All tests apart from the EEG are normal. Video-EEG is the single most important procedure for the diagnosis of eyelid myoclonia with or without absences. It shows frequent high-amplitude 3–6 Hz generalized discharges of mainly polyspikes and waves. These are brief (1–6 s, commonly 2 or 3 s) and they are typically related to eye closure, i.e. they occur immediately (within 0.5–2 s) after closing the eyes in an illuminated recording room. Eyelid myoclonia of varying severity often occurs during these EEG discharges. Photoparoxysmal discharges induced by photic stimulation occur in all untreated young patients, but may be absent in older patients or those on medication. Sleep EEG patterns are normal and generalized discharges are more likely to increase during sleep, but may also decrease. The EEG and clinical manifestations deteriorate consistently after awakening. A normal EEG is rare, even in well-controlled patients. Classification The International League against Epilepsy has not yet recognized<unk>  despite overwhelming evidence of its existence. Instead, only ‘eyelid myoclonia’ has been accepted as an “absence seizure with special features”. Differential diagnosis The diagnosis of<unk>  is simple because the characteristic eyelid myoclonia, if seen once, will never be forgotten or confused with other conditions. Furthermore, the EEG with the characteristic eye-closure-related discharges and photosensitivity leaves no room for diagnostic error. Nevertheless, eyelid myoclonia is often misdiagnosed as facial tics, sometimes for many years. The symptom/seizure of eyelid myoclonia alone is not sufficient to characterise<unk> , as it may also occur in symptomatic and cryptogenic epilepsies, which are betrayed by developmental delay, learning difficulties, neurological deficits, and abnormal MRI and background EEG.
Jeavons syndrome
Larsen syndrome
Peters-plus syndrome
McGillivray syndrome
Bogart–Bacall syndrome
Favre–Racouchot syndrome
Bowen–Conradi syndrome
Van Wyk and Grumbach syndrome