Seizure Disorders 0031-3955/89 $0.00 + .

20

Absence, Myoclonic, and

Atonic Seizures

Lawrence A. Lockman, MD*

The most prominent feature of absence seizures is loss of consciousness.

The historical term, petit mal seizure, has been applied not only to the
typical absence attack, but also to other epileptic events and thus lacks
specificity. Other seizure types share important features with typical
absence; they are grouped with it as atypical absence in the International
League Against Epilepsy Classification of Epileptic Seizures (Table 1).12

ABSENCE

In the typical simple absence attack, the patient abruptly loses con-
sciousness; ongoing activity ceases and the patient's eyes stare vacantly
straight ahead or may roll upwards. There is no change in posture and no
movement except, perhaps, for slight eyelid fluttering and twitching of the
perioral muscles. Consciousness and activity are resumed suddenly after a
few seconds. There is no postictal confusion or drowsiness. Dozens to
hundreds of seizures may occur in a single day,l1 and the presence of
seizure-free days casts doubt on the diagnosis. 3 This form of typical absence
epilepsy is also referred to as pyknolepsy (pyknos [Gk], "close together").
The' onset of seizures in most patients is between 4 and 8 years of age,
although cases under age 236 have been reported and a rare patient older
than 15 years. Development and neurologic examination are usually normal.
Abnormality of development or neurological examination has been reported
in as many as 21 per cent of patients with "typical absence. "13 Early or late
onset or resistance to treatment are findings significantly correlated with
lower intelligence. 49
The frequent interruptions of consciousness may interfere with intel-
lectual and school performance. Psychological testing will yield spurious
results in patients with frequent, uncontrolled absence attacks.

*Associate Professor of Neurology, Division of Pediatric Neurology, University of Minnesota

Medical School, Minneapolis, Minnesota

Pediatric Clinics of North America-Vol. 36, No.2, April 1989 331

332 LAWRENCE A. LOCKMAN

Table 1. International Classification of Epileptic Seizures and Generalized

Seizures
CLINICAL SEIZURE TYPE
A. Absence Seizure
1. Typical
a. Impairment of consciousness only
b. With mild clonic components
c. With tonic components
d. With tonic-clonic components
e. With automatisms
f. With autonomic components
(b through f may be used alone or in combination)
2. Atypical may have the following:
a. Changes in tone that are more pronounced than in A.l
b. Onset and/or cessation that is not abrupt
B. Myoclonic seizures, myoclonic jerks (single or multiple)
C. Clonic seizures
D. Tonic Seizures
E. Tonic-clonic seizures
F. Atonic seizures
(Astatic)
(Combinations of the above may occur; for example B and F, B and D)
Modifiedfrom Commission on Classification and Terminology of the International League
Against Epilepsy: Proposal for classification of epilepsies and epileptic syndromes. Epilepsia
26:268-278, 1985.

Many patients manifest variations or additional features. In fact, these

complex absence seizures are more common than the typical absence. 46
Absence with mild clonic components includes eyelid blinking, mouth
twitching, and finger, arm, or shoulder movements. Posture is not dis-
turbed, but objects may be dropped. Patients with absence with mild tonic
components have either symmetrical or asymmetrical increase in postural
tone. In absence with atonic components, the decrease in postural tone
results in head drooping or mild slumping, but the patient does not fall.
The diagnosis of absence seizures is confirmed by electroencephalog-
raphy (EEG). During the seizure, the tracing exhibits the sudden onset of
bilaterally synchronous, frontally predominant, generalized, 3-Hz spike-
and-slow-wave activity. Following the discharge the record returns to its
preictal appearance, with no intervening voltage depression or postictal
slowing. The interictal electroencephalogram is usually normal. Brief bursts
of spike-and-wave activity without obvious clinical seizures can be seen in
up to one third of patients.
Both complex and simple forms of absence with automatisms 44 occur.
More than 50 per cent of absence seizures lasting longer than 7 seconds
were accompanied by automatisms. Partial complex seizures often begin
with an aura, are accompanied by complex automatisms, are followed by
postictal confusion or drowsiness, and are usually infrequent, rarely occur-
ring at a particular time of day. Complex partial seizures of frontal lobe
origin are, however, often very similar to absence. Thus, differentiating
complex absence from partial complex seizures may be virtually impossible
on purely clinical grounds. Clinical and EEG correlation is sometimes
required, particularly in the patient with absence, in whom activation
techniques (i. e., hyperventilation) can precipitate the seizure.
ABSENCE, MYOCLONIC, AND ATONIC SEIZURES 333
In absence with autonomic phenomena, alterations in pupil size, skin
color, and heart rate suggest sympathetic nervous system effect. Urinary
incontinence can also occur.
In the 374 absence seizures analyzed by Penry et aI., 45 only 35 (9.4
per cent) were simple, whereas 90.6 per cent were complex, with autom-
atisms occurring during 63. I per cent, mild clonic components occurring
in 45.5 per cent, and decreased postural tone occurring in 22.5 per cent of
the seizures.
Absence seizures (and the associated EEG discharge) affect cognitive
function. Porter, Penry, and Dreifuss 47 studied 14 patients with absence
seizures using a computer-assisted reaction-time measurement triggered by
the onset of EEG paroxysms. Fifty-six per cent of the reaction times
measured were distinctly abnormal. Later-onset stimuli yielded even more
abnormal reaction times. In another study of 26 patients,47 413 auditory
reaction times were determined. They were normal during the second
before the EEG paroxysms. Forty-three per cent were normal at the onset
of the paroxysms, but only twenty per cent were normal after a delay of
0.5 seconds into the paroxysm. The authors concluded that regardless of
duration, the spike-wave paroxysm impaired consciousness. Therefore treat-
ment should attempt to control all spike-wave paroxysms.
Whether to pursue further diagnostic studies or treatment in the
asymptomatic patient with 3-Hz spike-and-wave activity noted incidentally
on EEG has not been directly studied, although some impairment of
function has been presumed. 1. 10 Transient cognitive impairment has been
demonstrated in 50 per cent of patients with asymptomatic generalized
epileptiform discharges, with 44 per cent having difficulty with a spatial
task and 28 per cent with a verbal task. 7
Simple absence is usually without a demonstrable etiology other than
genetic factors. Only rarely is it associated with progressive encephalopathy
(subacute sclerosing panencephalitis9 ), lysosomal storage disease,4, 27 or in
metabolic derangements such as uremia, hypoglycemia, and metrizamide
encephalopathy. 48
Treatment with barbiturate or hydantoin antiepileptic drugs is usually
not effective, and the seizures in some instances get worse. Ethosuximide
and valproate are considered the medications of choice. Accurate and
specific diagnosis is important because of the increasing specificity of
antiepileptic drugs, the Significant benefits of monotherapy, and the differ-
ent prognosis attached to the different absence seizure syndromes.
Atypical Absence
Atypical absence seizures are often very different in several respects
from the simple absence seizure, yet certain clinical or electroencephalo-
graphic features have led to the classification of these seizures with the
absence group. In atypical absence seizures, changes in tone are more
pronounced or the onset or the cessation is not abrupt. During the seizure
the electroencephalogram is more heterogeneous and may have fast activity,
irregular spike-and-slow-wave complexes, or other paroxysmal features. 19
Symmetry is not regularly seen, although bilaterality is the rule.
Syndromes with a variety of generalized seizures are often considered
334 LAWRENCE A. LOCKMAN

along with atypical absence because of the similarity of clinical and electro-
encephalographic features. These disorders include myoclonic, clonic, tonic,
and atonic seizures. When all of these types, as well as the typical absence
seizure are seen, the patient is considered to have the Lennox-Gastaut
syndrome.
Partial complex seizures are not classified with this group because of
distinguishing etiologic and electroencephalographic features, although clin-
ically the patient may display seizures consisting solely of loss of awareness.
The localized nature of the epileptogenic phenomen~ is an important
distinguishing feature.
Activation, particularly hyperventilation, often can precipitate electrical
and clinical seizures. Having the patient take apout 60 deep breaths per
minute for :1 or sometimes 4 minutes often precipitates a typical attack.
Hyperventilation has been found more effective than prolonged monitoring
in predicting clinical seizure frequency2 and prolonged monitoring is more
effective than parent or teacher observation. l l Hyperventilation is also
useful in assessing the effect of treatment.
Absence seizures have a genetic basis. There is a strong familial
tendency toward the spike-and-wave pattern in siblings (37 per cent),
particularly between the ages of 4.5 and 7.5 years (50 per cent).39 A high
concordance rate in twins for 3-Hz spike-and-wave discharges (84 per cent)
and absence seizures (75 per cent) also has been reported. 34 The observation
that generations are not skipped suggests a dominant mode of inheritance, 40
but the exact genetic mechanism has not yet been determined. 5
Treatment
Treatment with a single anti epileptic drug (monotherapy) is preferred.
Ethosuximide has been the drug of choice for 20 years. 42 More recently,
valproate has been shown to be equally effective, 50 but the higher incidence
of side effects, some serious, and the need to monitor hematologic and
liver function, make it ~ second choice for absence seizures.
Prognosis
About half of the patients with absence seizures become seizure free
and may be withdrawn from medication after 2 to 4 years. For the
remainder, either the absence seizures persist or the patient goes on to
develop tonic-clonic seizures. In a prospective study of 48 patients,5l 90
per cent of absence patients without tonic-clonic seizures, with normal or
better intelligence, and a negative history of family seizure disorders,
became seizure free. In another prospective study of 90 patients with
typical absence seizures followed for up to 15 years,37 those patients with
myoclonic or atonic absence had a poor prognosis. Patients with simple
absence or absence with automatisms often remitted. Overall remission
rate with absence was only 57.5 per cent. Tonic-clonic seizures developed
in 36 per cent of the subjects. These figures are in general agreement with
older retrospective studies. More recently, a followup study of 83 patients
followed for an average of 9.5 years found that the prognosis was better for
patients with normal intelligence quotient, male sex, normal neurologic
examination, and without hyperventilation-induced spikes-and-waves. 52 Sei-
AasENCE, MYOCLONIC, AND ATONIC SEIZURES 335

Table 2. Age of Occurrence of Absence Seizures (years)

1 2 3 4 5 6 7 8 9 10 11 12 13 14 15 16 17 18 19 20

-------Absence--------------
-----Impulsive Petit Mal---------
-----Myoclonic Absence----------------
------Juvenile absence---------------
Modifiedfrom Delgado-Escueta AV, Treiman DM, Walsh GO: The treatable epilepsies.
Part 1. N Engl J Med 308:1508-1514, 1983.

zures stopped in more than 90 per cent of the patients who had three or
more of these findings. Relapse is most likely to occur in the first year after
treatment is discontinued. 56 Thus, simple absence and absence with autom-
atisms are not benign diseases of childhood, and the prognosis is guarded.
Current pharmacologic management, al~hough often effective in controlling
the clinical seizures, does not seem to have influenced the long-term
outcome in a significant fashion.

JUVENILE MYOCLONIC EPILEPSY (IMPULSIVE PETIT MAL)

A specific syndrome of epileptic seizures that QCcurs in the second

decade of life has been called impulsive petit mal (Table 2).30 The same
syndrome has probably been described under many different names,
including juvenile myoclonic seizures, bilateral massive myoclonic jerks,
generalized epilepsy with myoclonia during adolescence llJld late childhood,
and myoclonic petit mal. The preferred name is now juvenile myoclonic
epilepsy. The seizures are easily confused with tonic-clonic seizures (grand
mal) leading to the use of ineffective antiepileptic drugs. I4 The syndrome
is said to occur in 4 per cent of patients with epilepsy. 6, 30
The myoclonic jerks occur shortly after awakening (morning my-
oclonus), are mild, and are not accompanied by loss of consciousness. The
interictal EEG signature of this seizure is fast (4-6 Hz) multiple spike-and-
slow-wave complexes (Table 3). Fast, diffuse spikes (16-24 Hz) are seen in
both hemispheres during the myoclonic jerks, 16 Photic stimulation leads to

Table 3. Electroencephalographic Features

FREQUENCY OF SPIKE-WAVE
SEIZURE TIPE DISCHARGE

Characteristics of Ictal EEG

Atypical absence 1-2.5 Hz
Simple absence 2.5-4 Hz
Myoclonic absence 4-6 Hz
Myoclonous absence 8-12 Hz
Juvenile myoclonic epilepsy 16--24 Hz Spikes
Characteristics of Interictal EEG
Simple absence Normal
Myocionic-astatic epilepsy 1-2.5 spike-and-wave
Juvenile myoclonic epilepsy 4-6 Hz polyspike-and-wave
336 LAWRENCE A. LOCKMAN

paroxysmal generalized EEG discharges, termed a photoconvulsive re-

sponse, or even to a photoconvulsive seizure. These patients often have a
variety of other seizure types, and 50 per cent of them also demonstrate
absence.!5
The disorder is frequently progressive. Particularly if the myoclonic
attacks become more frequent, the patients develop either clonic seizures
or clonic-tonic-clonic seizures (often misdiagnosed as tonic-clonic sei-
zures). Consciousness is lost during the clonic seizures. The patient falls to
the floor, passively, as a result of the sudden loss of muscle tone, or
forcefully, resulting from a massive myoclonic jerk. Rapid clonic jerking of
the face and extremities and autonomic changes ensue. Consciousness
returns quickly and there is little postictal depression. Additionally, the
patient may have a tonic phase separating the initial and terminal clonic
phases, leading to a clonic-tonic-clonic seizure.
There was a high family incidence of myoclonic events reported by
Tsuboi and Christian. 57 About 41 per cent of relatives of probands had these
abnormalities with a propensity among female relatives; 15 per cent of
relatives without clinical myoclonus showed the electroencephalographic
findings. . .
The treatment of choice is valproate, to which the seizures are said to
be very responsive!6; the prognosis is excellent, in part because of the age-
limited nature of the disorder. Long-term medication may be necessary.14

OTHER RARE FORMS OF ABSENCE

Several other varieties of absence have been described by Delgado-

Escueta et al.!5 and by Janz et al. 3! Clinically, juvenile absence is identical
to classic absence but has an EEG signature of8 to 12 Hz "diffuse rhythms."
Myoclonic absence is characterized by brief unconsciousness, with staring,
stereotyped automatisms, and bilateral symmetric clonic jerks lasting from
10 to 60 seconds. 55 There is tonic contraction around the shoulders. Some
patients also have tonic-clonic seizures. Mental retardation is' common.
The EEG exhibits regular, rhythmic 3-Hz spike-and-wave discharges.
Treatment with valproate is successful in some cases!6 although greater
success may be obtained with valproate and ethosuximide together. 32, 55
Unfortunately, seizures are often refractory and some patients go on to
develop the picture of atonic-astatic epilepsy (Lennox-Gastaut syndrome).
Myoclonus absence differs in that the clonic jerks are asymmetric and the
automatisms are reactive. These seizures all are said to respond to valproate,

MYOCLONIC-ASTATIC SEIZURES

The essential features of myoclonic-astatiC seizures, or the Lennox-

Gastaut syndrome, are early onset of epilepsy; multiple seizure types in
the same patient, including absence, myoclonic jerks, and atonic seizures;
impaired development and intellect; and generalized slow-spike-and-wave
discharges on EEG. Other terms that have been used for this form of
ABSENCE, MYOCLONIC, AND ATONIC SEIZURES 337

epilepsy are static seizures, akinetic epilepsy, minor motor epilepsy, pro-
pulsive petit mal, akinetic petit mal, and severe myokinetic epilepsy of early
childhood with slow-spike-and-wave.
Gibbs et al. 24 described the slow (about 2 Hz) spike-and-wave dis-
charge, referring to it as the "petit mal variant" pattern.25 Lennox and
others described the "petit mal triad,"34, 35 which included petit mal
(absence), myoclonic, and astatic seizures. Later, massive myoclonic jerks
were added, expanding the seizure variety to the "petit mal quartet. "34
They also noted a greater variety of seizures, earlier onset, and more
evidence of "brain damage" in these patients. Gastaut et al. 23 reported in
detail patients with "epileptic encephalopathy with diffuse slow spike-
waves." His criteria for diagnosis were onset between the ages of 1 and 6
years; frequent seizures, nearly always including tonic seizures, atonic
seizures, and a variant of absence; and pronounced mental retardation. The
interictal EEG contained generalized bursts of 1.5- to 2.5-Hz spike-and-
wave activity that did not respond to activation procedures.
It has been emphaSized by many authors that the slow spike-and-wave
discharges are interictal EEG phenomena. 38 They are activated by sleep. 38
Ictally, identical discharges may be seen during an atypical absence attack,
one in which there is less abrupt and complete loss of consciousness.
During seizures other EEG changes may supervene such as desynchroni-
zation of the record 8. 22. 23. 26 especially associated with tonic seizures,38 and
brief bursts of polyspikes or rapid spikes. 41 This lack of a one-to-one
relationship between the epileptiform EEG discharges and the clinical
seizures is in marked contrast with other generalized seizures, especially
simple absence. Dreifuss 18 stated, on the other hand, that some of these
children are in absence status for years at a time, suggesting that the slow-
spike-and-wave discharge may correlate with a clinical seizure state.
Periods of distressingly frequent seizures, and sometimes even absence
status are interspersed with times of relative freedom from seizures in
many patients. The frequent, often forceful, falls are a cause of recurrent
injury, particularly to the face and head, and result in lacerations, with
subsequent, often disfiguring scars. Football helmets, and more recently
hockey helmets, are of some benefit in reducing the severity of injury.
A specific etiology may be found in from 50 to 90 per cent of cases of
myoclonic-astatic epilepsy, in contradistinction to generalized absence and
juvenile myoclonic epilepsy, in which genetics plays a major role. 33, 38 The
most commonly attributed cause is perinatal neurologic insult. A significant
number of patients have a prior history of infantile spasms. Numerous
authors have noted a much higher incidence of epilepsy in the families of
patients with myoclonic-astatic epilepsy than in the general population,
but the seizures in the relatives are rarely of the myoclonic-astatic type.
Males predominate in most series comprising up to 71 per cent of patients.
A variety of other etiologies have been implicated in individual patients,
including tuberous sclerosis, lipid storage disease, and aminoacidopathies.
There is a high frequency of cerebral atrophy, particularly subcortical, on
CT scans.20
A rare form of myoclonic-astatic epilepsy with male preponderance,
and normal intelligence occurring in 1- to 5-year-old children, was described
338 LAWRENCE A. LOCKMAN

Table 4. Characteristics of the Generalized Epilepsies

Primary Generalized Epilepsies
Seizure type Absence
Myoclonic
Tonic-Clonic
Focal neurologic signs Absent
Mental retardation Absent
Etiologic factors Only genetic
Secondary Generalized Epilepsies
Seizure type As above plus
Tonic
Atonic
Focal neurologic signs Frequent
Mental retardation Frequent
Etiologic factors Often acquired

by Doose.17 Tonic seizures usually are not seen. There may be a genetic
predisposition, and the prognosis is much better than in the Lennox-
Gastaut syndrome.
Treatment
No other group of seizure patients is more challenging and frustrating
to the treating physician. Every antiepileptic drug, alone and in combina-
tion, has been used in these patients, often with no success whatsoever.
Most of these patients are subjected to polypharmaceutical assaults with no
detectable difference in seizure frequency or severity. Some agents may
make the seizures worse, or if not, then may make the patient worse. 53
Sedative anticonvulsants probably should be avoided. Phenobarbital almost
never influences the seizures and may cause cognitive and behavioral
aberrations.
Papini et a1. 43 found in a study of 16 patients for prolonged periods
that the average number of seizures per day was around 25. About 31 per
cent of the seizures occurred during drowsiness and 54 per cent occurred
during inactive wakefulness. Only 6 per cent occurred during sleep and 1
per cent during active wakefulness. The authors suggest that secondary
effects of sedative antiepileptic drugs may increase seizure frequency by
decreasing active alertness in these patients.
Clonazepam, originally thought to be effective in these seizures, often
exhibits tolerance, necessitating dosage increase and leading to eventual
sedation. Monotherapy with valproate is as likely to benefit the patient as
any other therapy, and early reports of its efficacy and of the ability of
some patients to discard their helmets, has turned out to be only slightly
overenthusiastic.
The ketogenic diet, originally proposed by Wilder, and later modified
by Huttenlocher et al.28, 29 to permit a more normal nutritional state, has
been extraordinarily effective in an occasional otherwise intractable patient.
Unpalatability, expense, and the social isolation imposed by such a stringent
diet are the major drawbacks to its use.
More recently, ACTH has been found effective for seizure control in
up to 80 per cent of the patients in an uncontrolled study.54
ABSENCE, MYOCLONIC, AND ATONIC SEIZURES 339
CONCLUSION

These varieties of absence seizures, including classic absence, juvenile

myoclonic epilepsy, the juvenile absence seizures with myoclonic phenom-
ena, and atonic-astatic seizures are not rare (Table 4).
The proper diagnosis requires careful historical elucidation of the actual
seizure events, family history of seizures, and other neurologic abnormali-
ties. Electroencephalographic study must include proper activation tech-
niques. Treatment is initiated with antiepileptic drugs likely to be effective
against generalized discharges, ethosuximide, and valproate.

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Department of Neurology
200 First Street, SW
Mayo Medical School
Rochester, Minnesota 55905