Eyelid myoclonia

C P Panayiotopoulos MD PhD (Dr. Panayiotopoulos of St. Thomas' Hospital has no relevant financial relationships to disclose.)
Jerome Engel Jr MD PhD, editor. (Dr. Engel of the David Geffen School of Medicine at the University of California, Los Angeles, has no relevant financial relationships to disclose.)
Originally released July 13, 1999; last updated May 22, 2016; expires May 22, 2019

This article includes discussion of eyelid myoclonia, eyelid myoclonia with and without absences, and Jeavons syndrome. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

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 with absences is the defining seizure type of an idiopathic 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 type of reflex photosensitive epilepsy that is clinically marked with eyelid myoclonia.

 

• Jeavons syndrome is myoclonic, rather than absence, epilepsy.

Historical note and terminology

The first documentation of eyelid myoclonia was by Radovici and colleagues. They reported and filmed the seizures of a 20-year-old man who, from the age of 10 years, had photically induced "frequent and spasmodic blinking of the eyelids with rhythmical movements [of] both rotating and elevating of the head towards the sun" (Radovici et al 1932). In 1977 Jeavons described eyelid myoclonia with absences as a clinico-EEG entity: "The characteristic seizure is a brief episode of marked jerking of the eyelids with upwards deviation of the eyes, associated with a generalized discharge of spike-wave, and occurring on closure of the eyes. All patients are photosensitive. The mean age of onset is 6 years" (Jeavons 1977). Eyelid myoclonia with absences has been studied extensively (Dalla Bernandina et al 1989; Gobbi et al 1989; Appleton et al 1993; Bianchi and Italian League Against Epilepsy 1995; Ferrie et al 1996; Giannakodimos and Panayiotopoulos 1996; Panayiotopoulos et al 1996a; Panayiotopoulos et al 1996b; Striano et al 2002; Striano et al 2009; Ferrie 2004; Covanis 2007; Covanis 2010; Panayiotopoulos 2005; Panayiotopoulos 2010; Joshi and Patrick 2007; Capovilla et al 2009; Caraballo et al 2009; Perez-Errazquin et al 2010; Vaudano et al 2014; Nar Senol et al 2015). In recognition of Jeavons' contribution, Duncan and Panayiotopoulos proposed the name “Jeavons syndrome” for eyelid myoclonia with absences in a book devoted to this condition (Duncan and Panayiotopoulos 1996); see also (Panayiotopoulos 2005; Panayiotopoulos 2010; Covanis 2007; Covanis 2010; Striano et al 2009). However, the ILAE formal reports do not recognize Jeavons syndrome (eyelid myoclonia with absences) as a separate syndrome (Engel 2001; Engel 2006; Berg 2010). Instead, eyelid myoclonia, the seizure, is classified as a myoclonic seizure type by the ILAE Task Force as follows:

 

Eyelid myoclonia: The degree to which these recurrent events (5 to 6 Hz) are associated with impairment of consciousness has not been adequately documented, and should be. In some patients, they can be provoked by eye closure. The seizure type, however, does exist as a unique entity (Engel 2006).

Furthermore, the current ILAE proposals classify eyelid myoclonia as a type of absence seizure with special features: (1) typical, (2) atypical, and (3) absence with special features (myoclonic absence and eyelid myoclonia), and the classification is detailed below (Berg et al 2010; Commission on Classification and Terminology of the International League Against Epilepsy 2016).

Absence with eyelid myoclonia. Absence with eyelid myoclonia is absence seizures accompanied by brief, repetitive, often rhythmic, fast (4 to 6 Hz) myoclonic jerks of the eyelids with simultaneous upward deviation of the eyeballs and extension of the head. Seizures are typically very brief (less than 6 seconds in duration), and multiple seizures occur on a daily basis. Mostly awareness is retained.

 

Caution. If myoclonic seizure with abduction of the upper limbs, then consider myoclonic absence seizures.

EEG background. Please refer to specific syndrome in which this seizure type occurs.

Ictal EEG. High amplitude generalized spike-and-wave and polyspike-and-wave at a frequency of 3 to 6Hz, triggered by eye closure and intermittent photic stimulation.

 

Caution. Slow spike-and-wave (less than 2.5 Hz) is not seen.

Differential diagnosis.

 

• Myoclonic absence: 3 Hz myoclonic jerks of upper limbs with tonic abduction.
• Typical absence (with eyelid flutter)

Related syndromes.

 

• Epilepsy with eyelid myoclonias
• Occasionally seen in other genetic generalized epilepsies and Dravet syndrome

A genetic generalized epilepsy is an epilepsy with generalized seizures associated with generalized epileptiform EEG patterns, such as generalized spike wave activity (Commission on Classification and Terminology of the International League Against Epilepsy 2016).

In this article and other publications (Burneo et al 2004; Panayiotopoulos 2005; Covanis 2007; Hannawi et al 2014; Brinciotti and Matricardi 2015), a significant number of video-EEG recordings are included to document that eyelid myoclonia alone is not associated with impairment of consciousness, whereas in eyelid myoclonia with absences, by definition consciousness is mildly impaired (absence).

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