Eyelid myoclonia in a child (video-EEG)

This video-EEG of a 10-year-old girl shows all types of ictal manifestations of Jeavons syndrome. There are frequent seizures consisting of eyelid fluttering and eyelid myoclonia, sometimes associated with myoclonic jerks, which could also occur independently. The seizures were spontaneous or mainly elicited by eye closure and photic stimulation. Sequence 1. Two of her habitual seizures occurred spontaneously after the end of the video-EEG, when she was combing her hair close to a window and facing the sun. Sequence 2. One of her typical habitual daily seizures was captured on video-EEG. On slow motion study, closing of the eyes was followed within half a second by three fast eyelid movements while the eyes remained closed. Opening of the eyes coincided with the first spike of the generalized discharge. During some of the seizures, there was also myoclonic jerking of limbs. The opening and closing of the eyes was an ictal event as it started together with the spike and wave. It is important to note the manifestations of the first half second without EEG abnormalities. Similar clinical events also occurred during intermittent photic stimulation immediately preceding the generalized discharges when closing the eyes on command. It seems that these were also ictal events induced by eye closure and light. However, one cannot completely deny the possibility of a light-dependent tic that may have generated a seizure. Certainly, these were not deliberate attempts for self-induction. Sequence 3. Generalized EEG discharges were associated with eyelid clinical manifestations of varying intensity and severity. These were mainly elicited by eye closure and intermittent photic stimulation. (Contributed by Dr. C P Panayiotopoulos.)