EPILEPSY WITH EYELID MYOCLONIA (EEM)

Overview

This syndrome (previously known as Jeavons syndrome) is characterized by frequent eyelid myoclonias, with or without absences, induced by eye closure and photic stimulation.

Clinical context

This syndrome is characterized by onset of seizures between 2-14 years (peak 6-8 years). Both sexes are affected with a female predominance (2:1). Antecedent and birth history is normal. Head size and neurological examination are normal. Development and cognition are typically normal although individuals with borderline intellectual functioning and intellectual impairment are seen. Seizures are often, but not always, drug resistant. In adult life, eyelid myoclonia may continue without EEG ictal pattern, as a movement disorder.

Epilepsy with eyelid myoclonia with prominent photic induction

A subgroup of patients are exist with this syndrome who have prominent light-seeking behaviour and photic induction of eyelid myoclonia with/without absence or myoclonic seizures. This was previously termed 'sunflower syndrome' due to sun-seeking behaviour - children would seek and turn their faces to the sun or other light source, and wave their hands in front of their eyes at the onset of seizures. Intellectual impairment and attentional problems are more common in this group.