Fixation-off and eyes closed catamenial generalized nonconvulsive status epilepticus with eyelid myoclonic jerks

Eyelid myoclonic jerks have been described in fixation-off-sensitive (FOS) epilepsy, but their relationship to nonconvulsive status epilepticus (NCSE) or to catamenial exacerbations is little reported.

We describe a woman of normal intelligence with catamenial periods of prolonged NCSE who exhibited various intra- and interseizure thresholds of polyspike suppression when her eyes were open, with particular visual inputs and with antiepileptic drug (AED) treatment.

In one episode, on the first day of the woman's menstrual period, bursts of bilateral synchronous polyspike activity were briefly suppressed with visual fixation but were more lastingly suppressed after administration of lorazepam (LZP). During another period of NCSE, the SE was completely suppressed by visual fixation on objects and patterned checkerboard screens and by ocular convergence, was incompletely suppressed when her eyes were open in a dark room and when her eyes were open without visual fixation, but was not suppressed by mental activation alone.

FOS polyspike bursts with eyelid myoclonic jerks may exhibit catamenial exacerbations, varying from completely suppressible with visual fixation to nonsuppressible during NCSE. These findings suggest an interplay between humoral factors, AEDs, and seizure threshold in this condition.