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Definisi

A seizure is a transient occurrence of signs and/or symptoms

resulting from abnormal excessive or synchronous neuronal
activity in the brain
 The International Classification of Epileptic
Seizures divides epileptic seizures into 2 large categories: In focal
(partial) seizures, the fi rst clinical and electroencephalographic
(EEG) changes suggest initial activation of a system of neurons
limited to part of one cerebral hemisphere

in generalized seizures, the fi rst clinical and EEG changes indicate

synchronous
involvement of all of both hemispheres ( Table 586-1).
 Febrile seizures are a special category.
Acute symptomatic seizures occur secondary to an acute
problem affecting brain excitability such as electrolyte imbalance
or meningitis. Most children with these types of seizures do well,
but sometimes such seizures signify major structural, inflammatory, or metabolic
disorders of the brain, such as meningitis,
encephalitis, acute stroke, or brain tumor; the prognosis depends
on the underlying disorder, including its reversibility or treatability and the
likelihood of developing epilepsy from it.
Unprovoked seizure is not an acute symptomatic seizure
Remote symptomatic seizure is thought to be secondary to a distant
brain injury such as an old stroke.
 The clinical diagnosis of epilepsy usually requires the occurrence of at least
1 unprovoked epileptic seizure with either a
second such seizure or enough EEG and clinical information to
convincingly demonstrate an enduring predisposition to develop
recurrences. For epidemiologic purposes epilepsy is considered to
be present when 2 unprovoked seizures occur in a time frame
of >24 hr.
 Seizure disorder is a general term
that is usually used to include any one of several disorders including
epilepsy, febrile seizures, and possibly single seizures and
seizures secondary to metabolic, infectious, or other etiologies
(e.g., hypocalcemia, meningitis)
 An epileptic syndrome is a disorder that manifests one or more
specific seizure types and has a specifi c age of onset and a specific
prognosis. Several types of epileptic syndromes can be distinguished (
Tables 586-2 to 586-4). This classifi cation has to be
distinguished from the classifi cation of epileptic seizures that
refers to single events rather than to clinical syndromes. In
general, seizure type is the primary determinant of the type of
medications the patient is likely to respond to, and the epilepsy
syndrome determines the type of prognosis one could expect
 An
epileptic encephalopathy is an epilepsy syndrome in which the
severe EEG abnormality is thought to result in cognitive and
other impairments in the patient.
 Idiopathic epilepsy is an epilepsy syndrome that is genetic or presumed
genetic and in which
there is no underlying disorder affecting development or other
neurologic function (e.g., petit mal epilepsy).
Symptomatic epilepsy is an epilepsy syndrome caused by an underlying
brain
disorder (e.g., epilepsy secondary to tuberous sclerosis).
A cryptogenic epilepsy (also termed presumed symptomatic epilepsy) is
an epilepsy syndrome in which there is a presumed underlying
brain disorder causing the epilepsy and affecting neurologic function, but
the underlying disorder is not known
 r acute evaluation of the 1st seizure, the physician should
search for potentially life-threatening causes of seizures such
as meningitis, systemic sepsis, unintentional and intentional
head trauma, and ingestion of drugs of abuse and other toxins.
 The 1st step in an evaluation is to determine whether the
seizure has a focal onset or is generalized.
Focal seizures may be characterized by motor or sensory symptoms and
include forceful
turning of the head and eyes to one side, unilateral clonic
movements beginning in the face or extremities, or a sensory
disturbance such as paresthesias or pain localized to a specific
area.
 ocalized lesion, but investigation of focal seizures during childhood may be
nondiagnostic. Focal seizures in a neonate may be
seen in perinatal stroke.

Clonic seizures consist of rhythmic muscle

contraction and relaxation; myoclonus is most accurately
described as shocklike contraction of a muscle
 he eyegrounds must be examined for the presence of papilledema, retinal
hemorrhages, chorioretinitis, coloboma, or
macular changes, as well as retinal phakoma. The finding of
unusual facial features or associated physical findings such as
hepatosplenomegaly point to an underlying metabolic or storage
disease as the cause of the neurologic disorder. Positive results of
a search for vitiliginous lesions of tuberous sclerosis using an
ultraviolet light source and examination for adenoma sebaceum,
shagreen patch, multiple caf-au-lait spots, a nevus flammeus,
and the presence of retinal phakoma could indicate a neurocutaneous
disorder as the cause of the seizure
 Localizing neurologic signs such as a subtle hemiparesis
with hyperreflexia, an equivocal Babinski sign, and a downward-drifting
extended arm with eyes closed might suggest
a contralateral hemispheric structural lesion, such as a slowgrowing
temporal lobe glioma, as the cause of the seizure disorder. Unilateral
growth arrest of the thumbnail, hand, or extremity
in a child with a focal seizure disorder suggests a chronic condition such as
a porencephalic cyst, arteriovenous malformation,
or cortical atrophy in the opposite hemisphere.
 imple febrile seizures do not have an increased risk of mortality
even though they are concerning to the parents. Complex febrile
seizures may have an approximately 2-fold long-term increase
in mortality, as compared to the general population over the
subsequent 2 yr, probably secondary to coexisting pathology.
There are no long-term adverse effects of having 1 simple febrile
seizures. Specifically, recurrent simple febrile seizures do not
damage the brain. Compared with age-matched controls, patients
with febrile seizures do not have any increase in incidence of
abnormalities of behavior, scholastic performance, neurocognitive
function, or attention.
 Although about 15% of children with epilepsy have had
febrile seizures, only 2-7% of children who experience
 Almost any type of epilepsy can be preceded by febrile seizures, and a few
epilepsy syndromes typically start with febrile
seizures. These are generalized epilepsy with febrile seizures plus
(GEFS+), severe myoclonic epilepsy of infancy (SMEI, also called
Dravet syndrome), and, in many patients, temporal lobe epilepsy
secondary to mesial temporal sclerosis.
GEFS+ is an autosomal dominant syndrome with a highly
variable phenotype. Onset is usually in early childhood and
remission is usually in mid-childhood. It is characterized by multiple febrile
seizures and several types of afebrile generalized
seizures, including generalized tonic-clonic, absence, myoclonic,
atonic, or myoclonic astatic seizures with variable degrees of
severity.
 Dravet syndrome is considered to be the most severe of the
phenotypic spectrum of febrile seizures plus. It constitutes a distinctive separate entity that
is one of the most severe forms of
epilepsy starting in infancy. Its onset is in the 1st yr of life, characterized by febrile and
afebrile unilateral clonic seizures recurring every 1 or 2 mo. These early seizures are typically
induced
by fever, but they differ from the usual febrile convulsions in that
they are more prolonged, are more frequent, and come in clusters.
Seizures subsequently start to occur with lower fevers and then
without fever. During the 2nd yr of life, myoclonus, atypical
absences, and partial seizures occur frequently and developmental delay usually follows.
This syndrome is usually caused by a
new mutation, although rarely it is inherited in an autosomal
dominant manner. The mutated gene is located on 2q24-31 and
encodes for SCN1A, the same gene mutated in GEFS+ spectrum.
However, in Dravet syndrome the mutations lead to loss of function and thus to a more
severe phenotype
 The majority of patients who had had prolonged febrile seizures and
encephalopathy after vaccination and who had been
presumed to have suffered from vaccine encephalopathy (seizures
and psychomotor regression occurring after vaccination and presumed to
be caused by it) have Dravet syndrome mutations,
indicating that their disease is due to the mutation and not secondary to
the vaccine. This has raised doubts about the very
existence of the entity termed vaccine encephalopathy.
Unprovoked Seizures

Acute evaluation of a fi rst seizure includes evaluation of vital

signs and respiratory and cardiac function and institution of
measures to normalize and stabilize them as needed. Signs of head
trauma, abuse, drug intoxication, poisoning, meningitis, sepsis,
focal abnormalities, increased intracranial pressure, herniation,
neurocutaneous stigmata, brain stem dysfunction, and/or focal
weakness should all be sought because they could suggest an
underlying etiology for the seizure.
 Motor seizures can be tonic (sustained contraction), clonic
(rhythmic contractions), myoclonic (rapid shocklike contractions, usually
<50 msec in duration, that may be isolated or
may repeat but usually are not rhythmic), atonic, or astatic.
Astatic seizures often follow myoclonic seizures and cause a
very momentary loss of tone with a sudden fall. Atonic seizures,
on the other hand, are usually longer and the loss of tone often
develops more slowly. Sometimes it is difficult to distinguish
among tonic, myoclonic, atonic, or astatic seizures based on the
history alone when the family reports only that the patient
falls ; in such cases, the seizure may be described as a drop
attack.
 only the head and neck; this seizure morphology is referred to
as a head drop. Tonic, clonic, myoclonic, and atonic seizures
can be focal (including one limb or one side only), focal with
secondary generalization, or primary generalized. Spasms (axial
spasms) consist of flexion or extension of truncal and extremity
musculature that is sustained for 1-2 sec, shorter than what is
seen in tonic seizures, which last >2 sec. Focal motor seizures
are usually clonic and/or myoclonic. These seizures sometimes
persist for days, months, or even longer. This phenomenon is
termed epilepsia partialis continua.
 Absence seizures are generalized seizures consisting of staring,
unresponsiveness, and eye fl utter lasting usually for few seconds.
Typical absences are associated with 3 Hz spike and slow wave
discharges and with petit mal epilepsy, which has a good prognosis.
Atypical absences are associated with 1-2 Hz spike and
slow wave discharges, with head atony and myoclonus during
the seizures and with Lennox-Gastaut syndrome, which has a
poor prognosis. Seizure type together with the other nonseizure
clinical manifestations helps determine the type of epilepsy syndrome with
which a particular patient is afflicted (Table 586-7;
Chapters 586.3 and 586.4 ).
 Head circumference can
indicate the presence of microcephaly or of macrocephaly. Eye
exam could show papilledema, retinal hemorrhages, chororetinitis,
colobomata (associated with brain malformations), a cherry
red spot, optic atrophy, macular changes (associated with genetic
neurodegenerative and storage diseases) or phakomas (associated
with tuberous sclerosis). Skin exam could show a trigeminal V-1
distribution capillary hemangioma (associated with Sturge-Weber
syndrome), hypopigmented lesions (sometimes associated with
tuberous sclerosis and detected more reliably by viewing the skin
under UV light), or other neurocutaneous manifestations such
as Shagreen patches and adenoma sebaceum (associated with
tuberous sclerosis), or whorl-like hypopigmented areas (hypomelanosis of
Ito, associated with hemimegalencephaly)
 Subtle asymmetries on the exam such as drift of one of the extended arms,
posturing of an arm on stress gait, slowness in rapid alternating
movements, small hand or thumb and thumb nail on one side,
or difficulty in hopping on one leg relative to the other can signify
a subtle hemiparesis associated with a lesion on the contralateral
side of the brain.