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Mandatory seizures

Eyelid myoclonias occur, these are brief, repetitive, often rhythmic, fast (4-6 Hz) myoclonic jerks of the eyelids with simultaneous upward deviation of the eyeballs and extension of the head. Seizures are brief (<6 seconds) but frequent (multiple times a day) and resistant to treatment. Eyelid myoclonias are induced by eye closure. All patients are photosensitive, resulting in light induction of seizures in daily life e.g. on going into sunlight. Eyelid myoclonic status epilepticus may occur (seen in up to one fifth of patients, repetitive and discontinuous episodes of eyelid myoclonias with mild impairment of awareness and responsiveness).

Eyelid myoclonias may or may not be associated with impairment in awareness, absences with eyelid myoclonia. If absences with eyelid myoclonias occur, the impairment in awareness is usually mild.

May have
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