Sign Up for a Free Account
  • Updated 02.09.2022
  • Released 07.13.1999
  • Expires For CME 02.09.2025

Eyelid myoclonia with and without absences

Introduction

Overview

Eyelid myoclonia with absences is a form of epileptic seizure manifesting with myoclonic jerks of the eyelids, often with brief absences. These seizures are mainly precipitated by closing of the eyes and lights. They occur in symptomatic, possibly symptomatic, and idiopathic generalized epilepsies. Most authors support the view that eyelid myoclonia on eye closure is the defining seizure type of an idiopathic generalized syndrome of reflex epilepsy, which is genetically determined, has age-related onset, and affects otherwise normal children, with a female preponderance. 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, 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.

• Eyelid myoclonia with absences is a generalized idiopathic epilepsy 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 type in Jeavons syndrome.

Historical note and terminology

The first documentation of eyelid myoclonia was by Radovici and colleagues (87).

In 1977, Jeavons described “eyelid myoclonia and absences” as follows (50):

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 (30; 48; 05; 09; 38; 46; 79; 81; 97; 95; 37; 77; 78; 21; 23; 24; 52; 18; 19; 85; 103; 71). However, eyelid myoclonia with absences (previously known as Jeavons syndrome) has been recognized as a distinct epilepsy syndrome by the International League Against Epilepsy (93). The mandatory seizures are brief, repetitive, often rhythmic, fast (4-6 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 (93).

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; 77; 21; 49; 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.

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

Support: service@medlink.com

Editor: editor@medlink.com

ISSN: 2831-9125