Sign Up for a Free Account
  • Updated 02.07.2024
  • Released 07.13.1999
  • Expires For CME 02.07.2027

Eyelid myoclonia with and without absences



Epilepsy with eyelid myoclonia with absences is a form of epileptic seizure manifesting with myoclonic jerks of the eyelids, often with brief absences. Epilepsy with eyelid myoclonia and absences (formerly Jeavons syndrome) is characterized by unique clinical and EEG features, which follow eye closure in the light and are pathognomonic of this syndrome. Slow eye closure (voluntary or on command) in the light induces eyelid myoclonia, upward deviation of the eyeballs and head, in association with generalized polyspike-wave or spike-wave EEG discharges 3 to 6 Hz. The concomitant absence may be conspicuous or inconspicuous relevant to the duration of brief generalized discharge. These discharges and clinical phenomena are invariably evoked during intermittent photic stimulation, not only on eye closure but also with the eyes opened. The electroclinical phenomena that follow eye-closure disappear when the eyes open and remain opened. Most authors support the view that eyelid myoclonia on eye closure is the defining seizure type of a generalized epilepsy syndrome, which is genetically determined, has age-related onset, and affects otherwise normal children, with a female preponderance. Epilepsy with eyelid myoclonia with absences is probably lifelong, with continuing seizures in adult life. Eyelid myoclonia is often confused with facial tics or self-induction of seizures. In this article, the author details developments in the clinical manifestations, pathophysiology, genetics, and pharmacological treatment of eyelid myoclonia with absences.

Key points

• Eyelid myoclonia with absences is a distinct type of epileptic seizure that is often misdiagnosed as facial tics or another nonepileptic paroxysmal event of eyelid jerking.

• The characteristic eyelid myoclonia on eye closure, if seen once, will never be forgotten or confused with other conditions.

• A main misconception is that eyelid myoclonia with or without absences is an attempt to self-induce seizures.

• Epilepsy with eyelid myoclonia with absences is a genetic generalized epilepsy syndrome characterized by eyelid myoclonia and absences on voluntary or on command eye closure in the light. Eye closure in the dark or passive eye closure does not trigger clinical symptoms or EEG discharges.

• Myoclonic and absence seizures are the predominant seizure types in the syndrome epilepsy with eyelid myoclonia, previously known as Jeavons syndrome.

Historical note and terminology

The first documentation of eyelid myoclonia was by Radovici and colleagues (93).

In 1977, Jeavons described “eyelid myoclonia and absences” as follows: “There is marked jerking of the eyelids associated with 3 c/s SWDs or Poly-SWDs often irregular, immediately after eye closure and invariably positive response to intermittent photic stimulation (IPS). The mean age of onset is 6 years and female predominate...” (53):

Therefore, eyelid myoclonia and absences show a marked jerking of the eyelids immediately after eye-closure and there is an associated brief bilateral spike and wave activity. The eyelid movement is like rapid blinking and the eyes deviate upwards, in contrast to the very slight flicker of eyelids which may be seen in a typical absence in which the eyes look straight ahead. Brief absences may occur spontaneously and are accompanied by 3 Hz spike and wave discharges... The spike-and-wave discharge seen immediately after eye closure does not occur in the dark. Generalized spike-wave discharges evoked immediately after eye closure during the baseline EEG are a very reliable warning that abnormality will be evoked by photic stimulation. The mean age of onset is 6 years.

Eyelid myoclonia with absences has been studied extensively (32; 50; 05; 09; 40; 48; 85; 87; 106; 104; 39; 83; 84; 23; 25; 26; 55; 18; 19; 91; 114; 76). However, eyelid myoclonia with or without absences, induced by eye closure and photic stimulation (previously known as Jeavons syndrome) has been recognized as a distinct genetic generalized epilepsy syndrome by the International League Against Epilepsy (101). The mandatory seizures are brief, repetitive, often rhythmic, fast (4 to 6 Hz) myoclonic jerks of the eyelids with simultaneous upward deviations of the eyeballs and extension of the head. All patients are photosensitive, and eyelid myoclonic status epilepticus may occur in up to one fifth of patients (101).

Although the triad of epilepsy with eyelid myoclonia is well defined with eyelid myoclonia, eye closure-induced EEG paroxysms and seizures, and photosensitivity, the syndrome has been recognized since 1977. However, many cases are underdiagnosed or misdiagnosed (53). In a comprehensive narrative review, there was a strong consensus among panelists that eyelid myoclonia may be present for many years prior to recognition as a seizure and that eyelid myoclonia may be inappropriately diagnosed as ocular tics (99).

This article and other publications present a significant number of video-EEG recordings documenting the fact that eyelid myoclonia is the more obvious clinical manifestation and can also occur alone without impairment of consciousness (15; 83; 23; 52; 14).

This is an article preview.
Start a Free Account
to access the full version.

  • Nearly 3,000 illustrations, including video clips of neurologic disorders.

  • Every article is reviewed by our esteemed Editorial Board for accuracy and currency.

  • Full spectrum of neurology in 1,200 comprehensive articles.

  • Listen to MedLink on the go with Audio versions of each article.

Questions or Comment?

MedLink®, LLC

3525 Del Mar Heights Rd, Ste 304
San Diego, CA 92130-2122

Toll Free (U.S. + Canada): 800-452-2400

US Number: +1-619-640-4660



ISSN: 2831-9125