patients with EEM, we identified those treated with VNS. Epilepsy history, VNS parameters, and response to VNS were abstracted. RESULTS: We identified 12 patients who were treated with VNS (50% female). The median age of epilepsy onset was 5 (range 4-14) years; age of VNS implantation was 16 (range 6-33) years, and median duration of VNS treatment was 13.5 (range 0-24) years. Four patients (33.3%) were responders reporting greater than 50% improvement in seizure frequency. All four patients (33.3%) showed response with generalized tonic-clonic seizures, myoclonic seizures, and three patients (25%) reported response with eyelid myoclonia and absence seizures. One patient had the VNS explanted due to lack of efficacy and one had it turned off for enrollment in a clinical trial. The median maximum tolerated output current settings for our cohort were 1.75 mA (IQR 1.38-1.94; range 0.75-2.25) with an OFF time of 1.8 min (IQR 1.1-3, range 0.2-3) and median duty cycle of 24% (IQR 16-29; range 12-58). CONCLUSIONS: From this small case series of patients with EEM, four (33.3%) were responders to VNS, with improvement noted in multiple seizure types. This indicates the potential role of VNS for patients with EEM.