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Pemicu 1B

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Adrian Pratama 405100018 Blok Neuropsikiatri
LO 1
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Definisi, etiologi, patofisiologi, klasifikasi
Definition
instantaneous loss of consciousness, alteration of perception
or impairment of psychic function, convulsive movements,
disturbance of sensation, or some combination thereof

Convulsion
an intense paroxysm of involuntary repetitive muscular contractions, is
inappropriate for a disorder that may consist only of an alteration of
sensation or consciousness

Seizure
preferable as a generic term, since it embraces a diversity of
paroxysmal events and also because it lends itself to qualification
Classification
Seizures
Etiology
Idiopathic (primary)
Symptomatic (secondary)
Site of origin
Clinical form
Generalized
Focal
Frequency
Isolated
Cyclic
Repetitive
Electrophysiologic correlates
Partial / focal seizures Generalized seizures
Simple Convulsive seizures
consciousness is undisturbed Tonic-clonic (grand mal)
Complex seizures
consciousness is altered Less common
Purely tonic, purely clonic,
generalized clonic-tonic-clonic
Non-convulsive seizures
brief lapse of consciousness
or absence (petit mal)
minor motor phenomena
(brief myoclonic, atonic, or
tonic seizures)
Etiology (seizures)
Pathophysiology
Mechanisms of Seizure Initiation and Propagation
Initiation phase
high-frequency bursts of action potentials
relatively long-lasting depolarization of the neuronal membrane due to
influx of extracellular calcium opening of voltage-dependent sodium
(Na+) channels influx of Na+ repetitive action potentials
Hypersynchronization
Followed by hyperpolarizing afterpotential mediated by G-aminobutyric
acid (GABA) receptors or potassium (K+) channels, but it is not functioning
well synchronized bursts from a sufficient number of neurons spike
discharge on the EEG

Seizure propagation phase


tonic-clonic, myoclonic, and atonic types mechanism still unknown
Mechanism of eliptogenesis
transformation of a normal neuronal network into one that is
chronically hyperexcitable
often a delay of months to years between an initial CNS injury
such as trauma, stroke, or infection and the first seizure
initiate a process that gradually lowers the seizure threshold in the
affected region until a spontaneous seizure occurs
in response to the loss of neurons (ex. Because of trauma)
reorganization or "sprouting" of surviving neurons in a way that affects
the excitability of the network
LO 2
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Menjelaskan epilepsi
Generalized seizure
Grand mal seizure (tonic-clonic seizure)
Petit mal seizure (Idiopathic Nonconvulsive Seizures)
Myoclonic seizure
Grand mal seizure
patient sometimes senses the approach of a seizure by one
of several subjective phenomena (a prodrome)
apathetic, depressed, irritable, or, the opposite (very rarely)
some hours
Myoclonic jerks of the trunk or limbs
turning of the head and eyes or whole body or intermittent
jerking of a limb (few seconds before consciousness is lost)
Abdominal pains or cramps
a sinking, rising, or gripping feeling in the epigastrium
pallor or redness of the face
Throbbing headache
constipation, or diarrhea
Initial motor signs
brief flexion of the trunk
an opening of the mouth and eyelid
upward deviation of the eyes
arms are elevated and abducted
elbows semiflexed
hands pronated
followed by a more protracted extension (tonic) phase 10-20s
first the back and neck, arms and legs
piercing cry as the whole musculature piercing cry as the whole musculature
closed vocal cords
respiratory muscles are caught up in tonic spasm breathing suspended
skin and mucous membranes may become cyanotic (few seconds)
Dilated pupil (unresponsive to light)
transition from the tonic to the clonic phase of convulsion
30s
mild generalized tremor (repetitive relaxation of the tonic
contraction)
violent flexor spasms that come in rhythmic salvos and agitate
the entire body
Face becomes violaceous and contorted by a series of grimaces
often the tongue is bitten
Autonomic signs are prominent
Pulse is rapid
blood pressure is elevated
pupils are dilated
Salivation & sweating are abudant
terminal phase of the seizure
all movements have ended
the patient lies still and limp
deep coma
Pupils equal / unequal begin to contract with light
Breathing may be quiet / stertorous

Several minutes later


Patient may open his eyes, confused and may be quite agitated, speak
and later not remember anything that he said drowsy and falls
asleep
When the patients fully recovered
no memory of any part of the spell
pulsatile headache
sore, bitten tongue and aching muscles
If violent enough
crush a vertebral body
Fracture
periorbital hemorrhage
subdural hematoma
EEG pattern
Repetitive spikes or spike-wave discharges lasting a few
seconds, followed by an approximately 10-s period of 10-Hz
spikes
spikes become mixed with slow waves EEG slowly assumes a
polyspike-and-wave pattern (clonic phase)
When all movement have ceased EEG tracing is nearly flat,
the brain waves resume their preseizure pattern
Other clinical state that simulate grand mal seizure
Clonic jerking of extended limbs that occurs with vasodepressor
syncope or a Stokes-Adams attack
basilar artery occlusion (Ropper) ischemia of the
corticospinal tracts in the pons
Limb-shaking TIA clonic movements of one limb or one side of
the body during an episode of cerebral ischemia
Hysterical (nonepileptogenic, psychogenic) seizure
Petit mal seizure
paucity of motor activity patients themselves are sometimes
not aware
resemble a moment of absentmindedness / daydreaming
The attack coming without warning
sudden interruption of consciousness

patient stares and briefly stops talking or ceases to respond


burst of fine clonic movements
eyelids, facial muscles, fingers or synchronous movements of both arms
Minor automatisms
lip-smacking, chewing, and fumbling movements of the fingers
Postural tone may be slightly decreased or increased
patients do not fall
After 2 to 10 s, occasionally longer patient re-
establishes full contact with the environment and resumes
preseizure activity
Typical absence seizures constitute the most characteristic
epilepsy of childhood

EEG pattern
generalized three-per-second spike and wave pattern
Other type of seizure that simulate petit mal
Atypical petit mal
long runs of slow spike-and-wave activity, usually with no apparent loss
of consciousness
External stimuli (asking the patient to answer a question) interrupt
the run of abnormal EEG activity

Juvenile myoclonic epilepsy


a common and relatively benign variety of myoclonic seizure occurs in
late childhood and adolescence
display symmetrical or asymmetrical myoclonic jerks without loss of
consciousness
Lennox-Gastaut syndrome
onset between 2 and 6 years of age
atonic, or astatic seizures (i.e., falling attacks), often succeeded by
various combinations of minor motor, tonic-clonic, and partial seizures
and by progressive intellectual impairment
distinctive, slow (1- to 2-Hz) spike-and-wave EEG pattern

West syndrome
infantile spasms
EEG picture (3-Hz hypsarrhythmia)
arrest in mental development
Myoclonic seizure
brusque, brief, muscular contraction
some myoclonic jerks are so small as to involve only one
muscle or part of a muscle
may occur intermittently and unpredictably or present as a
single jerk or a brief salvo

Signs
outbreak of several small, rhythmic myoclonic jerks may appear
with varying frequency as part of absence seizures
as isolated events in patients with generalized clonic-tonic-clonic
or tonic-clonic seizures
Quite benign & respond well to medication
Disseminated myoclonus (polymyoclonus)
Childhood
acute viral encephalitis
the myoclonus-opsoclonus-ataxia syndrome of Kinsbourne
Lithium or other drug toxicity
If lasting for few weeks subacute sclerosing panencephalitis
Chronic progressive polymyoclonus + dementia
group of juvenile lipidosis, Lafora type familial myoclonic epilepsy
progressive dementia, myoclonus, and episode of generalized seizure
certain mitochondrial disorders
other chronic familial degenerative diseases of undefined type (paramyoclonus
multiplex of Friedreich, dyssynergia cerebellaris myoclonica of Ramsay Hunt)
middle and late adult years, disseminated myoclonus + dementia
Creutzfeldt-Jakob disease; alzheimer disease (rare)
Juvenile Myoclonic Epilepsy
most common form of idiopathic generalized epilepsy in older
children and young adults
begins in adolescence (15 yo)
Clinical signs
generalized seizure often upon awakening
myoclonic jerks in the morning that involve the entire body
occasional myoclonic jerks of the arm and upper trunk that become
prominent with fatigue, during early stages of sleep, or after alcohol
ingestion
EEG pattern
bursts of 4- to 6-Hz irregular polyspike activity
Th/ valproic acid
Partial seizure
Frontal lobe partial seizure (focal & jacksonian)
Somatosensory, Visual, and Other Types of Sensory
Seizures
Complex Partial Seizures (Psychomotor Seizures, Temporal
Lobe Seizures)
Partial / focal seizure
Partial / focal seizure Generalized seizure
often the product of a clinical and EEG manifestations
demonstrable focal lesion or indicate bilateral and diffuse
EEG abnormality cerebral cortical involvement
in some part of the cerebral from the onset
cortex

Simple
Consciousness retained
arise from foci in the
sensorimotor cortex
Complex
focus in the temporal lobe on
one side or the other, but a
frontal localization is also well
known
Frontal Lobe Partial Seizures
(Focal Motor and Jacksonian Seizures)
Focal motor seizure
e/ discharging lesion of the opposite frontal lobe
Most common type originating in the supplementary motor
area
movement of the head and eyes to the side opposite the irritative focus
often associated with a tonic contraction of the trunk and limbs on that
side
entire seizure / generalized clonic movements, before or with loss of
unconsciousness
Clinical sign
forceful, sustained deviation of the head and eyes, and sometimes of
the entire body (versive / adversive) opposite side (contraversive);
same side (ipsiversive)
seizures of temporal lobe origin head turning ipsilaterally followed
by forceful contraversive head (and body) turning
Jacksonian motor seizure
tonic contraction of the fingers of one hand, the face on one
side, or the muscles of one foot clonic movements
Localized movement / spread movement (march from the part
first affected to other muscles on the same side of the body)
Classic jacksonian form
seizure spreads from the hand, up the arm, to the face, and down the leg;
if the first movement is in the foot, the seizure marches up the leg, down the
arm, and to the face (uncommon)
one-sided seizure activity turning of the head and eyes to
the convulsing side generalized seizure with loss of
consciousness
Consciousness is not lost if the sensorimotor symptoms remain confined
to one side
may be a transient paralysis of the affected limbs persists for minutes
or at times for hours after seizure (Todds paralysis)
If there are continued focal paralysis presence of a focal brain lesion
focus of excitation is usually in or near the rolandic (motor) cortex
onset of focal motor epilepsy in the hand
high medial frontal lesions (area 8 and supplementary motor complex)

Tonic elevation and extension of the contralateral arm (fencers posture)
and choreoathetotic and dystonic postures have been
complex, bizarre, and flailing movements of a contralateral limb (hysterical
seizure)
Seizure discharges arising from the cortical language areas brief
aphasic disturbance (ictal aphasia) and ejaculation of a word, or, more
frequently, a vocal arrest
Somatosensory, Visual, and
Other Types of Sensory Seizures
Somatosensory seizures
e/ a focus in or near the postrolandic convolution of the
opposite cerebral hemisphere
numbness, tingling, or a pins-and-needles feeling and
occasionally as a sensation of crawling, electricity, or movement
of the part
in the lips, fingers, or toes, and the spread to adjacent parts of the
body follows a pattern determined by sensory arrangements in the
postcentral convolution of the parietal lobe
Visual seizures
e/ Lesions in or near the striate cortex of the occipital lobe
elemental visual sensations of darkness or sparks and flashes of
light, which may be stationary or moving and colorless / colored
Seizure arising in one occipital lobe momentary blindness in
both fields
temporal half of a homonymous field defect
lesions on the lateral surface of the occipital lobe sensation
of twinkling or pulsating lights
focus in the posterior part of the temporal lobe near its junction
with occipital lobe visual hallucinations & auditory
hallucinations
Auditory hallucinations
Infrequent
e/ focus in one superior temporal convolution
buzzing or roaring in the ears
Human voice sometimes repeating unrecognizable words / the
sounds of music
Vertiginous sensations
e/ lesion in the superoposterior temporal region (junction
between parietal and temporal lobes)
sensation of vertigo
With a temporal focus vertigo is followed by an auditory
sensation
Giddiness, or light-headedness, is a frequent prelude of seizure
Olfactory hallucinations
e/ disease of the inferior and medial parts of the temporal
lobe
perceived odor is exteriorized (disagreeable or foul,
unidentifiable)
Gustatory hallucinations
e/ lesions of the insula and parietal operculum
salivation and a sensation of thirst may be associated
Complex Partial Seizures
(Psychomotor Seizures, Temporal Lobe Seizures)
Signs
the aura
focal seizure of simple type / hallucination (visual and auditory) /
perceptual illusion indicating temporal lobe origin
Objects or persons in the environment may shrink / enlarge in the
distance (micropsia/macropsia); perseverate as the head is moved
(palinopsia)
dyscognitive state (dj vu & jamais vu)
certain old memories or scenes may insert / abrupt interruption of
memory
Emotional experiences (sadness, loneliness, anger, happiness)

period of altered behavior and consciousness, the patient is


later found to be amnesic
Motor activity to seizure
automatisms such as lip-smacking, chewing or swallowing
movements, salivation, fumbling of the hands, shuffling of the
feet
walk around in a daze or act inappropriately
Certain complex acts that were initiated before consciousness is
lost (walking, chewing food, turning the pages of a book, or
driving)
patients are obviously out of contact with their surroundings /
partially responsive patients seizure originate at right temporal
lobe
Psychomotor triad by Lennox for patient with temporal lobe
seizure
motor changes
Automatic behavior
alterations in psychic function

Postictal behavior
global and nonfluent aphasia (left sided origin)
Prolonged disorientation for time and place (right sided origin)
Postictal nose wiping is carried out by the hand ipsilateral to the
seizure focus
Other manifestation
Amnesic seizure
Rarely, brief, recurrent attacks of transient amnesia (manifestations of
temporal lobe epilepsy)
Behavioural & psychiatric seizure
Post ictal state protracted paranoid-delusional or amnesic psychosis
lasting for days or weeks
Epileptic personality disorder
exhibit a number of abnormalities of behavior and personality during
the interictal period (slow and rigid in their thinking, verbose,
circumstantial and tedious in conversation, inclined to mysticism,
preoccupied with rather naive religious and philosophical ideas)
Special epileptic syndromes
Benign childhood epilepsy with centrotemporal spikes & epilepsy
with occipital spikes
5-9 years of age; autosomal dominant trait
nocturnal tonic-clonic seizure with focal onset; one side of the face
(centrotemporal spikes)
visual hallucinations, sensations of movements of the eyes, tinnitus, or
vertigo (occipital spikes)

West syndrome (infantil spasm)


first year of life
recurrent, single or brief episodes of gross flexion movements of the
trunk and limbs
Severe EEG abnormality (hypsarrhythmia continuous multifocal
spikes and slow waves of large amplitude)
left mentally impaired after the seizure appeared
Febrile seizure
Reflex epilepsy
seizures that evoked in certain epileptic individuals by a discrete
physiologic or psychologic stimulus (visual, auditory,
somatosensory, writing / reading, eating)
Epilepsia partialis continua
persistent rhythmic clonic movements of one muscle group (face,
arm, or leg) which are repeated at fairly regular intervals
every few seconds and continue for hours, days, weeks, or
months without spreading to other parts of the body
distal muscles of the leg and arm, especially the flexors of the hand
and finger affected more frequently
Hysterical seizure
most often a symptom of hysteria in female (Briquet disease)
completely asynchronous thrashing of the limbs and repeated
side-to-side movements of the head
Striking out at a person who is trying to restrain the patient
hand-biting, kicking, trembling, and quivering
pelvic thrusting and opisthotonic arching posture
screaming or talking during the ictus
Epilepsy
Definition
Condition of reccurent unprovoked seizures
an intermittent derangement of the nervous system due to an
excessive and disorderly discharge of cerebral nervous tissue on
muscles (Hughlings Jackson, 1870)
Sudden alteration of central nervous system (CNS) function
resulting from a paroxysmal high-frequency or synchronous low-
frequency, highvoltage electrical discharge
discharge arises from an assemblage of excitable neurons in any part
of the cerebral cortex / secondarily involved subcortical structures as
well
there need not be a visible lesion
Etiology
Role of hereditary
Clinical approach
Is it indeed a seizure?
Hows the clinical & EEG pattern & other characteristics?
Whats the underlying cause?

In the diagnosis of epilepsy history is the key


The examination in children & infant greater value
Finding of dysmorphic & cutaneus abnormalities highly characteristic
cerebral disease that give rise to epilepsy
Some lab studies (CBC, blood chemistries, liver & thyroid
function tests, EEG, imaging studies of brain: MRI, CT for
emergency & very young children)
LO 3
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Antiepileptic drugs
Surgical treatment
Surgical excision of epileptic foci in simple & complex partial
epilepsies that have not responded to intensive & prolonged
medical therapy
excision of cortical tissue outside of the temporal lobe
Vagal nerve stimulation
Found favor in case intractable partial & secondarily
generalizing seizures
A pacemaker-like device is implanted in the anterior chest wall &
stimulating electrode are connected to the vagus at left carotid
bifurcation
Ketogenic diet
Inducing ketosis by starvation 80-90% fat diet (initiated
during hospitaliztion)
LO 4
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Status epilepticus
reccurent generalized convulsions at a frequency that
precludes regaining of consciousness in the interval
between seizures (grand mal status)
Mortality rate 20-30%
Etiologies
Vary between age groups; adult viral paraneoplastic
encephalitis, old traumatic injury, epilepsy with idiopathic mental
retardation
Other clinical signs
Rising temperature, acidosis, hypotension, renal failure because
of myoglobinuria
Prolonged convulsive status (> 30 minutes) epileptic
encephalopathy
Treatment
LO 5
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Definition & classification
occur between the age of 6 and 60 mo
temperature of 38C or higher
not the result of central nervous system infection or any metabolic
imbalance
occur in the absence of a history of prior afebrile seizures

Simple febrile seizure


primary generalized; tonic-clonic
attack associated with fever
lasting for a maximum of 15 min
not recurrent within a 24-hour period
Complex febrile seizure
Focal; more prolonged (>15 min)
recurs within 24 hr
Febrile status epilepticus febrile seizure lasting > 30 minutes
Epidemiology
2-5% of neurologically healthy infants and children
experience at least 1, usually simple febrile seizure
Complex febrile seizure mortality (>2x)
recurrent simple febrile seizures do not damage the brain
Reccurency
30% of those experiencing a first episode
50% after 2 or more episodes
50% of infants <1 yr old at febrile seizure onset
Risk factor for recurrency
Genetic factors
Predicted polygenic factors
Identified single genes FEB 1, 2, 3, 4, 5, 6, and 7 genes on
chromosomes 8q13-q21, 19p13.3, 2q24, 5q14-q15, 6q22-24,
18p11.2, and 21q22
Function of FEB 2 a sodium channel gene (SCN1A)

GEFS+ (generalized epilepsy with febrile seizures plus)


autosomal dominant syndrome with a highly variable phenotype
Onset is usually in early childhood and remission is usually in mid-
childhood
Multiple febrile seizures and several types of afebrile generalized
seizures (generalized tonic-clonic, absence, myoclonic, atonic, or
myoclonic astatic seizures)
Dravet syndrome
Etiology
new mutation (2q24-31 and encodes for SCN1A); inherited in an
autosomal dominant manner
most severe of the phenotypic spectrum of febrile seizures plus
onset is in the 1st yr of life
febrile and afebrile unilateral clonic seizures recurring every 1
or 2 mo
Induced by fever more prolonged, more frequent, come in
clusters
2nd year of life myoclonus, atypical absences, and partial
seizures occur frequently and developmental delay usually
follows
Work up & evaluation

Febrile seizures often occur in


the context of otitis media,
roseola and human herpesvirus
6 (HHV6) infection, shigella, or
similar infections
Lumbar puncture
children <12 mo of age after their first febrile seizure; to rule out
meningitis
children >18 mo of age, a lumbar puncture is indicated in the
presence of clinical signs and symptoms of meningitis
EEG
delayed until or repeated after >2 wk have passed (because if < 2
wk nonspecific slowing)
performed for at least 30 min in wakefulness and in sleep according
to international guidelines
Blood studies
Neuroimaging
CT or MRI is not recommended in evaluating the child after a first
simple febrile seizure
Treatment
antiepileptic therapy, continuous or intermittent, is not recommended
for children with one or more simple febrile seizure
If the seizure lasts for >5 min acute treatment with diazepam,
lorazepam, or midazolam
recurrence of febrile seizure lasting >5 min rectal diazepam
Febrile status epilepticus Intravenous benzodiazepines,
phenobarbital, phenytoin, or valproate
< risk of febrile seizure
intermittent oral diazepam can be given during febrile illnesses (0.33
mg/kg every 8 hr during fever)
Intermittent oral nitrazepam, clobazam, and clonazepam (0.1
mg/kg/day)
intermittent diazepam prophylaxis (0.5 mg/kg administered as a rectal
suppository every 8 hr)
Phenobarbital (4-5 mg/kg/day in 1 or 2 divided doses)
Valproate (20-30 mg/kg/day in 2 or 3 divided doses)
Antipyretics < discomfort; not < risk for febrile seizure
Chronic antiepileptic therapy may be considered for
children with a high risk for later epilepsy
Iron deficiency has been shown to be associated with an
increased risk of febrile seizures
Reference
Adams & Victors principle of neurology; 8th & 9th edition
Harrisons principle of internal medicine; 17th edition
Nelsons pediatric; 19th edition