Jeavons first described eyelid myoclonia (EM) in 1977; thereafter Jeavons syndrome (JS) or EM with absences (EMA) has been one of the underreported epileptic syndromes characterized by (1) EM; (2) eye closure–induced seizures/electroencephalography (EEG) paroxysms; and (3) photosensitivity (Jeavons, 1977; Panayiotopoulos, 2005a; Covanis, 2010).
First, EM is a main seizure type for JS but is also observed in other idiopathic generalized epilepsies or cryptogenic and symptomatic epilepsies (Panayiotopoulos, 2005b). Second, eye closure–induced seizures/EEG paroxysms occur within 2–4 s after closing the eyes. This eye closure–induced seizure is seen only in the presence of uninterrupted light but not triggered by simple eye blinking (Panayiotopoulos, 2005a). Eye closure–induced seizures have been reported in either idiopathic generalized epilepsy or idiopathic occipital lobe epilepsy (Sevgi et al., 2007). Third, photosensitivity, including photosensitive seizures or photoparoxysmal EEG findings, is triggered by intermittent photic stimulation (IPS). The photosensitivity, however, is reduced with age and suppressed by antiepileptic medication (Striano et al., 2002; Panayiotopoulos, 2005b). In contrast to other photosensitive epilepsies, JS patients are sensitive to both flickering and nonflickering bright light (Panayiotopoulos, 2005b).
According to the 2006 International League Against Epilepsy (ILAE) classification of epileptic syndromes, JS was not recognized as an epileptic syndrome, but eyelid myoclonia was classified as a unique type of generalized seizures (Engel, 2006). However, it has been proposed as a reflex IGE by Panayiotopoulos (2005a) because of the presence of normal posterior dominant background activity, paroxysmal generalized ictal EEG discharges, and photosensitivity. It has also been reported as a well-defined idiopathic generalized epilepsy (Striano et al., 2009). Occipital lobe or alpha generator malfunctions were postulated as a mechanism for the eye closure–induced seizures (Panayiotopoulos, 2005a).
This article describes interictal, ictal, and clinical findings on video-EEG in pediatric patients with JS. We analyze clinical and EEG findings to determine the specific occipital lobe relation to this “eye closure–induced” reflex idiopathic generalized epilepsy of JS.