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Laino D, Mencaroni E, Esposito S. Management of Pediatric Febrile Seizures. 2018.

International Journal
of Environmental Research and Public Health. 2018; 15(-):1-8

a. Vasovagal syncope
b. Reflex anoxic seizures
c. Breath-holding attacks
d. Hyperventilation syncope
e. Compulsive valsalva
f. Neurological syncope
g. Imposed upper airways obstruction
h. Orthostatic intolerance
i. Long QT and cardiac syncope
j. Hyper-cyanotic spells
a. Daydreaming /inattention
b. Self gratification
c. Eidetic imagery
d. Tantrums and rage reactions
e. Out of body experiences
f. Panic attacks
g. Dissociative states
h. Non-epileptic seizures
i. Hallucinations in psychiatric disorders
j. Fabricated / factitious illness
a. Sleep related rhythmic movement disorders
b. Hypnogogic jerks
c. Parasomnias
d. REM sleep disorders
e. Benign neonatal sleep myoclonus
f. Periodic leg movements
g. Narcolepsy-cataplexy
a. Tics
b. Stereotypies
c. Paroxysmal kinesigenic dyskinesia
d. Paroxysmal nonkinesigenic dyskinesia
e. Paroxysmal exercise induced dyskinesia
f. Benign paroxysmal tonic upgaze
g. Episodic ataxias
h. Alternating hemiplegia
i. Hyperekplexia
j. Opsoclonus-myoclonus syndrome
a. Migraine with visual aura
b. Familial hemiplegic migraine
c. Benign paroxysmal torticollis
d. Benign paroxysmal vertigo
e. Cyclical vomiting
a. Benign myoclonus of infancy and shuddering attacks
b. Jitteriness
c. Sandifer syndrome
d. Non-epileptic head drops
e. Spasmus nutans
f. Raised intracranial pressure
g. Paroxysmal extreme pain disorder
h. Spinal myoclonus

There are a range of conditions associated with recurrent paroxysmal events that may imitate and
be misdiagnosed as epilepsies, these are presented in this section of with
reference to the epilepsies that they may imitate and important discriminating features. It is important
that these disorders are considered in the evaluation of paroxysmal events as misdiagnosis rates in
epilepsy are high throughout the world. History remains the key to a correct diagnosis with video
recordings very helpful. There are some conditions in which epileptic and non-epileptic events can


Syncope (fainting) describes the transient loss of consciousness, which occurs when there is an
abrupt reduction in blood flow and oxygen supply to the brain. An anoxic seizure is the collapse,
stiffening with or without tonic-clonic movements that occurs as a result of the lack of cortical control
of the brainstem. The movements are not due to epileptic discharges. Syncope is common, with the
neurally mediated syncopes (vaso-vagal syncope, reflex anoxic seizures, breath holding attacks)
having a lifetime prevalence of 40%.

Vasovagal syncope
Vasovagal syncope affects all ages from infancy to old age though younger children may present
initially with reflex anoxic seizures. A detailed history will usually identify triggers including prolonged
standing, dehydration, change in posture and emotional upset. The setting and stimulus, for example
hair brushing or blow drying after a bath, venepuncture in a doctor's surgery or standing up in a
place of worship are important. There is reduced blood pressure and slowing of the heart rate
causing lack of blood flow to the brain. Early symptoms include blurring and loss of vision, ringing in
the ears and dizziness. Pallor and autonomic symptoms such as flushing, sweating, feeling warm,
nausea and abdominal discomfort may occur. The fall in a vasovagal syncope may be flaccid with a
loss of tone but many people, possibly up to 50%, fall with hips and knees extended with some
moaning or growling as they fall. Visual hallucinations and dreamlike experiences may occur but
unlike epileptic seizures these are not stereotyped. Stiffening and tonic-clonic movements occur in at
least 50% of syncopes. These are brief, lasting seconds, and can be distinguished from tonic
seizures and generalized tonic-clonic seizures due to the shorter duration, triggers, associated
symptoms and recovery. The tonic-clonic movements are typically not rhythmic. Post episode
confusion is typically very brief after syncope whereas confusion greater >10 minutes suggests an
epileptic seizure. Injury can occur in syncope, as can tongue biting (this is often at the tip of the
tongue; whereas lateral tongue biting suggests a tonic-clonic seizure). Urinary incontinence is
common in syncope. A family history of vasovagal syncope is common. A family history of sudden
death, drowning or events triggered by exercise or fright are important clues to a cardiac syncope.
Interictal EEG is not of any diagnostic or prognostic value for syncope. Tilt table testing, which may
trigger syncope, may have some value in special situations, for example if someone has a wrong
diagnosis of epilepsy and confirmation that the diagnosis is syncope is required.

Reflex anoxic seizures

Reflex anoxic seizures or reflex asystolic syncope occurs from early infancy onwards, either
remitting pre-school age or evolving into vasovagal syncope. Alternative names include pallid breath
holding and pallid syncope. In these events an unpleasant, typically sudden stimulus such as a
bump, knock on the head, cut or abrasion leads to a profound vagal discharge with a dramatic drop
in the heart rate and transient asystole. These events are not due to temper tantrums. The child may
cry very briefly or let out a couple of grunts and then becomes exceedingly pale and loses
consciousness. Decerebrate posturing with extensor stiffening may mimic a tonic seizureand be
followed by flexor spasms and irregular tonic-clonic movements however the whole sequence of
abnormal movements will just last a few seconds. Recovery of consciousness may be rapid but
some children may sleep for hours after an event. The events appear very frightening for carers but
have a good prognosis. When reflex anoxic seizures are very frequent, atropine or cardiac pacing
may be considered. There is an uncommon situation in which an anoxic seizure may trigger a
secondary prolonged tonic-clonic seizure; the anoxic-epileptic seizure. The two phases of the event
can be distinguished by a careful history, as in most events individuals will have syncope without the
epileptic component.

Breath-holding attacks
Breath-holding attacks typically affect pre-school children. The child will begin crying after some form
of upset and then stop breathing in expiration with what appears a silent cry or a series of expiratory
grunts. With this prolonged expiratory apnoea the child's face becomes blue with deep cyanosis.
They may recover at this point and breathe in, or go on to a syncope with transient loss of
consciousness. An anoxic seizure identical to that seen in reflex anoxic seizures may occur.
Decerebrate posturing with extensor stiffening may mimic a tonic seizure and be followed by flexor
spasms and irregular tonic-clonic movements however the whole sequence of abnormal movements
will just last a few seconds. Recovery of consciousness may be rapid but some children may sleep
for hours after an event. The events appear very frightening for carers but have a good prognosis. In
contrast to reflex anoxic seizures there is no asystole, and it is thought the syncope is due to a
combination of intrapulmonary shunting, reduced venous return and hypoxia. The attacks are more
common if the child has iron deficiency anaemia. There can be clinical overlap between breath-
holding and reflex anoxic seizures and distinguishing them is not critical except for the very rare
situation when reflex anoxic seizures are so frequent that treatment is considered.

Hyperventilation syncope
Hyperventilation syncope is a transient loss of consciousness with or without an anoxic seizure
triggered by hyperventilation.

Compulsive valsalva
Compulsive valsalva can cause frequent syncope in people with learning disability, particularly those
with autism. Individuals learn that through hyperventilation, followed by breath holding and a valsalva
manoeuvre they can self-induce a syncope. It is presumed that this produces a pleasurable
sensation. These children may also have epileptic seizures, a particular example being Rett
syndrome. Video is invaluable in making the diagnosis.

Neurological syncope
Neurological causes of syncope include Chiari malformation, hyperekplexia (startle disease) and
paroxysmal extreme pain disorder. With a Chiari malformation, coughing and straining on the toilet
may trigger a transient loss of consciousness similar to a syncope. The exact mechanism is
uncertain but increased downward herniation of the brain may compress the vertebro-basilar arteries
and cranial nerves IX and X. A history of chronic headache and sensory symptoms associated with
the collapse should lead to consideration of neuroimaging as decompression surgery can be

Imposed upper airways obstruction

Imposed upper airways obstruction (suffocation) is a rare but important cause of life-threatening
syncope in infants. The events always occur in the presence of a particular individual with others
never seeing the beginning of an event. The syncope takes longer to evolve than in reflex anoxic
seizures or breath holding. This is one form of fabricated or induced illness.

Orthostatic intolerance
Orthostatic intolerance may result in syncope with changes in posture. Chronic orthostatic
intolerance / the postural orthostatic tachycardia syndrome (POTS) in adolescents and adults can
produce symptoms of light-headedness, dizziness, blurred vision, sweating, headache and nausea.

Long QT and cardiac syncope

Long QT and cardiac syncope are important to recognise as they may be life threatening. In long QT
syndrome a ventricular tachyarrhythmia may be spontaneous or triggered by fright, exercise,
surprise, and immersion in water. Syncope in sleep, a strong family history of syncope and a history
of sudden death or drowning should raise suspicions of a cardiac syncope. Sensorineural deafness
is associated with some types of long QT syndrome. A standard 12 lead ECG may be normal and a
strong suspicion should lead to cardiology referral as treatment may be life-saving.

Hyper-cyanotic spells
Hyper-cyanotic spells are most frequently seen in infants with Tetralogy of Fallot (known as 'tet
spells'), however can be seen in other congenital cardiac defects with pulmonary or subpulmonary
stenosis and a ventriculoseptal defect and in pulmonary hypertension. Spells may be precipitated by
tachypnea or tachycardia and by dehydration. Spells may be characterized by crying, panic, rapid
deep breathing, deepening cyanosis, limpness and subsequently a tonic-clonic episode. It is
important that hyper-cyanotic spells are recognized as failure to manage these events correctly can
lead to prolonged hypoxia and death.


Daydreaming /inattention
Daydreaming /inattention is common in childhood and events are frequently misdiagnosed
as absence seizures. Daydreams are often situational (seen more frequently at times when the child
is tired or relaxed or bored) and are longer than absences. Daydreams manifest as the child staring
forward blankly, whilst motionless and not responding to those around them. There is usually no loss
of body tone in a daydream and eyelid flickering does not occur. A daydream may be aborted by
measures to attract the child's attention from the daydream, whereas a child cannot be distracted out
of an absence seizure. Broadly stereotyped motor behaviors may accompany the daydream,
particularly in children with learning disability and autistic features. Daydreams are often more
pronounced in children with attention and learning difficulties.

Self-gratification or self-stimulation includes behavior which may be seen from infancy onwards,
more so in pre-school girls. Rhythmic hip flexion and adduction may be accompanied by a distant
expression, a flushed face and sometimes followed by sleepiness. The distant expression,
sometimes associated with straining and head turning, may be confused with a focal impaired
awareness seizure. The diagnosis is more difficult when the infant or young child seems unhappy
during or after the rhythmic movements. The relative frequency of events and occurrence in specific
circumstances, such as when bored or in a car seat or high chair, lends this behavior to home video
recording. Self-gratification or self-stimulation rather than terms such as masturbation are preferred
by parents and better reflect the mechanism.

Eidetic imagery
Eidetic imagery or childhood preoccupation is an activity engaged in by some children who are able
to enjoy bringing vivid visual images into their mind (eidetic imagery) allowing them to play and
interact in an imaginary visual world. Children may appear to stare into space or have un-vocalized
speech with imaginary individuals and perhaps seem to twitch or move one or more limbs for several
minutes at a time as they interact with the imaginary environment. These events may occur in the
middle of the night and can be misdiagnosed as focal seizures, however in eidetic imagery /
childhood preoccupation the events are longer with more complex interactions than seen in epileptic
events. There is no post-ictal state and with effort a child can be distracted out of the event. They
may be angry at the adult for disturbing their imaginary world.

Tantrums and rage reactions

Tantrums and rage reactions are almost never part of an epileptic seizure. Tantrums are common in
young children and are usually easy to distinguish from an epileptic seizure. Rage reactions,
episodic dyscontrol or intermittent explosive disorder describe situations in which there are recurrent
episodes of rage which seem to be out of proportion to relatively minor stimuli. Sustained outbursts
of aggression may occur for many minutes, sometimes for up to half an hour or longer. There may
screaming, swearing, aggression, damage to property and physical violence. Through the event it
may seem that the individual is not normally responsive. Individuals often report no memory for an
event afterwards, and may express remorse for their actions. Rage reactions are usually much
longer and are only very broadly stereotyped when compared to focal seizures. A rage reaction,
when closely analysed is likely to include a series of complex directed motor tasks, which would be
exceptionally rare for an epileptic seizure. Aggressive or violent behaviors in an epileptic seizure are
very rare, and if seen are typically confused and non-directed actions.

Out of body experiences

Out of body experiences are described in childhood and adulthood. In the episodes a person
appears to lose immediate contact with their bodies and may see himself or herself from above.
Such hallucinations have been described in epileptic seizures, anoxic seizures, migraine and as a
'normal' phenomenon.
Panic attacks
Panic or anxiety attacks are brief episodes, each lasting several minutes, which can recur. A sudden
feeling of apprehension, fear or terror is accompanied by symptoms including breathlessness (with
hyperventilation), choking sensation, palpitations, chest pain, paraesthesia (typically perioral and in
the hands), dizziness, sweating, trembling and feeling faint or loss of consciousness. It may not be
easy to identify a precipitant. Fear may be a manifestation of focal seizures therefore ictal EEG may
be required to make a correct diagnosis.

Dissociative states
Dissociation is a psychological state that may vary in severity from mild detachment from immediate
surroundings to more severe detachment and apparent unresponsiveness. In mild cases,
dissociation can be regarded as a coping mechanism in seeking to minimize or tolerate stress,
conflict or boredom. If a coping response to stress, the dissociative state may be preceded by a
phase where anxiety symptoms (e.g. hyperventilation) are apparent. More severe dissociative states
are seen in dissociative disorders and may include depersonalization and amnesia. Dissociative
disorders are sometimes triggered by traumatic experience, but may be preceded by only minor
stress or there may be no apparent trigger.

Non-epileptic seizures
Non-epileptic seizures (previously known as non-epileptic attacks, psychogenic seizures and
pseudoseizures) resemble epileptic seizures, but have no electrophysiological correlate or clinical
evidence for epilepsy. The etiology of non-epileptic seizures is heterogeneous, with different
predisposing, precipitating and promoting factors in different affected individuals. Psychogenic
factors may promote the emergence of non-epileptic seizures, but psychogenic factors may not be
able to be identified in all cases. The seizure-like event may include movement or impaired
awareness, and can imitate focal motor seizures or focal impaired awareness seizures. Motor
features that distinguish these attacks from seizures include a prominence of proximal or truncal
movements, waxing and waning pattern of movements, variable rate and direction of jerking,
horizontal movements of the head, crying during or after the event, and eye closure with resistance
to passive eye opening. Diagnosing non-epileptic seizures is important because of the potential
serious side effects of anti-seizure medications and associated procedures for treating epilepsy such
as intubation and ventilation. The failure to recognize the psychological factors contributing to these
events also delays implementation of appropriate psychological treatment. Video EEG monitoring
and concomitant psychological evaluation is typically required to establish the diagnosis and the
treatment plan.

Hallucinations in psychiatric disorders

Hallucinations in psychiatric disorders are commonly complex phenomena involving multiple sensory
modalities, in contrast to the elementary sensory hallucinations (colours/flashes of light,
ringing/buzzing sounds etc) that occur in focal epileptic seizures. Complex hallucinations with
hallucinations of seeing people or scenes, hearing voices or formed music and distortions of visual
perception may occur as an uncommon manifestation of focal seizures. The episodic nature of these
phenomena, together with the presence of other features of a seizure and the interval return to a
normal baseline helps distinguish these events from psychiatric hallucinations.

Fabricated / factitious illness

Fabricated / factitious illness may be presented as epilepsy and may be misdiagnosed as such
because of the reliance on truthful accounts of witnesses to make a clinical diagnosis of epilepsy. If
the witness is making up a story this may not be easily apparent to the clinician. There may be
complex psychological, psychosocial and family reasons behind this illness behaviour or it may
simply be because the diagnosis of epilepsy may lead to financial benefits. The illness behaviour
may be on the part of an adult who presents themselves as having seizures or a carer who presents
their child as affected. Factitious illness may be suspected if there are aspects of the clinical history
that seem inconsistent with an epilepsy diagnosis, if the seizures have only been witnessed by one
individual, if frequent seizures are accompanied by normal EEG (including prolonged studies) and if
seizures remain refractory on history to medication however there is no evidence of behavioural or
cognitive comorbidity in the child.


Sleep related rhythmic movement disorders
Sleep related rhythmic movement disorders include body rocking, rolling and head banging. These
are usually benign exaggerations of presumed self-comforting movements or habits that many
infants demonstrate in sleep wake transition as they fall asleep. Sometimes these events can be
accompanied by noise, which disturbs the rest of the family. If they purely occur at sleep wake
transition then good regular habits around sleep can be enough to result in resolution of events. If
events occur in older children and are seen repeatedly through the night, frontal lobe
seizures should be considered. Review of videos of the events is the most useful investigation. An
EEG is likely to be unhelpful unless the EEG is acquired during an event and with video.

Hypnogogic jerks
Sleep starts or hypnic or hypnogogic jerks are normal phenomena experienced by the majority of
children and adults at sleep onset, to variable degrees. They are more common in children with
motor and developmental disorders and are occasionally presented as potentially epileptic events if
they are repetitive or if the child has epileptic seizures at other times. Repetitive sleep starts can
cause recurrent wakening. They may be mistaken for myoclonic seizures or epileptic spasms.

Arousal parasomnias including night terrors, sleep walking and confusional arousals are behaviors
that arise out of deep non-REM sleep (stages 3 & 4), typically in the first third of the nights sleep.
Arousal parasomnias are common and can be regarded as part of normal sleep unless the behavior
produces disruption to the individual or their family. An arousal can vary from sitting up in bed and
making a few minor vocalisations and then lying down again to a night terror in which the individual
rouses, may walk, talk, appear to be agitated or frightened, shout and scream and fail to recognize
family members. These events may be misdiagnosed as temporal lobe seizures however
confusional arousals and night terrors are typically longer and are only broadly stereotyped. The
individual throughout this behavior is still sleeping with slow waves seen on the EEG. Arousal
parasomnias tend to occur more at times of anxiety and psychological stress. There is typically no
recollection of the arousal, however dramatic it is. There is often a family history suggesting a
genetic predisposition. If arousals are occurring more than once a night or every night then the
differential of nocturnal frontal lobe seizures should be considered. Video, including the onset of the
event, is usually the most useful investigation as ictal EEG is often obscured by movement artefact
and deep frontal lobe discharges may not be visible on surface EEG (the ictal EEG may be normal).
Video of multiple events will reveal the stereotyped nature of epileptic seizures. History from the
individual may reveal retained awareness during the event in frontal lobe seizures.
Parasomnias vs frontal lobe seizures:

 Frontal lobe seizures are usually brief events (< 2 minutes), with stereotyped features seen from
seizure to seizure and preserved awareness. Parasomnias are usually longer in duration (> 10
minutes), have variable features from event to event and are characterized by a confusional
state with the patient having no memory of the event afterwards.
 In parasomnias, clustering is rare and the common non-REM parasomnias typically occur 1-2
hours after falling asleep, in the first cycle of deep slow wave sleep. Nocturnal frontal lobe
seizures typically occur throughout the night, and more frequently within half an hour of falling
asleep or awakening.

REM sleep disorders

REM sleep disorders occur when the physiological REM atonia of dreaming sleep does not occur
normally, causing individuals to act out the motor movements of their dreams. Kicking, running,
shouting and even more complex movements can occur. As this behavior arises out of REM sleep, it
is more common in the last third of sleep time and the individual will recall the event. The events will
only be broadly stereotyped if an individual has similar dreams every night. Distinguishing these
events from frontal lobe seizures should be possible with a detailed history however video EEG may
be required. REM behavior disorder may be associated with neurodegenerative conditions in the
elderly and brainstem lesions in younger age groups, therefore detailed neurological assessment is

Benign neonatal sleep myoclonus

Benign neonatal sleep myoclonus is a normal sleep phenomenon which can be very frequent in
some infants leading to misdiagnosis as myoclonic seizures or generalized tonic-clonic seizures.
The movements may begin in the neonatal period and be observed for many months and sometimes
years. The myoclonus only occurs in sleep and the infants have a normal neurological examination,
with normal feeding and behavior. Benign neonatal sleep myoclonus can be identified reliably on
parental home video. Video is usually easily obtained as events are frequent, at predictable times
and prolonged (often occurring over 30 minutes or so). An EEG is not required. On video, myoclonus
is seen to affect all limbs and close observation reveals that there may be synchronous myoclonus
of upper limbs, of lower limbs or all limbs. Sometimes one arm or one leg may be affected. The face
is only exceptionally affected. The myoclonus occurs in brief flurries lasting a second or so with
pauses of variable duration. The myoclonus also varies in amplitude. Waking the child abolishes the
movements. A dramatically exaggerated form of sleep myoclonus is seen in infants of opiate-
dependent mothers. The differential diagnosis for benign neonatal sleep myoclonus is myoclonic
seizures, however neonates with myoclonic seizures would be expected to have a severe early
onset epilepsy, with associated neurological deficits - such neonates would not be expected to feed
and behave normally.

Periodic leg movements

Periodic leg movements in sleep are more common with advancing age but can occur in adults and
children and may be associated with restless legs syndrome. The movements occur principally in
stage 1 and 2 of non REM sleep and are characteristically repetitive stereotyped flexion of toes,
ankles, knees and hips though sometimes the upper limbs may also flex. This may be unpleasant for
the sleeping partner and can cause the affected individual to rouse. Strong jerks of the legs may be
mistaken for myoclonic seizures. In restless legs syndrome there is an urge to move the legs due to
an uncomfortable / unpleasant sensation. There is frequently a family history of restless legs. As well
as genetic factors, other etiologies which should be considered include iron deficiency (particularly in
association with renal disease), magnesium and folate deficiency. A sleep study with
polysomnography including EMG to measure frequency of movements and effect on arousal may be
required. Periodic leg movements and restless legs may respond to medication.

Narcolepsy-cataplexy is a lifelong neurological disorder of sleep state boundary control in which the
distinctions between sleep states, particularly REM sleep, and wakening are blurred. Onset is
typically in the teenage years though it can occur in younger children and begin later in life. It is
thought to be an acquired disorder with an autoimmune mechanism in individuals with a genetic
predisposition. There is a very strong HLA association, evidence of reduced levels of a neuropeptide
called orexin in CSF as well as post mortem evidence of damage to orexin producing neurons in the
hypothalamus. Diagnosis is often delayed for several years and misdiagnosis with epilepsy can
occur for several reasons. The condition is characterised by excessive daytime sleepiness,
cataplexy (loss of tone in response to strong emotion), hypnagogic hallucinations, sleep paralysis
and disturbed night time sleep. Cataplexy comprises a sudden onset of physiological REM atonia
associated with a strong emotion, particularly laughter. This may cause a head drop, sagging of
facial features, buckling of knees and a fall. All or some of these may occur in an event. The
individual remains conscious, though the eyes may close, and through a degree of motor control try
not to fall. This may give the appearance of repetitive jerks. The falls and head drops may be
misdiagnosed as myoclonic seizures, the sagging of the face and lack of response may lead to a
diagnosis of absence seizures. Attacks of sleep or the desire to sleep may lead to a variable level of
responsiveness and a misdiagnosis of focal impaired awareness seizures may be made. The
hypnagogic hallucinations, dreamlike often frightening visions in the awake state may be
misdiagnosed as focal cognitive seizures. A good sleep history, and if possible video of cataplexy,
can be sufficient to make a secure diagnosis but most individuals would require further evaluation
including polysomnography, multiple sleep latency testing, HLA status and in selected cases CSF
orexin estimation. Any association with learning disability or abnormalities on neurological
examination should make the clinician consider Niemann Pick Type C, a brainstem lesion or Coffin
Lowry syndrome.


Tics are involuntary, sudden, rapid, repetitive, non-rhythmic, simple or complex movements or
vocalizations. Simple motor tics involve a single muscle or group of muscles (including ocular
muscles) and may be misdiagnosed as myoclonic seizures. Complex motor tics involve a cluster of
simple actions or coordinated sequence of movements that may be purposeful or non-purposeful
and may be misdiagnosed as focal impaired awareness seizures, particularly in individuals with
learning disability and / or communication problems. Tics are common in childhood and have a
tendency to wax and wane in frequency over time. An urge or compulsion to perform the tic, and an
ability to suppress the tic (to some degree) are important features on history that support the events
being tics.

Stereotypies (or mannerisms) are repetitive movements, postures, or utterances that may be simple
(such as body rocking, head banging) or complex (such as finger movements or wrist
flexion/extension). They may be primary (seen in otherwise normal individuals) or secondary
(associated with autism, intellectual impairment and other disorders). Stereotypies can be
distinguished from epileptic automatisms by the characteristic movements (a video of events can be
helpful to aid diagnosis). Epileptic automatisms are expected to occur in a person who has impaired
awareness, and are seen in association with generalized absence seizures or focal impaired
awareness seizures. Epileptic automatisms can occur in association with preserved awareness, in
non dominant temporal lobe seizures, however in this situation, other features of temporal lobe
seizures are expected to also co-exist.

Paroxysmal kinesigenic dyskinesia

Paroxysmal kinesigenic dyskinesia is a hyperkinetic movement disorder characterised by brief (less
than 1 minute) attacks of abnormal movements triggered by a sudden normal movement. The
triggering movements are typically whole body movements and can include standing up from sitting
or getting out of a car. Some individuals describe a feeling prior to the abnormal movement. This
may be described as a "rush" through the body or a feeling of tightness or numbness. The abnormal
movements are usually dystonic in nature, though they can appear choreiform, and can affect limbs
on one or both sides of the body. Paroxysmal kinesigenic dyskinesia can be sporadic or familial,
inherited in an autosomal dominant fashion, and may co-exist with epilepsy in the syndrome
of familial infantile epilepsy (familial infantile epilepsy and paroxysmal kinesigenic dyskinesia
syndrome, also known as ICCA syndrome). The movement disorder typically has its onset in mid-
childhood or adolescence and may remit in the third decade. Paroxysmal kinesigenic dyskinesia with
or without associated epilepsy is associated with mutations in the PRRT2 gene. Attacks may
mimic frontal lobe seizures however movement as a trigger is the key differentiating feature in the
history. Paroxysmal kinesigenic dyskinesia can respond dramatically to low dose carbamazepine.

Paroxysmal nonkinesigenic dyskinesia

Paroxysmal nonkinesigenic dyskinesia is a hyperkinetic movement disorder characterised by mixed
dystonia, choreoathetosis and dysarthria which last from a few minutes to several hours. The attacks
may be triggered by emotional stress, alcohol or coffee. Attacks typically start in infancy or early
childhood and can be inherited in an autosomal dominant manner associated with mutations in the
myofibrillogenesis (MR-1) gene. The retained awareness and history of triggers should prevent
misdiagnosis as focal seizures.

Paroxysmal exercise induced dyskinesia

Paroxysmal exercise induced dyskinesia represents a genetically heterogenous group of conditions
in which dystonia or choreoathetosis are triggered by exercise. It may be inherited in an autosomal
dominant manner or present as a sporadic case. Attacks may last several minutes to half an hour
and more frequently affect the lower than upper limbs. This is one of the many phenotypes
associated with glucose transporter 1 (GLUT1) deficiency which is typically caused by mutations in
the SLC2A1 gene.

Benign paroxysmal tonic upgaze

Benign paroxysmal tonic upgaze presents in early infancy and is characterised by prolonged or
intermittent upgaze which may last hours or days. Children may be ataxic during episodes and
attacks occur more frequently during intercurrent illnesses. The attacks remit after a few years but
can be associated with learning disability in a significant proportion of individuals.

Episodic ataxias
Episodic ataxias are rare autosomal dominant disorders divided into two major categories: episodic
ataxia type 1 (EA1) and 2 (EA2) both of which are channelopathies in which a movement disorder
and epilepsy may co-exist.
EA1 is associated with mutations in a potassium ion channel gene KCNA1. Brief episodes of
cerebellar ataxia lasting seconds or minutes are triggered by sudden movements, emotion or
intercurrent illness. Onset is typically in mid childhood with attacks occurring throughout life, though
frequency may vary significantly with long periods of remission. During the episodes the individual
may have dysarthria, limb and gait ataxia and titubation (coarse tremor) of the head. The
movements may appear dystonic or choreiform in some individuals therefore misdiagnosis as
paroxysmal kinesigenic dyskinesia or focal seizures may occur. The potassium channel mutation
also causes peripheral nerve hyperexcitability which results in continuous stimulation of muscles.
This may give the appearance of subtle rippling of muscles (myokymia) seen best under the eyelids
or as continuous side to side movements of the fingers seen when the hands are outstretched. With
intercurrent illness, particularly vomiting illnesses, the continuous stimulation of muscles may cause
generalized stiffness (neuromyotonia). This may also be seen independent of illness in early infancy,
with apparent (non-fixed) flexion contractures of the limbs and fisting of hands which gradually
lessens over the first year. About 10% of people with EA1 have epileptic seizures, these may
be focal seizures which can evolve to focal to bilateral tonic-clonic seizures. These epileptic seizures
are a manifestation of the ion channel mutation causing neuronal hyperexcitability.
EA2 is characterised by periods of cerebellar ataxia lasting minutes to hours, which are triggered by
physical and emotional stress. Gait and upper limb ataxia may be accompanied by dysarthria,
nystagmus, vertigo, nausea and headache. EA2 can be distinguished from seizures by recognition
of triggers, family history and retention of awareness during events. EA2 is associated with
mutations in the calcium ion channel gene CACNA1A. Variants in this gene are associated with
familial hemiplegic migraine and spinocerebellar ataxia type 6 and some phenotypic overlap with
these disorders may occur. There may be gaze-evoked nystagmus in between episodes and over
time vertical nystagmus may develop. As events are prolonged it should be possible to capture them
on home video. Acetazolamide can be a very effective treatment.

Alternating hemiplegia
Alternating hemiplegia of childhood is a rare disorder, with onset in the first year of life, characterised
by recurrent attacks of hemiplegia affecting either side of the body. There may be bilateral weakness
from the onset of episodes or during the attacks. Attacks may last minutes to more than half an hour.
Other signs include nystagmus, pallor, crying, eye deviation, autonomic symptoms and dystonic and
tonic elements during the episodes, with choreoathetosis between episodes. Events may be
mistaken for focal seizures. Parkinsonian features may develop over time. Events may be triggered
by stress, water, certain foods and exercise. Sleep allows the symptoms in an episode to resolve,
however they may return 10-20 minutes after waking. Affected infants have learning disability and
abnormal motor development. A significant proportion of individuals will also have focal seizures.
The vast majority of individuals (about 80%), have mutations in the ATP1A3 gene.

Hyperekplexia is characterised by an exaggeration of the normal startle response and has several
genetic associations (GLRA1, GPHN, GLRB, ARHGEF9 and SLC6A5) all linked to dysfunction of
the inhibitory glycinergic pathway in the nervous system. Symptoms are evident from the neonatal
period or early infancy. Infants are commonly hypertonic, with rigidity, rather than spasticity, which is
relieved by sleep. In response to normal touch, noise or any unexpected stimulus they can startle
excessively with flexion of the limbs and retraction of the head. A gentle tap using the tip of the
examiner's finger on the tip of the individual's nose should trigger an excessive startle that does not
habituate with repeated nose taps. The startle may be a rapid jerk or series of jerks, which can
mimic a myoclonic, tonic or tonic-clonic seizure. If an EEG is performed during an episode of
stiffening, rhythmic muscle action potentials may be misdiagnosed as spikes. A severe startle
response may be associated with apnoea and cyanosis. Severe attacks are particularly linked to
SLC6A5 mutations and may be linked to sudden infant death in this syndrome. Severe attacks can
be aborted by flexing the trunk and neck of the child - the Vigevano manouvre. Clonazepam may be
effective in reducing the startle and increased tone. The symptoms tend to resolve after infancy, but
adults may have increased startle-induced falls and/or experience nocturnal muscle jerks. There are
rarer subtypes of hyperekplexia associated with mutations in the gephyrin and collybistin genes in
which epilepsy can co-exist. The onset of excessive startle in later childhood or adult life may be
associated with development of autoantibodies to the glycine receptor.

Opsoclonus-myoclonus syndrome
Opsoclonus-myoclonus syndrome is an autoimmune neurological disorder that may be seen in
association with neuroblastoma, following viral infections or may be of unknown cause. The earliest
feature is often ataxia followed by opsoclonus (multidirectional, erratic, darting eye movements).
Myoclonus is a mixture of small- and larger-amplitude muscle jerks, giving rise to a tremulous
appearance. Myoclonus is primarily action-induced, but in severe cases is present at rest. The eye
movement abnormality and myoclonus may initially be thought to be epileptic, however the pattern of
eye movements allows them to be distinguished from eye movements seen in typical absence


Migraine with visual aura
Migraine with aura and its variants are very common and it is well established that migraine and
epilepsy often co-exist as co-morbid disorders. The visual aura of migraine preceding a headache
can take a variety of forms but is typically in one visual field and contains positive phenomena such
as flashes, arcs of lights (fortification spectra), specks, or flames and negative phenomena such as
scotoma with blanking out or greying of the visual field. Visual phenomena of occipital seizures are
more likely to be coloured and can include a variety of different shapes including diamonds, squares,
circles and lines. More complex sensory illusions or perceptions that may or may not precede a
headache include the feeling that a body part or parts, such as the hands, have grown dramatically
or shrunk or that everything in the environment is louder. These may be misdiagnosed as a seizure,
but are more likely to be a feature of the Alice in Wonderland syndrome, considered a migraine

Familial hemiplegic migraine

Familial hemiplegic migraine is a subtype of migraine with aura in which focal weakness +/- speech
disturbance, visual symptoms and paraesthesia develop prior to the onset of headache. Confusion
and, in rare severe cases, coma may accompany weakness. The sequence of symptoms and the
familial nature should prevent misdiagnosis as focal seizures, though sporadic cases due to
presumed de novo mutations occur. Mutations in three genes CACNA1A, ATP1A2 and SCN1A are
associated with familial hemiplegic migraine. Severe attacks may be triggered by trauma and
intercurrent illness. Phenotypic overlap with episodic ataxia type 2 may occur.

Benign paroxysmal torticollis

Benign paroxysmal torticollis is considered a migraine variant of infancy and early childhood. Attacks
of retro-, lateral or torticollis may last minutes to hours. The infant may have associated pallor,
vomiting and appear distressed. Older children may have ataxia. In later childhood affected
individuals may develop migraine. Rare cases have been associated with mutations in
the CACNA1A gene.

Benign paroxysmal vertigo

Benign paroxysmal vertigo is considered a migraine variant of childhood and is characterized by a
subjective experience described by the child of the world spinning (vertigo). Affected children may
appear anxious and will want to stop moving, hold onto an adult or lie down. The episodes last
minutes to, less commonly, hours and may be accompanied by vomiting and nystagmus. They may
follow on from benign paroxysmal torticollis and may evolve into migraine. Attacks may mimic focal

Cyclical vomiting
Cyclical vomiting is characterized by stereotyped periods of recurrent vomiting which may last hours
to days and may be separated by weeks during which the individual has no symptoms. It is
considered a migraine variant as there is often a family history of migraine headache, though the
pathophysiology is not well understood. Usually no trigger to a particular episode can be defined.
Recurrent vomiting may produce physiological disturbance but awareness should not be impaired
and the attacks are longer than expected in focal seizures. Episodes may begin in early childhood
and evolve into abdominal migraine and then to classic migraine with headache. If the vomiting
persists into adult life it remains paroxysmal but may be less cyclical in nature.

Benign myoclonus of infancy and shuddering attacks
Benign myoclonus of infancy and shuddering attacks are both benign (self limiting) variants of
normal behaviour in infants and may be related conditions with differing durations of attacks. These
attacks typically have onset from 4 months of age and can persist up to the age of 6-7 years, with a
remitting and relapsing course. Attacks can be very frequent and can last a few seconds in duration.
Attacks can be triggered by certain activities such as feeding, head movements or certain tasks. The
events may be a form of self-stimulation and are most likely to occur in the high chair or car seat. In
these events children have a brief bilateral jerk or more sustained shudder (vibratory tonic flexion of
head and trunk), sometimes associated with a change in facial expression and flexion of the upper
limbs. The events do not cause the child any distress and they return to their previous activity with
no impairment of awareness during the episode. They may be misdiagnosed as myoclonic
seizures or epileptic spasms. Shuddering attacks may be one form of benign non-epileptic spasms.
A video recording of events is the most useful investigation.

Jitteriness is common in the newborn period, commonly occurring in an otherwise well-appearing
infant on the first day of life as a transient, self-limiting finding. Medical causes of jitteriness may
include hypocalcemia and neonatal abstinence syndrome. Jitteriness can be distinguished from
epileptic seizures as it can be increased when the infant is unwrapped, stimulated, startled or crying,
but suppresses when the infant is wrapped or the affected limb is held gently.

Sandifer syndrome
This syndrome is seen in young children with gastro-oesophageal reflux (with or without vomiting).
Events are often seen with or after feeding. Typically there is arching of the back, dystonic posturing
of the limbs and turning/tilting of the head. The events may be frequent. The arching of the back and
the trigger of events during or after feeding are key features that distinguish this disorder from
epileptic seizures. Early treatment of the gastro-oesophageal reflux results in resolution of

Non-epileptic head drops

Non-epileptic head drops can occur in infants and may mimic epileptic spasms or atonic seizures.
Infants typically experience frequent (more than a hundred) events per day, the head drop can be
intense, may occur in a series (resulting in head bobbing) and may be accompanied by crying. The
fall of the head (flexion of the neck) and rise of the head occur at the same velocity unlike in an
epileptic seizure where the fall of the head is typically more rapid than the rise of the head. Non-
epileptic head drops begin between age 3 - 6 months, and resolve by 12 months. Infants develop

Spasmus nutans
This disorder of eye movement is seen in infants, typically with onset between 4 and 12 months of
age. The cause is unknown; neuroimaging is required to exclude structural brain abnormalities.
Vertical eye movements occur, these are rapid and side-to-side. There may be a head tilt and head
nodding. The events resolve with time.
Raised intracranial pressure
Raised intracranial pressure may cause decerebrate or decorticate posturing, which can be
paroxysmal and mistaken for tonic-clonic ortonic seizures. Affected individuals with raised
intracranial pressure are expected to show signs of encephalopathy including alteration in conscious
level (not improving in the minutes after the event, as one would expect in a post-ictal state),
abnormalities of tone and reflexes and pupillary abnormality.

Paroxysmal extreme pain disorder

Paroxysmal extreme pain disorder is a rare genetic condition associated with mutations in the
sodium ion channel gene SCN9A and is characterised by paroxysms of extreme pain. It was
previously known as familial rectal pain syndrome. Symptoms begin in infancy, often on the first day
of life, and may be life-long. Gain of function mutations in SCN9A result in abnormal pain
transmission (loss of function mutations result in insensitivity to pain). Excruciating attacks of pain
occur affecting the perineal area (buttocks, rectum, genitals), the mouth and jaw or the eyes.
Triggers to events tend to reflect the distribution of the pain. Changing nappies, defecating or wiping
the perineal area may trigger an episode of perineal pain. Eating may trigger pain in the mouth and a
cold wind may trigger attacks in the eye. The pain is described as extreme and burning or stabbing.
The episode is typically accompanied by autonomic features with flushing of the body, which may be
harlequin-like, affecting one limb or one side of the face or body. The episodes may last from
seconds to minutes, rarely they can be more prolonged. Events are most prominent in early infancy
and in this age group bradycardia and asystole may accompany the attack, resulting in syncope and
a non-epileptic anoxic seizure with tonic posturing. The events may be misdiagnosed as tonic
seizures. Though rare, this is an important diagnosis, as it is extremely distressing for the individual
affected and symptoms may improve with treatment with carbamazepine.

Spinal myoclonus
Spinal myoclonus results in myoclonic jerks of the body that may not be modified by sleep or by
voluntary action (therefore it may be present awake and asleep and at rest or during movement).
Spinal segmental myoclonus is usually symptomatic of an underlying structural spinal lesion such as
syringomyelia. It is confined to one or few contiguous myotomes and may occur irregularly or
quasirhythmically, with a variable frequency. Propriospinal myoclonus is a form of spinal myoclonus
where the axial muscles are recruited extensively along long propriospinal pathways. Typically, there
are axial flexion jerks involving the neck, trunk and hips with a frequency of 1-6 Hz. Propriospinal
myoclonus typically occurs spontaneously, especially in the recumbent position, or may be provoked
by tapping of the abdomen or by eliciting tendon reflexes. As opposed to segmental myoclonus,
most patients with propriospinal myoclonus have no clear etiology, although psychogenic forms are
increasingly recognized. Brainstem myoclonus, although also axial in distribution, can be
distinguished from propriospinal myoclonus as there is involvement of the face and myoclonus may
be triggered by auditory stimuli.
Kejang adalah perubahan bersifat paroksismal dari fungsi neurologis yang disebabkan oleh
pelepasan neuron yang berlebihan dan hipersinkronisasi dalam otak.
"Kejang epileptik" digunakan untuk membedakan kejang yang disebabkan oleh hiperaktivasi
neuron abnormal dari kejadian kejang nonepilepsi, seperti kejang psikogenik. Epilepsi adalah
kejang berulang tanpa penyebab yang jelas. Epilepsi memiliki banyak penyebab, masing-masing
mencerminkan disfungsi otak yang mendasarinya (Shorvon et al. 2011). Kejang yang diprovokasi
oleh kejadian reversibel (mis. demam, hipoglikemia) tidak termasuk dalam definisi epilepsi karena
merupakan kondisi sekunder dan bersifat akut, bukan kondisi kronis.
"Sindrom epilepsi" mengacu pada sekelompok karakteristik klinis yang konsisten terjadi bersama-
sama, dengan tipe kejang yang serupa, berdasarkan onset penyakit, temuan EEG, faktor pemicu,
genetika, riwayat alami, prognosis, dan respons terhadap obat antiepilepsi (AED). Istilah yang
tidak spesifik seperti gangguan kejang (EN: seizure disorder) harus dihindari.
Epilepsi adalah salah satu kondisi neurologis yang paling umum, dengan insidensi sekitar 50 kasus
baru per tahun per 100.000 populasi (Hauser dan Hersdorffer 1990). Sekitar 1% populasi
menderita epilepsi, dan sekitar sepertiga dari pasien memiliki epilepsi refrakter (mis., kejang yang
tidak dapat dikendalikan oleh dua atau lebih obat antiepilepsi yang lebih tepat dipilih atau terapi
lain). Sekitar 75% epilepsi dimulai selama masa kanak-kanak, mencerminkan kerentanan yang
meningkat bagi otak yang sedang berkembang untuk kejang.
Kejang merupakan kelainan neurologi yang paling sering terjadi pada anak, di mana

ditemukan 4 – 10 % anak-anak mengalami setidaknya satu kali kejang pada 16 tahun pertama

kehidupan. Studi yang ada menunjukkan bahwa 150.000 anak mengalami kejang tiap tahun, di

mana terdapat 30.000 anak yang berkembang menjadi penderita epilepsi.17 Faktor resiko

terjadinya epilepsi sangat beragam, di antaranya adalah infeksi SSP, trauma kepala, tumor,

penyakit degeneratif, dan penyakit metabolik. Meskipun terdapat bermacam-macam faktor resiko

tetapi sekitar 60 % kasus epilepsi tidak dapat ditemukan penyebab yang pasti. Berdasarkan jenis

kelamin, ditemukan bahwa insidensi epilepsi pada anak laki – laki lebih tinggi daripada anak

Kejang adalah kedaruratan neurologis yang sering dijumpai pada praktik sehari-hari.

Hampir 5% anak berumur di bawah 16 tahun minimal pernah mengalami satu kali kejang.

Sebanyak 21% kejang pada anak terjadi pada 64% dalam lima tahun pertama.

Kejang dapat sederhana, berhenti sendiri, memerlukan pengobatan lanjutan, merupakan

gejala awal suatu penyakit berat dan menjadi status epileptikus. Langkah awal dalam menghadapi

kejang adalah memastikan bahwa anak memang kejang. Tata laksana kejang meliputi stabilisasi

pasien, identifikasi etiologi, terapí sesuai dengan etiologi, dan pemantauan secara

berkesinambungan. Pada makalah ini akan dibahas mengenai kejang sederhana hingga status



Kejang adalah manifestasi klinis yang disebabkan oleh lepasnya muatan listrik di neuron.

Kejang dapat disertai oleh gangguan kesadaran, tingkah laku, emosi, motorik, sensorik dan atau


Kejang dapat dibagi atas kejang fokal dan kejang umum. Kejang fokasl berasal dari fokus

lokal di otak, dapat melibatkan sistem motorik, sensorik maupun psikomotor. Kejang umum

melibatkan kedua hemisfer, dapat berupa kejang non-konvulsif (absans) dan konvulsif.


Patofisiologi kejang pada tingkat selular berhubungan dengan terjadinya paroxysmal

depolarization shift (PDS) yaitu depolarisasi potensial pascasinaps yang berlangsung lama (50

ms). Paroxysmal depolarization shift merangsang lepas muatan listrik yang berlebihan pada

neuron otak dan merangsang sel neuron lain untuk melepaskan muatan listrik secara bersama-sama

sehingga timbul hipereksitabilitas neuron otak.

Paroxysmal depolarization shift diduga disebabkan oleh kemampuan membran sel

melepaskan muatan listrik yang berlebihan, berkurangnya inhibisi oleh neurotransmiter asam

gama amino butirat (GABA), atau meningkatnya ekssitasi sinaptik oleh neurotransmitter glutamat

dan aspartat melalui jalur eksitasi yang berulang.

Pada pasien dengan epilepsi fokal, terdapat sekelompok sel neuron yang bertindak sebagai

pacemaker lepasnya muatan listrik disebut sebagai fokus epileptikus. Sekelompok sel neuron ini

akan merangsang sel di sekitarnya untuk melepaskan muatan listriknya. Keadaan ini merupakan

transisi fokal interiktal atau gelombang paku iktal pada elektroensefalografi.

Manifestasi klinis bergantung pada luasnya sel neuron yang tereksitasi. Pasien epilepsi

umum pembentukan gelombang paku-ombak terjadi pada struktur korteks. Terdapat penyebaran

cepat proses eksitasi (spike) dan inhibisi (gelombang ombak) pada kedua hemisfer otak melalui

jaras kortikoretikular dan talamokortikal. Status epileptikus terjadi akibat proses eksitasi yang

berlebihan berlangsung terus menerus yang diikuti oleh proses inhibisi yang tidak sempurna.

Diagnosis kejang ditegakkan berdasarkan anamnesis dan akan lebih mudah bila serangan

kejang tersebut terjadi di hadapan kita. Pada awal penanganan, sangatlah penting membedakan

berdasarkan anamnesis dan pemeriksaan fisis apakah serangan yang terjadi adalah kejang atau

serangan yang menyerupai kejang. Perbedaan di antara keduanya dapat dilihat pada Tabel 5.1.

Perlu diingat bahwa pada pasien epilepsi dapat terjadi serangan yang menyerupai kejang, seperti

aritmia, sinkop atau distonia. Oleh karenanya, deskripsi akurat dari serangan saat itu sangat



Jenis kejang dapat ditentukan berdasarkan deskripsi serangan yang akurat. Penentuan jenis

kejang ini sangatlah penting untuk menentukan jenis terapi yang akan diberikan. Pemilihan obat

anti kejang/obat anti epilepsi (OAE) jangka panjang sangat dipengaruhi oleh jenis kejang pasien.

Ada obat diindikasikan untuk jenis kejang tertentu, misalnya karbamazepin untuk jenis kejang

fokal atau asam valproat untuk kejang tipe absans. Pemilihan OAE yang salah dapat memperberat

jenis kejang tertentu, misalnya penggunaan karbamazepin dan fenitoin dapat memperberat kejang

umum. Idiopatik seperti kejang absans, atonik, dan mioklonik.

Saat ini klasifikasi kejang yang digunakan adalah berdasarkan Klasifikasi International

League Against Epilepsy of Epileptic Seizures tahun 1981 (Tabel 5.2). Jenis kejang harus

ditentukan setiap kali pasien mengalami serangan. Tidak jarang ditemukan bahwa jenis kejang saat

ini berbeda dengan sebelumnya. Semakin banyak jenis serangan kejang yang dialami pasien,

semakin sulit penanganan kejang dan pemilihan obat anti kejang.


Penentuan etiologi kejang berperan penting dalam tata laksana kejang selanjutnya.

Keadaan ini sangat penting terutama pada kejang yang sulit diatasi atau kejang berulang. Etiologi

kejang pada seorang pasien dapat lebih dari satu. Etiologi kejang yang tersering pada anak dapat

dilihat pada Tabel 5.3.

Anamnesis dan pemeriksaan fisis

Anamnesis dan pemeriksaan fisis yang baik diperlukan untuk memilih pemeriksaan

penunjang yang terarah dan tata laksana selanjutnya. Aloanamnesis dimulai dari riwayat

perjalanan penyakit sampai terjadinya kejang, dilanjutkan dengan pertanyaan terarah untuk

mencari kemungkinan faktor pencetus atau penyebab kejang. Anamnesis diarahkan pada riwayat

kejang sebelumnya, kondisi medis yang berhubungan, obat-obatan, trauma, gejala infeksi,

gangguan neurologis baik umum maupun fokal, serta nyeri atau cedera akibat kejang.

Pemeriksaan fisis dimulai dengan menilai tanda vital, mencari tanda trauma akut kepala,

dan ada tidaknya kelainan sistemik. Pemeriksaan ditujukan mencari cedera yang terjadi

mendahului atau selama kejang, adanya penyakit sistemik, paparan zat toksik, infeksi, dan

kelainan neurologis fokal. Bila dijumpai kelainan fokal, misalnya paralisis Todd's, harus dicurigai

adanya lesi intrakranial. Bila terjadi penurunan kesadaran perlu dilakukan pemeriksaan lanjutan

untuk mencari faktor penyebab. Edema papil yang disertai tanda rangsang meningeal

menunjukkan adanya peningkatan tekanan intrakranial akibat infeksi susunan saraf pusat.


Untuk menentukan faktor penyebab dan komplikasi kejang pada anak, diperlukan beberapa

pemeriksaan penunjang meliputi pemeriksaan laboratorium, pungsi lumbal, elektroensefalografi,

dan pencitraan neurologis. Pemilihan jenis pemeriksaan penunjang ini ditentukan sesuai dengan


a. Pemeriksaan laboratorium

Pemeriksaan laboratorium pada anak dengan kejang berguna untuk mencari etiologi dan

komplikasi akibat kejang lama. Jenis pemeriksaan yang dilakukan bergantung pada kondisi klinis

pasien. Pemeriksaan yang dianjurkan pada pasien dengan kejang lama adalah kadar glukosa darah,

elektrolit, darah perifer lengkap, dan masa protrombin. Pemeriksaan laboratorium tersebut bukan

pemeriksaan rutin pada kejang demam. Jika dicurigai adanya meningitis bakterialis perlu

dilakukan pemeriksaan kultur darah dan kultur cairan serebrospinal. Pemeriksaan polymerase

chain reaction (PCR) terhadap virus herpes simpleks dilakukan pada kasus dengan kecurigaan


b. Pungsi lumbal

Pungsi lumbal dapat dipertimbangkan pada pasien kejang disertai penurunan kesadaran

atau gangguan status mental, perdarahan kulit, kaku kuduk, kejang lama, gejala infeksi, paresis,

peningkatan sel darah putih, atau pada kasus yang tidak didapatkan faktor pencetus yang jelas.

Pungsi lumbal ulang dapat dilakukan dalam 48 atau 72 jam setelah pungsi lumbal yang pertama

untuk memastikan adanya infeksi susunan saraf pusat. Bila didapatkan kelainan neurologis fokal

dan peningkatan tekanan intrakranial, dianjurkan melakukan pemeriksaan CT Scan kepala terlebih

dahulu untuk mencegah risiko terjadinya herniasi.

The American Academy of Pediatrics merekomendasikan bahwa pemeriksaan pungsi

lumbal sangat dianjurkan pada serangan kejang pertama disertai demam pada anak usia di bawah

12 bulan karena manifestasi klinis meningitis tidak jelas atau bahkan tidak ada. Pada anak usia 12-
18 bulan dianjurkan melakukan pungsi lumbal, sedangkan pada usia lebih dari 18 bulan pungsi

lumbal dilakukan bila terdapat kecurigaan adanya infeksi intrakranial (meningitis).

c. Elektroensefalografi

Pemeriksaan EEG digunakan untuk mengetahui adanya gelombang epileptiform.

Pemeriksaan EEG mempunyai keterbatasan, khususnya interiktal EEG. Beberapa anak tanpa

kejang secara klinis ternyata memperlihatkan gambaran EEG epileptiform, sedangkan anak lain

dengan epilepsi berat mempunyai gambaran interiktal EEG yang normal. Sensitivitas EEG

interiktal bervariasi. Hanya sindrom epilepsi saja yang menunjukkan kelainan EEG yang khas.

Abnormalitas EEG berhubungan dengan manifestasi klinis kejang, dapat berupa gelombang paku,

tajam dengan/atau tanpa gelombang lambat. Kelainan dapat bersifat umum, multifokal, atau fokal

pada daerah temporal maupun frontal.

Pemeriksaan EEG segera dalam 24-48 jam setelah kejang atau sleep deprivation dapat

memperlihatkan berbagai macam kelainan. Beratnya kelainan EEG tidak selalu berhubungan

dengan beratnya klinis. Gambaran EEG yang normal atau memperlihatkan kelainan minimal

menunjukkan kemungkinan pasien terbebas dari kejang setelah obat antiepilepsi dihentikan.

d. Pencitraan neurologis

Foto polos kepala memiliki nilai diagnostik kecil meskipun dapat menunjukkan adanya

fraktur tulang tengkorak. Kelainan jaringan otak pada trauma kepala dideteksi dengan CT scan

kepala. Kelainan gambaran CT scan kepala dapat ditemukan pada pasien kejang dengan riwayat

trauma kepala, pemeriksaan neurologis yang abnormal, perubahan pola kejang, kejang berulang,

riwayat menderita penyakit susunan saraf pusat, kejang fokal, dan riwayat keganasan.
Magnetic Resonance Imaging (MRI) lebih superior dibandingkan CT scan dalam

mengevaluasi lesi epileptogenik yang tertutup struktur tulang misalnya daerah serebelum atau

batang otak. MRI dipertimbangkan pada anak dengan kejang yang sulit diatasi, epilepsi lobus

temporalis, perkembangan terlambat tanpa adanya kelainan pada CT scan, dan adanya lesi

ekuivokal pada CT scan.


Umumnya kejang tonik klonik berhenti spontan dalam 5 menit. Bila kejang tidak berhenti

dalam 5 menit, maka kejang cenderung berlangsung lama. Status epileptikus (SE) adalah kejang

lama lebih dari 30 menit atau kejang berulang tanpa pulihnya kesadaran di antara kejang. Terdapat

dua jenis status spileptikus yaitu SE konvulsif (parsial/fokal motorik dan tonik klonik umum) dan

SE non-konvulsif (absans dan parsial kompleks).

Status epileptikus konvulsif pada anak merupakan kegawatan yang mengancam jiwa

dengan risiko terjadinya gejala sisa neurologis. Risiko ini tergantung pada penyebab dan lamanya

kejang. Makin lama kejang berlangsung, makin sulit untuk menghentikannya.

Tujuan tata laksana kejang tonik klonik umum lebih dari 5 menit adalah menghentikan

kejang dan mencegah terjadinya status epileptikus.

Langkah-langkah penanganan kejang terbagi atas tata laksana fase akut dan fase meliputi
a. Fase akut: penghentian kejang

0-5 menit:

 Yakinkan bahwa aliran udara pernapasan baik

 Monitor tanda vital, berikan oksigen, pertahankan perfusi oksigen ke jaringan.

 Bila keadaan pasien stabil, lakukan anamnesis terarah, pemeriksaan umum dan neurologis

secara cepat.

 Cari tanda-tanda trauma, kelumpuhan fokal, dan infeksi.

5-10 menit

 Pemasangan akses intravena.

 Pengambilan darah untuk pemeriksaan: darah perifer lengkap, glukosa, dan


 Pemberian diazepam 0,2-0,5 mg/kgBB secara intravena (kecepatan 5

mg/menit), atau dapat diberikan diazepam rektal 0,5 mg/kgBB (untuk berat badan <10 kg

diberikan 5 mg, bila berat badan > 10 kg diberikan 10 mg, dosis maksimal 10 mg/kali).

 Atau dapat diberikan lorazepam 0,05-0,1 mg/kgBB intravena (maksimum 4

mg). Alternatif lain adalah midazolam 0,05-0,1 mg/kgBB intravena. Pemberian diazepam

intravena atau rektal dapat diulang 1-2 kali setelah 5-10 menit, lorazepam 0,1mg/kgBB

dapat diulang sekali setelah 10 menit

 Jika didapatkan hipoglikemia, berikan cairan dekstrosa 25% 2 ml/kgBB.

10-15 menit

 Cenderung menjadi status konvulsivus.

 Berikan fenitoin 15-20 mg/kgBB intravena diencerkan dengan NaCl 0,9%

diberikan dengan kecepatan 25-50 mg/menit.

 Dapat diberikan dosis ulangan fenitoin 5-10 mg/kgBB, sampai maksimum

dosis 30 mg/kgBB.

Lebih dari 30 menit

 Pemberian antikonvulsan masa kerja panjang (long acting).

 Fenobarbital 10 mg/kgBB intravena bolus perlahan-lahan dengan kecepatan

100 mg/menit. Dapat diberikan dosis tambahan 5-10 mg/kgBB dengan interval 10-15


 Pemeriksaan laboratorium sesuai kebutuhan meliputi analisis gas darah,

elektrolit. gula darah. Koreksi kelainan yang ada. Awasi tanda-tanda depresi pernapasan.

 Bila kejang masih berlangsung siapkan intubasi dan kirim ke Unit

Perawatan Intensif. Berikan fenobarbital 5-8 mg/kgBB secara bolus intravena, diikuti

rumatan fenobarbital drip dengan dosis 3-5 mg/kgBB/jam (Diagram 5.1).

Penanganan pasien dengan status konvulsivus/epileptikus tidak hanya bertujuan untuk

menghentikan kejang, tetapi juga mencegah terjadinya komplikasi sistemik yang timbul pasca

status konvulsivus. Pengenalan dini, intervensi yang adekuat, dan pencegahan komplikasi penting

untuk prognosis pasien.

Pada kejang lama dapat terjadi hipoksia terjadi akibat gangguan ventilasi, sekresi air liur

dan sekret trakeobronkial yang berlebihan, serta peningkatan kebutuhan oksigen. Hipoksia

mengakibatkan asidosis, yang selanjutnya menyebabkan penurunan fungsi ventrikel jantung,

penurunan curah jantung, hipotensi, dan mengganggu fungsi sel dan neurorn.
Pada SE terjadi pengeluaran katekolamin dan perangsangan saraf simpatis yang

menyebabkan peningkatan tekanan darah, denyut jantung, dan tekanan vena sentral. Edema otak

terjadi akibat adanya hipoksia, asidosis, atau hipotensi. Pada kejang yang tidak dapat teratasi, dapat

terjadi hiperpireksia sehingga dapat terjadi mioglobinuria dan peningkatan kreatin fosfokinase

akibat rabdomiolisis. Beberapa macam obat yang sering digunakan untuk mengatasi status

konvulsivus dapat dilihat pada Tabel 5.4.

Pengobatan jangka panjang

Pengobatan pada pasien yang mengalami kejang simptomatik akut ditujukan pada faktor

penyebab. Apabila faktor penyebab dapat segera diobati, maka tidak diperlukan pemberian obat

anti epilepsi jangka panjang. Risiko berulangnya kejang terjadi dalam satu tahun pertama,

khususnya dalam tiga bulan pertama. Bila selama tiga bulan pertama tanpa pengobatan tidak

didapatkan kejang, maka pasien tidak memerlukan pengobatan jangka panjang.

Pengobatan selalu dimulai satu jenis obat (monoterapi). Dosis dinaikkan dengan dengan

titrasi sampai tercapai konsentrasi terapeutik serum atau dosis terapeutik. Jika dengan dosis

maksimal kejang masih tidak terkontrol, pertimbangkan kombinasi terapi dengan OAE lainnya.

Jika kejang terkontrol, pertimbangkan penurunan dosis OAE yang pertama kali diberikan. Tidak

ada satu jenis OAE yang merupakan pilihan utama untuk semua jenis epilepsi. Beberapa OAE

lebih efektif untuk jenis kejang tertentu atau sindrom tertentu. Saat ini pengobatan jangka panjang

yang dianjurkan adalah selama dua atau tiga tahun setelah kejang yang terakhir.