Eyelid myoclonia, not the absences, is the hallmark of<unk> . Eyelid myoclonia consists of marked jerking of the eyelids often associated with jerky upwards deviation of the eyeballs and retropulsion of the head (eyelid myoclonia without absences). This may be associated with or followed by mild impairment of consciousness (eyelid myoclonia with absences). The seizures are brief (3–6 s), and occur mainly and immediately after closing of the eyes (eye closure) and consistently many times a day. All patients are photosensitive. Generalised tonic-clonic seizures, either induced by lights or spontaneous, are probably inevitable in the long term and are provoked particularly by precipitating factors (sleep deprivation, alcohol) and inappropriate AED modifications. Myoclonic jerks of the limbs may occur, but are infrequent and random. Eyelid myoclonic status epilepticus, either spontaneous (mainly on awakening) or photically induced, occurs in a fifth of patients. It consists of repetitive and discontinuous episodes of eyelid myoclonia with mild absence, rather than continuous non- convulsive absence status epilepticus. Onset is typically in childhood with a peak at age 6–8 years (range 2–14 years). There is a twofold preponderance of girls. Prevalence and incidence is probably low. Precipitating factors The most potent precipitating factor is eye closure, whether voluntary, involuntary or reflex. Most and, in some patients, all of the seizures are induced immediately after closure of the eyes in the presence of uninterrupted (non-flickering) light. Eye closure in total darkness is ineffective. Contrary to other forms of photosensitive epilepsies that are sensitive only to flickering lights, patients with<unk>  are also sensitive to bright, non-flickering lights. This is probably due to the enhancing effect of bright light on the sensitivity of eye closure. Self-induction in<unk>  Self-induced seizures in<unk>  are rare. Eyelid myoclonia is a seizure and not an attempt for self-induced seizures. These patients do not need to produce conditions of intermittent photic stimulation for self-induction. Simply closing the eyes would be more potent.
Jeavons syndrome
Larsen syndrome
Peters-plus syndrome
McGillivray syndrome
Bogart–Bacall syndrome
Favre–Racouchot syndrome
Bowen–Conradi syndrome
Van Wyk and Grumbach syndrome