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  • Updated 02.22.2021
  • Released 07.13.1999
  • Expires For CME 02.22.2024

Eyelid myoclonia with and without absences

Introduction

Overview

Eyelid myoclonia with or without 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 (Jeavons syndrome) of reflex epilepsy, which is genetically determined, has age-related onset, and affects otherwise normal children, with a female preponderance. Jeavons syndrome 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.

• Jeavons syndrome 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 (81).

In 1977, Jeavons described “eyelid myoclonia and absences” as follows (48):

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... All patients are photosensitive. The mean age of onset is 6 years.

Eyelid myoclonia with absences has been studied extensively (28; 46; 05; 09; 36; 44; 73; 75; 89; 87; 35; 71; 72; 20; 22; 23; 50; 18; 19; 79; 96; 66). In recognition of Jeavons’s contribution, Duncan and Panayiotopoulos proposed the name “Jeavons syndrome” for eyelid myoclonia with absences in a book devoted to this condition (33); see also (71; 72; 20; 22; 23; 87; 86). However, the ILAE has not, as yet, recognized Jeavons syndrome (eyelid myoclonia and absences) as a separate syndrome.

The latest ILAE position paper of the operational classification of seizure types divides generalized seizures into motor and nonmotor (absence) seizures. Nonmotor (absence) seizures are typical or atypical, or seizures that present prominent myoclonic activity or eyelid myoclonia (40; 39). However, this ILAE operational classification of epileptic seizures has been criticized by a number of experts (94; 59). A comprehensive assessment of absence seizures concluded the following (94): “The classification as “generalized nonfocal and nonmotor (absence) seizure” does not covey the complex semiology of a patient's clinical events.”

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; 71; 20; 47; 14).

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