Epilepsia, 44(Suppl.

11):712, 2003
Blackwell Publishing, Inc.
C
International League Against Epilepsy
Clinical Presentations and Phenomenology of Myoclonus
Edward Faught
UAB Epilepsy Center, University of Alabama School of Medicine, Birmingham, Alabama, U.S.A.
Summary: The termmyoclonus has been used to describe het-
erogeneous phenomena involving sudden movements, but there
is no generally accepted, precise definition of myoclonus. My-
oclonus canoftenbe classifiedbasedonelectroencephalographic
(EEG) and/or electromyographic (EMG) data. Some myoclonic
epilepsy syndromes, including juvenile myoclonic epilepsy, may
frequently be misdiagnosed because of failure to obtain a com-
plete patient history and/or failure to appreciate characteristic
EEG changes. A good understanding of the features associated
with myoclonic disorders (particularly the myoclonic epilepsies)
and of features associated with other neurologic disorders that
are often confused with myoclonic disorders is an invaluable aid
in obtaining an accurate diagnosis and will ultimately help in de-
termining the best course of treatment for patients. Key Words:
MyoclonusSeizuresEpilepsy.
DEFINITION OF MYOCLONUS
In 1968, Gastaut (1) referred to the term and concept
myoclonus as a long-standing source of confusion and
debate, and this confusion remains today. There is no
generally accepted, precise definition of myoclonus, de-
spite the many reviews of the subject (16). Definitions in
textbook articles are variations on a theme: quick muscle
jerks, either irregular or rhythmic (4), a sudden, brief in-
voluntary movement (7), and a brief, shocklike jolt in a
muscle (8). The scope of definitions ranges fromthe very
general [any brief muscular contraction (1)] to the more
qualified [a sudden, brief, shock-like muscle contraction
arising from the CNS (9) and abrupt, jerky, involuntary
movements unassociated with loss of consciousness(6)].
Halliday (10) noted the difference between the
lightning-like movements of epileptic myoclonus and
the rhythmic movements of subcortical myoclonus but
retained the latter within the definition of myoclonus.
This concept has been followed by Hallett (4). The in-
clusion of rhythmic movements within the definition of
myoclonus leads to the categorization of epilepsia par-
tialis continua (EPC, continual focal motor seizures), seg-
mental myoclonus, and palatal myoclonus as myoclonic
disorders. For myoclonus to arise only from the central
nervous system would exclude some peripheral disorders
often termed myoclonic. A logical and appealing classifi-
Address correspondence and reprint requests to Dr. E. Faught at UAB
Epilepsy Center, University of Alabama School of Medicine, Civitan
International ResearchCenter 312, 17196thAvenue South, Birmingham,
AL 35294, U.S.A. E-mail: [email protected]
cation was proposed by Adams and Victor (11), who de-
scribed myoclonus as a shocklike, irregular jerk, clonus
as rhythmic, uniphasic jerking, and tremors as bipha-
sic jerking. Following this reasoning, EPC and palatal
myoclonus are forms of clonus. Palatal myoclonus might
better be termed palatal tremor since it is biphasic.
One problem with all of the definitions given is the
question of whether or not movements clearly falling
within these definitions, but not often described as my-
oclonic, should be considered myoclonus or not. Tics, star-
tle responses (epileptic and nonepileptic), fasciculations,
myokymia, hemifacial spasm, chorea, and even infan-
tile spasms are examples of such movements. Although,
strictly speaking, such movements are myoclonic in na-
ture, and some authors include some of these terms un-
der the umbrella of myoclonus (5), describing them as
such or categorizing them as myoclonic disorders con-
fuses, rather than clarifies, diagnostic schemes. Even in
1881, Friedreichs purpose in originally coining the term
paramyoclonus, which was later shortened by others to
myoclonus, was to distinguish myoclonic epilepsy from
chorea (12). Noting that hiccoughs, hypnic jerks, and simi-
lar physiological phenomena are in fact examples of phys-
iological myoclonus causes less confusion than including
pathological terms within the definition of myoclonus.
This report will describe the phenomenology of my-
oclonic movements, particularly as they are observed in
the common varieties of myoclonic epilepsies; examine
the differences between these movements and similar sud-
den, jerky movements that imply different diagnoses; and
review the most common clinical settings in which my-
oclonus is observed.
7
8 E. FAUGHT
EPILEPTIC MYOCLONUS
Juvenile myoclonic epilepsy
This common, genetic, primary generalized epilepsy
usually brings a teenager or young adult to medical at-
tention after the occurrence of a generalized tonic-clonic
seizure. A careful patient history often reveals that early
morning myoclonic jerks have been present for months or
years before a seizure occurrence. The diagnosis is often
missed if this history is not solicited. Common reasons
for diagnostic error are failure to ask about myoclonus
and failure to appreciate the typical electroencephalo-
gram (EEG) findings associated with juvenile myoclonic
epilepsy (JME) (13).
Myoclonic jerks in JME can be asymmetrical or even
unilateral, which can lead to a misdiagnosis of focal motor
seizures due to localization-related epilepsy. The presence
of the characteristic generalized polyphasic spike-wave
EEG discharge, often with an initial frequency of 3.5
4 Hz, usually clarifies this issue. Simple partial seizures
with motor manifestations will have unilateral frontal dis-
charges, or sometimes no EEG correlate, with the move-
ment. The latter phenomenon does not occur with JME,
and an EEGcorrelate of the myoclonic jerk will always be
present. However, the reverse is not true in JME; that is,
EEG discharges up to several seconds long are sometimes
seen without a discernible jerk.
Myoclonic jerks in JME typically involve the upper
body, including the arms, hands, and sometimes the head
and trunk. They may affect flexors or extensors. Probably
the most common movement is elevation at the shoulder
with flexion at the elbow. The amplitude varies consider-
ably, from rather violent upper body flexion resembling
an infantile spasm to very minimal hand or finger twitch-
ing, which can resemble chorea or fasciculation. This
small-amplitude twitching has been called minipolymy-
oclonus (14).
Although myoclonus is almost always defined as a
quickmovement, some patients exhibit relativelyslowarm
movement (up to 1 s in duration) with an even slower re-
laxation phase. Such myoclonus can resemble voluntary
movement or a normal response to an environmental stim-
ulus. The EEG can confirm the epileptic nature of such
atypical myoclonic movements.
The phenomenologyof the myoclonic jerks seeninJME
is similar to that of other primary generalized epilepsies
that are relativelybenign. These syndromes include benign
myoclonus of infancy, which is seen before age 3 years;
childhood absence epilepsy (mainly with photic stimula-
tion); and adult-onset benign myoclonic epilepsy.
The myoclonus of progressive myoclonic epilepsies
The diverse etiologies of progressive myoclonic epilep-
sies (PMEs) are discussed in the article by Leppik in this
supplement (15) and in a previous article (16). However,
the phenomenology of myoclonus in the PMEs is rela-
tively similar across different pathophysiological causes
and differs in some respects from the myoclonus of JME
(16). For example, there is no early-morning predomi-
nance of myoclonus in PMEs. The myoclonus in PMEs
tends to be multifocal, of variable amplitude with many
small jerks, relatively constant, and increased by volun-
tary movement. In these characteristics it may resemble
chorea.
Somatosensory or auditory reflex precipitation of
seizures is more common in PME and light precipitation
of seizures is more common in JME (17). Exceptions to
this rule include PMEcausedbyUnverricht-Lundborgdis-
ease, in which photosensitivity is marked; the sensitivity to
single flashes seen in PME due to some forms of neuronal
ceroid lipofuscinosis; and benign myoclonic epilepsy of
adulthood (possibly a late-onset variant of JME), in which
somatosensory precipitation occurs.
Several EEG features are useful in distinguishing PME
from more benign epilepsies (18) (Table 1). Unlike JME,
most of the myoclonic movements in PME syndromes
especially the almost continual, small-amplitude jerks
are not time-locked to EEGdischarges. Whether they rep-
resent subcortical phenomena or restricted-field cortical
discharges is unclear. Large-amplitude jerks may have
EEG correlates, often very high-amplitude generalized
spike-wave bursts, which are slower and less rhythmic
than those associated with JME.
The distinction between PME and more benign my-
oclonic epilepsies like JME does not ordinarily depend on
recognition of differences in the myoclonic movements
themselves. The associated neurological deterioration af-
fectingcognitive function, EEGbackgroundactivity, other
motor systems, and(insome PMEsyndromes) retina, skin,
and other organs, eventually leaves no doubt as to the cat-
egory to which a particular patient belongs.
NEGATIVE MYOCLONUS
This paradoxical term refers to sudden relaxations in
tonic muscle contraction. It might better be referred to as
an atonic phenomenon. Tassinari et al. (19) first described
it as the related epileptic silent period, but the termneg-
ative myoclonus appears to have been coined by Shahani
and Young in 1976 (20) in reference to posthypoxic in-
tention myoclonus. They noted that the events appeared
TABLE1. Characteristics of the EEG in patients with
progressive myoclonic epilepsies (17)
Generalized multiple spike waves
Slow background, disorganized sleep
Photosensitivity (to single flash: neuronal ceroid lipofuscinosis)
Imperfect relation of spikes to myoclonus
Giant somatosensory evoked potentials
Focal, especially occipital, epileptiform discharges
Vertex positive spikes in sialidosis
Epilepsia, Vol. 44, Suppl. 11, 2003
MYOCLONUS PHENOMENOLOGY 9
clinically to be myoclonus but were associated with a
pause in electromyographic (EMG) activity. Later, these
authors noted that asterixis is an essentially identical phe-
nomenon (21). Fahn et al. (3) reported that asterixis is
associated with a 25- to 200-ms pause in the EMG, simi-
lar to other forms of negative myoclonus.
Negative myoclonus can be seen in various myoclonic
epilepsies (3,19,22). It has beendescribednot onlyinsome
PMEs and other secondary or cryptogenic generalized
epilepsies, but also in a syndrome that is not often consid-
ered to be a myoclonic epilepsy, benign epilepsy with cen-
trotemporal spikes (benign Sylvian-Rolandic epilepsy). In
this case, the negative myoclonic movement occurs simul-
taneously with the centrotemporal spike-wave complex
(22).
Although classifying atonic seizures as negative my-
oclonus may be logical, doing so would create diagnostic
confusion by replacing a term in common use with an
alternate one that generally refers to a phenomenon seen
in completely different syndromes. The termnegative my-
oclonus may be reserved for atonias affecting limbs, rather
than the trunk or head.
DISTINGUISHING MYOCLONUS FROM OTHER
MOVEMENT DISORDERS
The distinction between myoclonus and other move-
ment disorders is not always clear, and some writers con-
sider all manner of sudden involuntary jerks to be my-
oclonus. Nevertheless, differentiation of epileptic from
nonepileptic myoclonus and of different varieties of jerks
of presumed subcortical origin from one another is obvi-
ously important.
Simple partial seizures with motor manifestations
As previouslydiscussed, EEGdifferences exist between
simple partial seizures and myoclonic epilepsies, even
though the jerks of the latter can be unilateral or focal.
However, consistent focality, unilaterality, or Jacksonian
spread strongly suggests simple partial seizures. Some va-
rieties of myoclonus, such as cortical reflex or reticular
reflex myoclonus (discussion follows) (4), may involve
sequential activation of cranial nerve and spinally inner-
vated muscles in different orders, but this phenomenon
occurs rather quickly (i.e., within a few seconds). Jackso-
nian spread across the motor cortex is usually relatively
slow, taking many seconds or even minutes. Motor sim-
ple partial seizures often involve the upper extremities
but, unlike myoclonic seizures, also commonly involve
the lower extremities, alone or with the upper extremities.
Some authors note that the jerks of simple partial seizures,
especiallythe long-continuingmovements of the EPCsyn-
drome, are rhythmic, while myoclonic jerks are arrhyth-
mic (11). However, motor jerks that occur with simple
partial seizures are not always strictly rhythmic, and they
TABLE2. Characteristics of myoclonus versus those of tic
Myoclonus Tic
No preceding urge to move Urge to move
Simple movement Simple or complex movement
Often bilateral upper Midline (facial) or lateralized,
extremities, little variable, never bilateral
variation in location extremities
No vocalization Vocal tics 530%
(Tourettes syndrome)
Nonpurposeful May appear purposeful
Nonsuppressible Temporarily suppressible
are not always constant in amplitude or unidirectional. At
the bedside, distinguishing among EPC, segmental my-
oclonus, or evenpsychogenic jerking(pseudoseizure) may
be difficult or impossible.
Tics
Although many tics are visibly indistinguishable from
myoclonus, some differences are diagnostically helpful
(Table 2). Focal tics may resemble segmental myoclonus.
Tics are usually facial or vocal, but segmental myoclonus
is most often thoracic and is more continuous and rhyth-
mic. The multifocal tics of Tourettes syndrome resemble
the small-amplitude myoclonus seen in PMEs, including
the exacerbation by voluntary movement. The presence
of vocal tics suggests Tourettes syndrome, which can be
useful in differentiation.
Tics must be differentiated from myoclonic disorders
(or other myoclonic disorders if one classifies tics as my-
oclonus). Drugs for the treatment of myoclonus, whether
epileptic (cortical) or nonepileptic (subcortical), are not
usually effective for tic disorders, and neuroleptics used
for tics, such as haloperidol or pimozide, may occasionally
make seizures worse.
Chorea
Similar problems are encountered when differentiating
chorea from myoclonus. However, chorea usually coex-
ists with athetoid movements, which are not a feature of
myoclonic disorders. Also, the patient with chorea often
attempts to incorporate choreiform movements into vol-
untary actions, which is rare in patients with myoclonus.
Fasciculations
Fasciculations may be misidentified as myoclonus. In
fact, the term minipolymyoclonus was first used to de-
scribe the fasciculations caused by the denervation of
spinal muscular atrophy (23). Confinement of the twitches
to single muscle fascicles versus larger units suggests lo-
cal neuromuscular pathology. An EMG and the presence
of other clinical features of denervating diseases should
allow easy differentiation between fasciculations and
myoclonus.
Epilepsia, Vol. 44, Suppl. 11, 2003
10 E. FAUGHT
Startle syndromes
This category encompasses startle epilepsy, which
usually occurs as an auditory reflex epilepsy, hyperekplex-
ias, and culturally conditioned excessive startle responses
(24). Startle epilepsy is commonly of frontal lobe or mid-
line hemispheric origin and is often (but not always) as-
sociated with cognitive deficiency or cerebral palsy. The
initial tonic axial movement was asymmetrical in 16 of
19 patients in one series (25). It often converts quickly to
a versive or fencing movement of the head and body and
sometimes to a generalized tonic-clonic seizure. The ex-
aggerated startle responses seen in the nonepileptic startle
syndromes are usually axial, upper limb, and bilateral, but
the EEG is normal. An exception is the abnormal periodic
sharp-wave EEG pattern of Creuzfeldt-Jakob disease, but
the sharp waves do not necessarily coincide with sponta-
neous or startle-induced myoclonic jerks.
EPILEPTIC VERSUS NONEPILEPTIC
MYOCLONIC DISORDERS
The distinction commonly made between epileptic and
nonepileptic myoclonus is often considered synonymous
with the presence or absence of EEG epileptiform dis-
charges time-locked to the jerks. Unfortunately, the patho-
physiology is not that simple. In many diseases that in-
volve the cortex, including the PMEs, Creuzfeldt-Jakob
disease, Alzheimers disease, posthypoxic brain damage,
and many other metabolic encephalopathies, myoclonus
is a prominent or occasional feature but there may be no
EEG discharge associated with the jerks. In a series of
81 patients with Alzheimers disease, 10% had seizures
or myoclonus, but they never occurred together (26). Per-
haps there is also some subcortical pathology to explain
the myoclonus in these disorders. Other possible expla-
nations are that the cortex participates in a nonepileptic
fashion or that epileptiform cortical discharges are so spa-
tially restricted that they do not reach the scalp.
Hallett and others (4,5,7) distinguished no fewer than
three categories of epileptic myoclonus that involve the
cortex. However, in two of these categories, cortical in-
volvement is not the first event. In cortical reflex my-
oclonus, considered in this schema to be a fragment of
partial epilepsy, there is somatosensory precipitation and a
giant somatosensory-evokedpotential, followedbyactiva-
tion of cranial nerve and segmental myotomes in numeric
(rostral to caudal) order. In reticular reflex myoclonus,
which seems to originate within the brainstem (but in this
schema is considered to be a fragment of a generalized
epilepsy), the cranial nerves are activated caudally to ros-
trally, in reverse numeric order, with cortical involvement
occurring last. Only in primary generalized epileptic my-
oclonus (which is considered to be a fragment of primary
generalized epilepsy) is the cortex the prime mover of the
jerk, with no discernible sequential activation. One can
argue which among these physiological sequences should
be called epilepsy. Other anatomic-physiological subdi-
visions of myoclonic disorders based on correlations of
EMG with EEG, duration of EMG discharges, action or
reflex precipitation, and anatomy can also be made (4,7).
Less ambiguously, there are several quick-movement
syndromes often referred to as myoclonus that are def-
initely of subcortical and nonepileptic origin. These in-
clude palatal myoclonus, probably better described as
clonus since it is rhythmic (at 0.53 Hz) and unipha-
sic, and segmental myoclonus, which is probably bet-
ter described as segmental tremor since it is relatively
rhythmic and biphasic. There is a syndrome of essential
myoclonus, which is familial or sporadic and is associ-
ated with a normal EEG and no other movement disorder
(27). Asterixis may be considered a type of nonepileptic
negative myoclonus. Combinations of myoclonic move-
ments with other disordered movements, such as dystonia
or ballism are also seen; this combination is a good in-
dication of a nonepileptic, subcortical origin. However,
a Parkinsonism-myoclonus combination is a feature of
some PMEs, including a rare adult-onset autosomal dom-
inant form of neuronal ceroid lipofuscinosis (28).
POSTANOXIC MYOCLONUS
Three very different phenomena are referred to as
postanoxic myoclonus. The first two may be considered
epileptic; they are seen soon after rather severe anoxic cor-
tical insult during coma. The third is a late complication
in survivors and may originate subcortically.
Early focal/multifocal postanoxic myoclonus
Close observation of comatose patients often reveals
subtle, low-amplitude facial twitching, eye jerks or rhyth-
mic nystagmus, tonic eye deviation, or low-amplitude
multifocal twitches. The EEG of such patients very fre-
quently reveals irregular generalized or bilateral but asym-
metric spike-wave activity on a slow background. This
phenomenon is considered by some to be a form of non-
convulsive or myoclonic status epilepticus, but may
more accurately be termed subtle generalized convulsive
status (29). It most frequently occurs after an anoxic brain
insult but may also develop from other causes (30). It can
occur as a late stage of convulsive status epilepticus from
any etiology, or as an initial form of status epilepticus af-
ter other severe causes of widespread cortical dysfunction
(29).
Not all multifocal, low-amplitude postanoxic my-
oclonus is associated with an epileptiform EEG. In such
cases, the myoclonus may be subcortical, and antiepilep-
tic drug (AED) treatment is unlikely to be beneficial. This
distinction may not be possible at the bedside, and an
emergent EEG must be obtained.
Epilepsia, Vol. 44, Suppl. 11, 2003
MYOCLONUS PHENOMENOLOGY 11
Early bilateral flexor postanoxic myoclonus
This formof myoclonus is alsoseensoonafter ananoxic
event but implies even more severe cortical damage than
the lower-amplitude postanoxic myoclonus. It consists of
sudden flexion movements of the trunk, sometimes in-
volving the head or extremities, and is sometimes stim-
ulus induced. This form of myoclonus should be distin-
guished from decorticate or decerebrate posturing. The
EEG shows a burst-suppression pattern, and unlike the
subtle postanoxic jerks, the bursts coincide with the flex-
ion spasms. This form of myoclonus has an extremely
poor prognosis. Disagreement persists as to whether this
phenomenon should be considered status epilepticus or
simply an agonal brain rhythm, and whether or not drug
treatment is of any use.
Late postanoxic intention myoclonus
This syndrome, often called the Lance-Adams syn-
drome (31), occurs in survivors of anoxic brain injuries
and consists of interruptions of voluntary movements by
positive jerks or brief negative atonias, both of which are
considered myoclonus. The jerks are not associated with
EEG discharges, are probably of subcortical origin, and
do not improve after treatment with standard AEDs. How-
ever, improvement in this condition associated with pirac-
etam and levetiracetam use has been reported (32).
MYOCLONIC STATUS EPILEPTICUS
If the termsubtle generalized convulsive status epilep-
ticus is used for the phenomenon described previously,
then myoclonic status epilepticus is extremely rare. Gas-
taut divided myoclonic status epilepticus into three types:
Type 1 in idiopathic generalized epilepsy, Type 2 in sec-
ondary generalized epilepsy, and Type 3 as part of an en-
cephalopathy (33). He did not consider Type 3 to be true
status epilepticus. Arguably, the existence of Type 2 is
questionable, and Type 1 is rare.
Patients with JME sometimes have very frequent jerks
or a series of jerks immediately preceding a tonic-clonic
seizure, but the myoclonic seizures are almost never suf-
ficiently continual or prolonged to warrant a designation
as status epilepticus. There are only four well-described
cases of JME in which the jerks recurred continually (ev-
ery 115 s) over a long enough time period (124 h) to be
designated as status epilepticus (3436). In some cases,
what is described as myoclonic status epilepticus is prob-
ably either subtle generalized convulsive status epilepti-
cus or subcortical encephalopathic myoclonus (Gastauts
Type 3) (36). The presence of eye or facial twitches some-
times seen in patients with absence status epilepticus or
motor movements caused by involvement of motor ar-
eas during complex partial status epilepticus does not
constitute a sufficient reason to rename the status event
myoclonic.
CONCLUSION
Myoclonus is not a diagnosis. It is a physical signseenin
diseases of manydifferent pathologies andinnormal phys-
iological situations. Myoclonus is universally understood
to be a quick, involuntary movement, but opinions diverge
as to the boundaries of the term. A narrower definition
that excludes rhythmic movements aids in differentiating
myoclonic disorders from tremors and clonus, including
clonic seizures. However, there is no way to construct a
strictly phenomenological definition of myoclonus since
many movements generally agreed to be myoclonic are
clinically indistinguishable from tics, chorea, startle dis-
orders, and other phenomena. In conclusion, one must
rely mostly on convention, with the rather circular con-
clusion that myoclonus occurs in myoclonic disorders.
Nevertheless, the effort of diagnosis is not trivial and has
major therapeutic and prognostic implications. A good
understanding of the features associated with myoclonic
disorders, particularly the myoclonic epilepsies, and the
features associated with other neurological disorders that
resemble myoclonic disorders, is the best aid to accurate
diagnosis.
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