| HISTORICAL NOTE AND NOMENCLATURE
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 in recent
years (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; 1996b). In recognition
of Jeavon’s 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).
However, the current ILAE Task Force on Classification did not recognize
eyelid myoclonia with absences as a separate syndrome (Engel 2001). Eyelid
myoclonia with and without absences is the only accepted separate seizure
type, and is the topic of this chapter.
CLINICAL MANIFESTATIONS
Eyelid myoclonia with absences has two components. The initial and more
prominent is eyelid myoclonia. This may or may not progress to the second
component, which is mild impairment of consciousness (absence). The seizure
starts and ends abruptly with a duration of 3 to 5 seconds.|{video:EMACP1v.avi}{caption:Video-EEG
of eyelid myoclonia with absences}{label:The patient exhibits eyelid myoclonia
with absences mainly on eye-closure and intermittent photic stimulation.}|The
clinical manifestations of eyelid myoclonia with absences were documented
with video-EEG studies by Giannakodimos and Panayiotopoulos as follows
(Giannakodimos and Panayiotopoulos 1996):
Eyelid myoclonia
Eyelid myoclonia occurs mainly during the first second of the EEG discharge
and consists of repetitive, often rhythmic, fast (4 to 6 Hz), small- or
large-range myoclonic jerks of the eyelids. The eyelid jerks vary in force,
amplitude, and numbers—even for the same patient. In each seizure,
there are more than three repetitive eyelid jerks.|{video:EMACP1v.avi}{caption:Video-EEG
of eyelid myoclonia with absences}{label:The patient exhibits eyelid myoclonia
with absences mainly on eye-closure and intermittent photic stimulation.}||{diagram:eap1.bmp}{label:EEG
of seizure in eyelid myoclonia with absences (1)}{title:EEG of seizure
in eyelid myoclonia with absences}|Occasionally there may be only a single
jerk, but often there is a series of rapid eyelid tremor-like jerks.|{video:EMACP3v.avi}{caption:Video-EEG
of long-standing eyelid myoclonia with absences}{label:In this Video-EEG
of a woman with long-standing eyelid myoclonia with absences, eyelid myoclonia
is consistently induced by eye closure in a lit room. This is completely
abolished when eye closure is performed in total darkness achieved with
underwater goggles covered with opaque tape.}||{diagram:eap2.bmp}{label:EEG
of seizure in long-standing eyelid myoclonia with absences}{title:EEG
of seizure in long-standing eyelid myoclonia with absences}|There is typically
a concomitant tonic contraction of the eyelids, and the eyes often assume
a semi-open position, irrespective of whether the seizure started when
the eyes were open or closed. Vertical jerking and upwards deviation of
the eyeballs as well as simultaneous jerks of the eyebrows and the head
frequently occur, as does lateral deviation of the eyes and the head.
Exceptionally, eyelid myoclonia may be associated with jerks of the hands.|{video:eap3v.avi}{caption:Video-EEG
of seizure in eyelid myoclonia with absences}{label:Video-EEG of a 17-year-old
woman with eyelid myoclonia with absences. Note that hand jerking occurs
during the eyelid myoclonia.}|Rarely, the tonic component of the eyelid
semi-opening and the deviation of the eyes and head may be more predominant
than the clonic components.
Once seen, eyelid myoclonia will not be forgotten. |{video:EMACP1v.avi}{caption:Video-EEG
of eyelid myoclonia with absences}{label:The patient exhibits eyelid myoclonia
with absences mainly on eye-closure and intermittent photic stimulation.}||{video:EMACP3v.avi}{caption:Video-EEG
of long-standing eyelid myoclonia with absences}{label:In this Video-EEG
of a woman with long-standing eyelid myoclonia with absences, eyelid myoclonia
is consistently induced by eye closure in a lit room. This is completely
abolished when eye closure is performed in total darkness achieved with
underwater goggles covered with opaque tape.}||{video:eap3v.avi}{caption:Video-EEG
of seizure in eyelid myoclonia with absences}{label:Video-EEG of a 17-year-old
woman with eyelid myoclonia with absences. Note that hand jerking occurs
during the eyelid myoclonia.}||{video:eap4v.avi}{label:Video-EEG of 10-year-old
girl with eyelid myoclonia}{title:Video-EEG of 10-year-old girl with eyelid
myoclonia}|It is distinctly different from the random or rhythmic eyelid
closing or fluttering during the course of typical absences in other epileptic
syndromes (Panayiotopoulos et al 1996a).
Impairment of consciousness (absence)
Absence follows the eyelid myoclonia while the eyelid jerking continues
less violently than in the onset. Impairment of consciousness is usually
mild, manifested with cessation, repetition, errors, and delays of breath-counting
on video-EEG. Automatisms are not observed.
An interesting symptom is the rare occurrence of a "pleasurable" feeling
during the absence, which may be related to "self-induction" in some patents.
Precipitating factors of eyelid myoclonia with absences
The most potent precipitating factor is eye closure—voluntary,
involuntary, or reflex.|{video:EMACP1v.avi}{caption:Video-EEG of eyelid
myoclonia with absences}{label:The patient exhibits eyelid myoclonia with
absences mainly on eye-closure and intermittent photic stimulation.}||{video:EMACP3v.avi}{caption:Video-EEG
of long-standing eyelid myoclonia with absences}{label:In this Video-EEG
of a woman with long-standing eyelid myoclonia with absences, eyelid myoclonia
is consistently induced by eye closure in a lit room. This is completely
abolished when eye closure is performed in total darkness achieved with
underwater goggles covered with opaque tape.}|The majority of the seizures
are induced immediately after closure of the eyes in the presence of uninterrupted
light. Eye closure in total darkness is ineffective. Intermittent photic
stimulation potentates the effect of eye closure and is capable of inducing
seizures when eyes are open or closed. Photosensitivity declines with
age, whereas eye closure is likely to remain a life-long precipitating
factor (Giannakodimos and Panayiotopoulos 1996). However, there are patients
in whom eyelid myoclonia occurs only during photic stimulation.|{video:eap6v.avi}{caption:Video-EEG
and interview of a photosensitive patient not suffering from eyelid myoclonia
with absences}{label:In this Video-EEG and interview of a photosensitive
patient not suffering from eyelid myoclonia with absences, note that eyelid
myoclonia is induced by intermittent photic stimulation. She also has
self-induced seizures by rapid successive movements of the left hand in
front of her eyes while watching television or in the sun. Generalized
discharges of spike-and-wave occurred during hyperventilation with no
detectable clinical symptoms.}|
LOCALIZATION
Eyelid myoclonia with absences is a generalized epileptic seizure
with generalized multiple spike- and slow-wave discharges that may be
of higher amplitude in the anterior or posterior regions.
PATHOPHYSIOLOGY
The EEG ictal accompaniments of eyelid myoclonia with absences are
generalized discharges of mainly polyspikes and polyspike-slow waves at
a frequency of 3 to 6 Hz (usually more than 4 Hz) and a duration of 3
to 6 seconds (typically around 3 seconds and seldom more than 5 seconds).|{video:EMACP1v.avi}{caption:Video-EEG
of eyelid myoclonia with absences}{label:The patient exhibits eyelid myoclonia
with absences mainly on eye-closure and intermittent photic stimulation.}||{diagram:eap1.bmp}{label:EEG
of seizure in eyelid myoclonia with absences (1)}{title:EEG of seizure
in eyelid myoclonia with absences}||{video:EMACP3v.avi}{caption:Video-EEG
of long-standing eyelid myoclonia with absences}{label:In this Video-EEG
of a woman with long-standing eyelid myoclonia with absences, eyelid myoclonia
is consistently induced by eye closure in a lit room. This is completely
abolished when eye closure is performed in total darkness achieved with
underwater goggles covered with opaque tape.}||{diagram:eap2.bmp}{label:EEG
of seizure in long-standing eyelid myoclonia with absences}{title:EEG
of seizure in long-standing eyelid myoclonia with absences}|Polyspikes
are more abundant and are often continuous (uninterrupted by slow waves)
in the first 1 to 2 seconds from onset. Polyspike-and-slow waves usually
follow this multiple-spike opening phase of the discharge. The onset of
the EEG discharge either precedes the eyelid jerks or is simultaneous
to them (Giannakodimos and Panayiotopoulos 1996). However, on some occasions
fast eyelid movements (with eyes closed) following eye closure may precede
the discharge.|{video:eap4v.avi}{label:Video-EEG of 10-year-old girl with
eyelid myoclonia}{title:Video-EEG of 10-year-old girl with eyelid myoclonia}||{diagram:eap3.bmp}{label:EEG
of habitual seizure of eyelid myoclonia}{title:EEG of habitual seizure
of eyelid myoclonia}||{diagram:eap4.bmp}{label:EEG of photic stimulation-induced
eyelid myoclonia}{title:EEG of photic stimulation-induced eyelid myoclonia}|
The underlying pathogenesis is not known. Eyelid myoclonia with absences
mainly manifests with regional eyelid myoclonia. As opposed to other photosensitive
epilepsies, eye closure is more potent than photic stimulation as a triggering
factor. However, eye closure requires the presence of light, and it is
entirely ineffective in darkness (Giannakodimos and Panayiotopoulos 1996;
Panayiotopoulos 1998), which may explain why continuous light also triggers
seizures in these patients. Another intriguing feature is that some patients
may manifest with features of photosensitivity and fixation-off sensitivity,
which have opposing characteristics (Panayiotopoulos 1998). It is possible
that in patients with eyelid myoclonia with absences, there is a malfunctioning
of alpha-rhythm generators (Panayiotopoulos et al 1996b) and that both
the magnocellular and parvocellular systems are functionally disturbed
(Wilkins 1995).
Though eyelid myoclonia (the seizure) may occur in various conditions,
the syndrome of eyelid myoclonia with absences appears to be a genetically
determined homogeneous syndrome that may be different from most other
syndromes of idiopathic generalized epilepsy (Bianchi and Italian League
Against Epilepsy 1995; Parker et al 1996). In a large-scale genetic study
of idiopathic generalized epilepsies, Bianchi and colleagues reported
concordance of the syndrome in families of probands with eyelid myoclonia
with absences (Bianchi and Italian League Against Epilepsy 1995). Parker
and colleagues reported that of 18 patients with eyelid myoclonia with
absences, 14 had a family history of epilepsies (Parker et al 1996). Four
patients had other family members affected by a similar epileptic condition
of eyelid myoclonia with absences.
DIFFERENTIAL DIAGNOSIS
Eyelid myoclonia with or without absences is usually the prominent seizure
type of the syndrome of eyelid myoclonia with absences (Jeavons syndrome).
However, eyelid myoclonia may also occur in symptomatic or idiopathic
generalized epilepsies with absences.
Symptomatic or cryptogenic epilepsies
In symptomatic or cryptogenic epilepsies, eyelid myoclonia with absences
may be a predominant seizure type with or without photosensitivity. Diagnostic
confusion between patients with symptomatic or cryptogenic epilepsies
who exhibit ictal eyelid myoclonia and those with the syndrome of eyelid
myoclonia with absences will rarely be a problem if the clinical background
is considered in detail. Mental retardation, neurologic signs, and severely
abnormal background EEG as found in patients with symptomatic or cryptogenic
epilepsies is incompatible with an idiopathic syndrome such eyelid myoclonia
with absences. Focal EEG abnormalities are common in the syndrome of eyelid
myoclonia with absences, but when severe or persistent, they are likely
to indicate an underlying structural abnormality. Seizures in this syndrome
are brief and stereotyped; occasional independent myoclonic jerks of the
limbs or body and GTCS are the only other seizure types that occur (Giannakodimos
and Panayiotopoulos 1996). In cryptogenic or symptomatic cases, eyelid
myoclonia may be an inconstant ictal feature and may be associated with
seizures both of variable duration and of different types, such as atypical
absences and tonic, atonic, or partial seizures. Activation of seizures
by eye closure in symptomatic or cryptogenic cases may be rarer than in
the syndrome of eyelid myoclonia with absences (Ferrie et al 1996).
Idiopathic generalized epilepsies with absences
In idiopathic generalized epilepsies with absences, eyelid myoclonia
should be differentiated from other eyelid manifestations that may occur
during the absence. In particular, Panayiotopoulos and colleagues studied
90 patients with idiopathic generalized epilepsy and 536 video-EEG recordings
of typical absences (greater than 2.5 to 3 Hz spike- or polyspike-and-slow
waves with clinical manifestations) (Panayiotopoulos et al 1996a). All
patients had typical absences either alone, with myoclonic jerks or GTCS,
or with both. Syndromes included: childhood absence epilepsy, juvenile
absence epilepsy, myoclonic absence epilepsy, perioral myoclonia with
absences, phantom absences with GTCS, absences with single myoclonic jerks,
and other unclassified syndromes of idiopathic generalized epilepsy with
typical absences (Panayiotopoulos 1997). Thirty-nine (43.3%) of the 90
patients had eyelid or eye-related ictal clinical manifestations.
Only the most prominent eyelid manifestations were considered. They were
classified as consistent if they occurred in every absence of the same
patient and inconsistent if they were present in some but not all of the
absences. Symptoms often overlapped in the same patient and even for the
same seizure. It was found that
-
Eyelid myoclonia consistently occurred in five patients.
-
Eyelid fluttering consistently occurred in four patients and was
inconsistent in another four patients.
-
Random or repetitive eyelid blinks, similar to spontaneous normal
eyelid blinking, consistently occurred in six patients. Another 14
had inconsistent eyelid blinking during the absence ictus.
-
Predominantly rhythmic and vertical eyebrow oscillations were observed
in five patients.
-
One patient had vertical nystagmus.
Thus, 39 out of the 90 patients had ictal manifestations from the eyelids
and eyes, but in only five of them (5.6%) were the ictal manifestations
similar to those occurring in the syndrome of eyelid myoclonia with absences.|{diagram:eap5.bmp}{caption:EEG
of eyelid myoclonia with absences but without photosensitivity}{label:Eyelid
myoclonia and the impairment of cognition during discharge is followed
with eyelid tremor-like movements and disturbance of consciousness despite
the apparent cessation of the discharge. The duration of the clinical
seizure is indicated by the arrows. The patient was counting (numbers
are annotated at top).}|Only one of those five patients was photosensitive,
and only one experienced absences provoked by eye closure.
Conversely, 20 patients (22.2 %) had clinical or video-EEG-documented
photosensitivity, but only 5 of them had eyelid and eye-related ictal
clinical manifestations during the absences. Two patients had random eyelid
blinking, which is strikingly different from the eyelid myoclonia of Jeavons
syndrome. Two patients had fast rhythmic eyelid fluttering. One patient
had eyelid myoclonia with absences-like manifestations.
Eye closures and eyelid myoclonia-like attempts for self-induction
in photosensitive epilepsy
Eyelid myoclonia with absences, often occurring after eye closure
in the presence of light and often exaggerated by intermittent photic
stimulation, should not be considered as a self-induced maneuver (Panayiotopoulos
et al 1996b). The EEG ictal discharge precedes or coincides with the eyelid
myoclonia. These patients do not need to produce conditions of intermittent
photic stimulation for self-induction; simply closing of the eyes would
be more potent.
However, self-induced photosensitive epilepsy is well known and well documented.
These patients need to produce intermittent photic stimulation in order
to deliberately self-induce seizures. They do this by using various maneuvers
such as waving their hands in front of their eyes or with repetitive eye-closures
while looking at the sun or a bright light source. The latter may sometimes
imitate eyelid myoclonia.
A clinical vignette from one patient shows the difficulty in differentiating
genuine seizures of eyelid myoclonia from self-induced eyelid myoclonia.
This 10-year-old girl had onset of frequent daily seizures at 4 years
of age. Seizures averaged 3 to 5 seconds. They consisted of marked eyelid
myoclonia and tonic upward deviation of the eyes accompanied by, at most,
a mild impairment of consciousness. In more severe attacks there was retropulsive
jerk of head, shoulders, and sometimes limbs, which occasionally would
lead to falls. The vast majority of the seizures were light-induced with
sudden increases in background illumination, especially when going into
bright sunlight. Watching television and playing video-games were less
powerful stimuli. Occasional independent myoclonic jerks of her limbs
were described. Typical absences unaccompanied by eyelid phenomena did
not occur. She did not have GTCS (Parker et al 1996).
One of her habitual daily seizures was captured on Video-EEG.|{video:eap4v.avi}{label:Video-EEG
of 10-year-old girl with eyelid myoclonia}{title:Video-EEG of 10-year-old
girl with eyelid myoclonia}|On slow motion study, closing of the eyes
is followed within half a second by three fast eyelid movements while
the eyes remain closed. Opening of the eyes coincides with the first spike
of the generalized discharge. During some of the seizures, there is also
myoclonic jerking of limbs.
The opening and closing of the eyes is an ictal event as it starts together
with the spike and wave.|{video:eap4v.avi}{label:Video-EEG of 10-year-old
girl with eyelid myoclonia}{title:Video-EEG of 10-year-old girl with eyelid
myoclonia}||{diagram:eap3.bmp}{label:EEG of habitual seizure of eyelid
myoclonia}{title:EEG of habitual seizure of eyelid myoclonia}|It is important
to note the manifestations of the first half second without EEG abnormalities.
Similar clinical events also occurred during intermittent photic stimulation
immediately preceding the generalized discharges when closing the eyes
on command.|{diagram:eap4.bmp}{label:EEG of photic stimulation-induced
eyelid myoclonia}{title:EEG of photic stimulation-induced eyelid myoclonia}|It
seems that these are also ictal events induced by eye closure and light.
However, one cannot completely deny the possibility of a light-dependent
tick that may generate a seizure (Kent et al 1998). Certainly, these are
not deliberate attempts for self-induction.
Forced eyelid closures and eyelid fluttering in occipital seizures
Forced eyelid closure and eyelid blinking is also an ictal clinical
symptom in partial seizures described both in symptomatic and idiopathic
occipital epilepsy. It may be an early ictal sign and has a forced quality
that may be distinguished from the more casual blinking associated with
many other partial seizures. It usually occurs after the phase of visual
hallucinations, at a stage in which consciousness is impaired, and heralds
the impending secondarily generalized convulsions (Panayiotopoulos 1999a).
However, it may also occur alone, inconspicuous in appearance and not
suspected as a seizure event, documented only with video-EEG recordings
in occipital photosensitive patients (Panayiotopoulos 1999a).
Eyelid manifestations in normal people
With the possible exception of the violent myoclonic jerks of eyelid
myoclonia, all other eyelid symptoms during absence seizures are manifested
by normal people in their every day life. The eyes and the eyelids are
the most sensitive and expressive parts of our body. There is considerable
literature on the subject (Hall 1945).
From a small sample of people appearing in televised interviews, round
table discussions, and debates, Panayiotopoulos and colleagues reached
the following conclusions (Panayiotopoulos et al 1996a):
-
When under tension, the rate of the spontaneous blinks increases
significantly. In some people this may become repetitive in clusters
of 3 to 5 per second, showing close similarities to that described
in self-induced photosensitive epilepsy. This type of repetitive blinking
was apparent either at the beginning of the utterance (like a breath
before starting a sentence) or at the end (like a relief). It was
particularly apparent when talking on sensitive matters and was not
observed when the same people were not participating in the discussion.
These eyelid manifestations are like ticks, which are a motor expression
of emotional disturbance. Other normal subjects may semiclose their
eyelids with brief transient fluttering.
-
Slow and sustained eye-closure was rarely manifested. It was observed
mainly in women who were discussing their distress, anger, or frustrations.
-
Less frequently, eyes were widely opened with cessation of spontaneous
blinks.
What the eyelids do could not be simpler: they open and close.
However, this limited repertoire belies the complexity of the behavioral
requirements of the eyelids. They must protect the eyes and especially
the cornea . . . For an efficient compromise between and unobstructed
vision the eyelids must change position in synchrony with vertical
eye movements . . . Eyelid position is modulated by autonomic functions
regulating, for example, arousal. . . .They are intimately concerned
with facial expression . . . Thus, we have a simple movement which
is under voluntary, automatic, reflex, emotional, and autonomic
control (Plant 1996).
DIAGNOSTIC WORKUP
The EEG, preferably video-EEG, is the single most important diagnostic
procedure in eyelid myoclonia with absences. The EEG is dominated with
high amplitude discharges of spike-and-slow waves at 3 to 6 Hz. These
are brief (1 to 5 seconds) and are commonly associated with clinical manifestations
of eyelid myoclonia of varying severity; they occasionally appear simultaneous
with impairment of cognition. They often occur immediately (within 0.5
to 2 seconds) after closing the eyes in an illuminated recording room.
They are eliminated in total darkness. Photoparoxysmal responses are recorded
from all untreated young patients and may be absent in older patients
on medication. Photosensitivity and fixation-off sensitivity may occur
together (Panayiotopoulos 1998). A normal EEG is rare, even in well-controlled
patients. EEG discharges are also enhanced by hyperventilation.
Sleep EEG patterns are normal. Generalized discharges of polyspikes-and-slow
waves are more likely to increase during sleep, but a reduction is occasionally
observed. The discharges are shorter and devoid of discernible clinical
manifestations of any type, even in those patients who have numerous seizures
during the alert state.
EEG and clinical manifestations are consistently deteriorated after awakening.
SYNDROMES AND DISEASES IN WHICH THE SEIZURE TYPE OCCURS
Eyelid myoclonia with absence is usually the prominent seizure type
of the syndrome of eyelid myoclonia with absences (Jeavons syndrome).
However, it may also occur in symptomatic or cryptogenic epilepsies as
a predominant seizure type with or without photosensitivity. Eyelid myoclonia
may also occur in other idiopathic generalized epilepsies with absences.
Eyelid and other ictal manifestations in the syndrome of eyelid myoclonia
with absences (Jeavons syndrome)
The following conclusions are based on long video-EEG studies and
extensive reviews of 17 adults and 3 children with the syndrome of eyelid
myoclonia with absences (Panayiotopoulos et al 1996a, 1996b; Giannakodimos
and Panayiotopoulos 1996; Ferrie et al 1996; Parker et al 1996):
-
Eyelid myoclonia with absences is the most typical type of seizures
in this syndrome.|{video:EMACP1v.avi}{caption:Video-EEG of eyelid
myoclonia with absences}{label:The patient exhibits eyelid myoclonia
with absences mainly on eye-closure and intermittent photic stimulation.}||{video:EMACP3v.avi}{caption:Video-EEG
of long-standing eyelid myoclonia with absences}{label:In this Video-EEG
of a woman with long-standing eyelid myoclonia with absences, eyelid
myoclonia is consistently induced by eye closure in a lit room. This
is completely abolished when eye closure is performed in total darkness
achieved with underwater goggles covered with opaque tape.}||{video:eap3v.avi}{caption:Video-EEG
of seizure in eyelid myoclonia with absences}{label:Video-EEG of a
17-year-old woman with eyelid myoclonia with absences. Note that hand
jerking occurs during the eyelid myoclonia.}||{video:eap4v.avi}{label:Video-EEG
of 10-year-old girl with eyelid myoclonia}{title:Video-EEG of 10-year-old
girl with eyelid myoclonia}|
-
Eyelid myoclonia is often associated with jerks of the eyeballs,
head, or other muscles and may terminate without discernible absence.
This is the commonest type of seizure in this syndrome, and for treated
patients, this may be the only seizure type. These seizures are brief,
usually from 1 to 2 seconds. The ictal EEG manifestations are generalized
mainly polyspike discharges of brief duration (1 to 2 seconds, or
rarely 3 to 5 seconds).
-
Milder ictal eyelid manifestations consist of abortive eyelid myoclonia
with eyelid tremor-like jerks or fast eyelid fluttering. The eyes
remain closed while the upper eyelids exhibit small range, fast fluttering
that would be difficult to appreciate without close-up video-EEG recordings.
They are brief seizures, usually less than 1 second or, exceptionally,
1 to 3 seconds. These may be seen together with the more severe types
of eyelid myoclonia with or without absences. However, they can also
be the only type of seizures, particularly in patients on appropriate
medication. They are associated with polyspikes in the ictal EEG.
Slow eye-closure may occur, but the EEG discharges of mainly polyspikes
are usually apparent before the termination of the eye-closure artifact,
indicating that the tonic component of the eye-closure was an ictal
phenomenon. We have never observed the repetitive eyeblink-like movements
of self-induced photosensitive epilepsy in ictal absences of eyelid
myoclonia with absences, and absences never occurred without some
form of eyelid myoclonia preceding their onset.
-
Absence status epilepticus with eyelid myoclonia. Continuous clusters
of prolonged seizures of eyelid myoclonia with absences may occur
as the result of accumulating precipitating factors, discontinuation,
or inappropriate treatment (Agathonikou et al 1998). It is more likely
to occur on awakening.
Eyelid ictal manifestations in typical absence seizures
of other idiopathic generalized epilepsies
Eyelid myoclonia only rarely can be seen in typical absence seizures
of idiopathic generalized epilepsies other than the syndrome of eyelid
myoclonia with absences. In a study of 60 patients with video-EEG recorded
absence seizures, only 5 had eyelid myoclonia. Of these 5 patients, only
1 was also photosensitive, and only 1 experienced seizures precipitated
by eye-closure (Panayiotopoulos et al 1996a).
Some patients may have eyelid myoclonia with absences without photosensitivity.|{diagram:eap5.bmp}{caption:EEG
of eyelid myoclonia with absences but without photosensitivity}{label:Eyelid
myoclonia and the impairment of cognition during discharge is followed
with eyelid tremor-like movements and disturbance of consciousness despite
the apparent cessation of the discharge. The duration of the clinical
seizure is indicated by the arrows. The patient was counting (numbers
are annotated at top).}|Also rarely, eyelid myoclonia with or without
absences may occur in the same child together with typical absence seizures
without eyelid myoclonia (Ferrie et al 1996).|{diagram:eap6.bmp}{caption:EEG
of eyelid myoclonia and absences together or independently.}{label:This
EEG is from a normal 9-year-old girl with onset of seizures at 6 years
of age. These consisted of episodes lasting up to 45 seconds of psychomotor
arrest, accompanied by head slumping either to the right or left. Video-EEG
showed characteristic eye-closure-related abnormalities accompanied by
prominent eyelid myoclonia. She also had typical absences, both preceded
by and independently of seizures with eyelid myoclonia.}|
Eyelid myoclonia with absences in cryptogenic or symptomatic generalized
epilepsies
Eyelid phenomena are commonly seen during non-convulsive seizures
in children with cryptogenic and symptomatic generalized epilepsies. These
include eyelid blinking and flutter. True eyelid myoclonia is less common
(Ferrie et al 1996; Panayiotopoulos 1998).
PROGNOSIS AND COMPLICATIONS
Eyelid myoclonia is not only the most characteristic symptom of Jeavons
syndrome but also the most resistant to treatment, occurring many times
per day, often without apparent absences, and even without demonstrable
photosensitivity, which declines with age. Generalized tonic-clonic seizures
and absence status may be unavoidable in adult life, either as the result
of accumulating precipitating factors or more often due to inappropriate
medication.
MANAGEMENT
Life-long treatment with medication may be necessary for patients with
eyelid myoclonia with absences. In the absence of controlled therapeutic
trials, it is not possible to draw scientifically-valued conclusions about
the most effective approach to the drug treatment of eyelid myoclonia
with absences (Richens 1996). Based on anecdotal evidence, the drugs of
choice are those also used for other idiopathic generalized epilepsies
with absences and myoclonias (Panayiotopoulos 1999b). A combination of
sodium valproate and ethosuximide appears to be the most effective regimen.
However, clonazepam, which is highly effective in myoclonic jerks, may
be a valid option. Small doses of lamotrigine added to sodium valproate
may result in dramatic improvement because of their possible pharmacodynamic
interaction (Ferrie et al 1995).
Carbamazepine, vigabatrin, and tiagabine are contra-indicated, and the
same may be true for phenytoin.
Nonpharmacological treatments in photosensitive patients as detailed by
Wilkins may have a beneficial effect and should be employed (Wilkins 1995).
REFERENCES CITED
Agathonikou A, Panayiotopoulos CP, Giannakodimos S, Koutroumanidis
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ILAE
ILAE Copyright Notice
ABBREVIATIONS
MRI:magnetic resonance imaging
GTCS:Generalized tonic clonic seizures
SYNONYMS
Jeavons syndrome
MAJOR KEYWORD DESCRIPTORS
absences
alpha rhythm generators
eye closure
eyelid myoclonia
eyelids
idiopathic generalized epilepsies
intermittent photic stimulation
jerks of eyeballs, eyebrows, head, hands
myoclonic jerks of eyelids
photosensitivity
self-induction
spike-and-slow waves
tonic-clonic seizures
uninterrupted light
video-EEG studies
MINOR KEYWORD DESCRIPTORS
epilepsy
hyperventilation
seizures
AGE OF PRESENTATION
02-05 years
06-12 years
13-18 years
AGE OF TYPICAL PRESENTATION
06-12 years
13-18 years
GLOSSARY - ILLUSTRATION CAPTIONS
Video-clip 1. Three video-EEG seizures of the same patient are presented.
The first is during hyperventilation with onset when the eyes were closed.
The ictal discharge starts before the eyelid myoclonia. The second seizure
is elicited by eye closure, and that last by intermittent photic stimulation.
Video clip 2. Video EEG of a woman with long-standing eyelid myoclonia
with absences. Eyelid myoclonia is consistently induced by eye closure
in a lit room, but this is completely abolished when eye closure is performed
in total darkness (achieved with underwater goggles covered with opaque
tape).
Video clip 3. Video EEG of a 17-year-old lady with eyelid myoclonia
with absences. Note that hand jerking occurs during the eyelid myoclonia.
Video clip 4. Video EEG of a 10-year-old normal girl with onset of frequent
daily seizures at age of 4 years (Parker et al 1996). Seizures lasted
3 to 5 seconds. They consisted of marked eyelid myoclonia and tonic upward
deviation of the eyes accompanied by, at most, a mild impairment of consciousness.
In more severe attacks, there was retropulsive jerk of head, shoulders,
and sometimes limbs--occasionally leading to falls. Typical absences unaccompanied
by eyelid phenomena have not occurred. She did not have GTCS.
Video clip 5. From an interview with a patient with eyelid myoclonia
with absences explaining the pleasurable feelings of the eyelid myoclonia
seizure. Self-induction was suspected, but she firmly denied it, despite
good rapport and a long follow up with us.
Video clip 6. Video EEG and interview with a photosensitive patient
not suffering of the syndrome of eyelid myoclonia with absences. Note
that eyelid myoclonia is induced by intermittent photic stimulation. She
also has self-induced seizures by rapid successive movements of the left
hand in front of her eyes (which she illustrates) while watching television
or in the sun. Generalized discharges of spike and wave occurred during
hyperventilation with no detectable clinical symptoms.
Video clip 7. Video EEG and interview with a photosensitive patient
not suffering of the syndrome of eyelid myoclonia with absences. She also
has self-induced seizures by rapid successive eye closures looking at
the sun as she admits and imitates on video.
Figure 1. EEG during a seizure from video clip 1.
Figure 2. EEG during a seizure from video clip 2.
Figure 3. EEG during a habitual seizure from video clip 4.
Figure 4. EEG during an intermittent photic stimulation-induced seizure
from video clip 4.
Figure 5. From video-EEG of a 10 years old normal girl with eyelid myoclonia
with absences. She is not clinically or EEG photosensitive. Thirteen out
of 23 eye-closures were followed by generalized discharges that were clinically
manifested with eyelid myoclonia, upward tonic or clonic deviation of
the eyeballs and occasionally by a single backward jerk of the head. Mild
impairment of cognition was always a feature and was manifested with errors
in counting. The discharges were totally inhibited in darkness. In this
figure, eyelid myoclonia and impairment of cognition during the discharge
is followed with eyelid tremor-like movements and disturbance of consciousness
despite the apparent cessation of the discharge.
The duration of the clinical seizure is indicated with the arrows. Patient
was counting (annotated numbers).
Figure 6. From video EEG of a normal 9-year-old girl with eyelid myoclonia
and absences together or independently from age 6 years. Seizures, lasting
up to 45 seconds, consisted of psychomotor arrest, accompanied by head
slumping to the right or left. She was photosensitive. Video-EEG showed
characteristic eye-closure related abnormalities accompanied by prominent
eyelid myoclonia. In addition, she had typical absences, both preceded
by and independently of seizures with eyelid myoclonia.
PERMUTED TOPIC, SYNONYMS, VARIANTS
Eyelid myoclonia with and without absences
myoclonia with and without absences, Eyelid
with and without absences, Eyelid myoclonia
without absences, Eyelid myoclonia with and
absences, Eyelid myoclonia with and without
Jeavons syndrome
RELATED TOPICS
Absence status epilepticus
Epilepsy
Eyelid myoclonia with absences
Myoclonic absences
Other visual-sensitive epilepsies
DIFFERENTIAL DIAGNOSIS
symptomatic generalized epilepsy
idiopathic generalized epilepsy with absences
cryptogenic generalized epilepsy
atypical absences
tonic seizures
atonic seizures
partial seizures
random eyelid blinking
self-induced photosensitive epilepsy
self-induced eyelid myoclonia
attraction movement
tics
obsessive-compulsive symptoms
forced eyelid closures
eyelid fluttering
symptomatic occipital epilepsy
idiopathic occipital epilepsy
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