Epilepsy 1

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Dr.

Zuraini, sps

What Is the Difference Between


Epilepsy & Seizures?

A seizure is a brief, temporary


disturbance in the electrical activity
of the brain
Epilepsy is a disorder characterized
by recurring seizures (also known as
seizure disorder)

A seizure is a symptom of epilepsy


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A seizure is as a sudden, disorderly


discharge of cerebral neurons.
Seizures involve a
transient alteration in
brain function
involving motor,
sensory, autonomic,
or psychic clinical
manifestations

The occurrence of repeated unprovoked


seizures*
Unprovoked seizure: events occurring in
absence of a recognized etiological or risk
factor (idiopathic and cryptogenic) and
events occurring in patients with antecedent
stable (non-progressive) CNS insults (remote
symptomatic seizure).
A chronic disorder characterized by recurrent
(more than 2) unprovoked seizures.

*ILAE, Epilepsia1993;34:592-6

About 2.3 million Americans have


epilepsy
(0.5-1% of the population)
Roughly 181,000 new cases of seizures
and epilepsy occur each year
50% of people with epilepsy develop
seizures by the age of 25; however,
anyone can get epilepsy at any time
Now there are as many people with
epilepsy who are 60 or older as children
aged 10 or younger
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In about 70% of people with


epilepsy, the cause is not known
In 30%, most common causes
are:
- Head trauma
- Infection of brain tissue
- Brain tumor and stroke
- Heredity
- Prenatal disturbance of brain development
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1.
2.

3.

Is this a seizure?
Is this a provoked or unprovoked
seizure?
What is the probability of
recurrence of seizures (to establish
a diagnosis of epilepsy)?

Primary epilepsy
Secondary epilepsy
Prenatal and perinatal factors
2. Trauma and surgery
3. Metabolic cuases
4. Toxic causes
1.

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5. Infectious and inflammatory


causes
6. Cerebral vascular diesease
7. Intracranial tumors
8. Hypoxia
9. Degenerative disease

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Electrical discharges between neurons


are usually restricted, and produce the
normal rhythm recorded on the EEG
(electroencephalogram).
When a seizure occurs, large groups of
neurons are activated repetitively and
hypersynchronously, with dysfunction of
the inhibitory synaptic contact between
neurons. This produces the high-voltage
spike-and-wave activity on the EEG.
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The onset of the epileptic discharge


may include the whole cortex
(primary generalized), may be
confined to one area of the
cortex(partial), or may start focally
and then spread to involve the
whole cortex (secondary
generalization of a partial seizure).

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Psychogenic nonepileptic spell


(pseudoseizure)
Syncope
Migraine
Transient Ischemic attack
Sleep behaviors and disorders
Movement disorders

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Characteristics

Prolonged duration of event (10-30 min)


Preservation of consciousness despite
whole body jerking
Bizarre and asynchronous motor
movements
Pelvic thrusting movements
Not stereotypical

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Metabolic abnormalities

Alcohol withdrawal
Acute neurological insult

Hypo/Hyperglycemia
Hyponatremia
Hypocalcemia

Infection (meningitis, encephalitis)


Stroke (ischemic, hemorrhagic)
Head trauma

Illicit drug intoxication the lowers seizure


threshold (i.e. Theophylline, tricyclic
antidepressant)
High fever in children
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Seizure
Loss of consciousness at the onset?
yes

No

Generalized seizures

Partial seizures
Alteration of consciousness
Yes

No

???
Complex partial

Simple Partial

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Focal seizures

account
for 80% of adult epilepsies
Simple partial seizures
Complex partial seizures
Partial seizures secondarilly
generalised

Generalised seizures

Unclassified seizures
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I. Partial (focal, local) seizures

A. Simple partial seizures (consciousness


not impaired)

B. Complex partial seizures (with


impairment of consciousness)

C.partial seizures evolving to generalized


seizures

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II. Generalized seizures

A. Absence seizures
1. Absence seizures
2. Atypical absence seizures

B. Myoclonic seizures

C. Clonic seizures

D. Tonic seizures

E. Tonic-clonic seizures

F. Atonic seizures (astatic seizures)

III. Unclassified seizures

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Simple partial seizures


Motor, sensory, vegetative or psychic
symptomatology
Typically consciousness is preserved

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Complex partial seizures (= psychomotor seizures)


Initial subjective feeling (aura), loss of
consciousness, abnormal behavior (perioral
and hand automatisms)

Usually originates in TL

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Partial seizures evolving to tonic/clonic


convulsions secondary generalised
tonic/clonic seizures (sGTCS)

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Absences
Myoclonic seizures
Clonic seizures
Tonic seizures
Atonic seizures

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I.

Localization-related (focal, local, partial)


epilepsies and syndromes
A. Idiopathic (with age-related onset). At present,
two syndromes are established:
1. Benign childhood epilepsy with centro
temporal spikes
2.Childhood epilepsy with occipital
paroxysms
B. Symptomatic. This category comprises
syndromes of great individual variability.
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Temporal lobe epilepsy


Frontal lobe epilepsy
Parietal lobe epilepsy
Occipital lobe epilepsy
Absence epilepsy
Myoclonic epilepsy

C. Cryptogenic

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II. Generalized epilepsies and syndromes


A. Idiopathic (with age-related onset, in order of age
appearance)
1. Benign neonatal familial convulsions
2. Benign neonatal convulsions
3. Benign myoclonic epilepsy in infancy
4. Childhood absence epilepsy (pyknolepsy, petit
mal)
5. Juvenile absence epilepsy
6. Juvenile myoclonic epilepsy (impulsive petit
mal)
7. Epilepsy with grand mal seizures on awakening

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B. Idiopathic, symptomatic, or both (in order of


age of appearance)
1. Infantile Spasms
2. Lennox Gastaux
3. Epilepsy with myoclonic-astatic
seizures
4. Epilepsy with myoclonic absences

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C. Symptomatic
1. Nonspecific cause, early myoclonic
encephalopathy
2. Specific syndromes. Epileptic seizures
may
complicate many disease states.
Under this
heading are included those
diseases in
which seizures are a
presenting or
predominant feature.

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III. Epilepsies and syndromes undetermined as to


whether they are focal or generalized
A. With both generalized and focal seizures
1.Neonatal seizures
2. Severe myoclonic epilepsy in infancy
3. Epilepsy with continuous spikes and
waves during slow-wave sleep
4. Acquired epileptic aphasia (LandauKleffner syndrome)
B. Without unequivocal generalized or
focal features

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IV. Special syndromes


A. Situation-related seizures
1. Febrile convulsions
2.Seizures related to other identifiable
situations, such as stress, hormones,
drugs, alcohol, or sleep deprivation
B. Isolated, apparently unprovoked
epileptic events
C. Epilepsies characterized by the specific
modes of seizures precipitated
D. Chronic progressive epilepsia partialis
continua of childhood

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Epilepsy syndrome

Localization-Related Epilepsy

Generalized Epilepsy

Idiopathic

Idiopathic

Symptomatic

Symptomatic

Cryptogenic

Cryptogenic

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Idiopathic epilepsy
Localization-related

Generalized

BFEC (Benign Rolandic Epilepsy of


Childhood)

Benign neonatal convulsion

Benign Occipital Epilepsy of


childhood

Benign myoclonic epilepsy in


infancy

Autosomal Dominant Nocturnal


Frontal Lobe Epilepsy

CAE

Primary reading epilepsy

JAE
JME
Epilepsy with generalized tonicclonic seizures on awakening
Some reflex epilepsy
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Symptomatic epilepsy
Localization-related

Generalized

Temporal lobe

Early myoclonic encephalopathies

Frontal lobe

Early infantile epileptic with


suppression burst (Ohtahar
syndrome)

Parietal lobe

Cortical abnormalities dysplasias

Occipital lobe

Metabolic abnormalities

Rasmussen encephalitis

West syndrome

Most reflex epilepsies

Lennox-Gastaut syndrome

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Cryptogenic epilepsy
Localization-related
Any occurrence of partial
seizure without obvious
pathologic cause

Generalized
Epilepsy with myoclonicastatic seizures
Epilepsy with myoclonic
absence seizures

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Medial basal

Lateral temporal

Aura: epigastric discomfort, autonomic sign


eg. Pallor, flushing, fearful, olfactory and
gustatory
Auditory/visual disturbance/speech

Lasting > 1 min, amnesia, post-ictal


confusion with gradual recovery to
normal

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Brief duration
Complex partial with little or no postictal
confusion
Rapid secondary generalization
Prominent tonic or postural movement
Frequent complex gestural automatism at
onset
Frequent falling when discharges are
bilateral.
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Sensory at the onset


Tactile, tingling, electricity, crawling,
stiffness, cold, or pain, or
unpleasant dysaesthesias.
Post-ictal transient deficit eg.
Cortical sensory deficit

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Eye movement, head turning and/or


visual hallucinations
Hallucination:
Posteriorly:

unstructured light
Anteriorly: structured image or visual
distortions such as macropsia or
micropsia

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Stress, emotion
Sleep/sleep deprivation
Hyperventilation
Fever
Medications, metabolic disturbance
Reflex epilepsy
Photic stimuli: TV, flashing lights, visual
patterns
Startle, music, reading, eating

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age of onset 3-8 years


abrupt cessation of activity with change of facial
expression and blank gaze
duration short usually < 15 seconds
child returns to normal and no postictal period
automatisms sometimes
activated by hyperventilation
characteristic EEG 3 Hz spike & wave
treat with AEDs (Ethosuxsimide, Valproate,
Topamax, and Lamictal)
patients usually grow out of seizures by teen
years

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3 Hz Spike &
Wave

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Also called Janz syndrome


First described in 1867
Triad includes myoclonic jerks, absence,
& tonic clonic seizures
Normal development
Normal imaging

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A common epilepsy syndrome: 10-15% of


all epilepsies
Age of onset 12-18 years
F=M
Accounts for 25% of patients with
idiopathic generalized epilepsies.
Most have myoclonic jerks, 85% have
GTCs, and 15-38% have absence

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autonomic dominant
onset 3-13yrs with peak 6-8 years
usually nocturnal or during sleep
infrequent episodes that awake the child with
drooling, speech arrest, ipsilateral facial
twitching or twisted to one side that are only
minutes in duration
can sometimes generalize
development and exam are normal
characteristic EEG that shows Midtemporal
(T3,T4) and Central (C3,C4) spikes
Treatment usually not indicated if infrequent
but can treat with AEDs
usually outgrown by 14 years
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onset 15mos-15years, usually 4-8 years


initial seizure manifestations include visual
hallucinations (flashing lights), blindness,
amaurosis, micropsia, metamorphopsia,
loss of consciousness can occur
can have migraine and nausea afterward
different seizure types (GTC, CPS, unilateral
clonic) and occur mostly when transitioning from
wakefulness to sleep
EEG shows occipital spike & wave 1.5-2.5 Hz and
eye opening enhances and sleep inhibits

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specific type of seizure seen in infancy


and early childhood
onset is predominantly in the first year of
life, typically < 1 year
characteristic EEG called hypsarrhythmia
typical pattern is a sudden bending
forward and stiffening of the body, arms,
and legs.

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Spasms tend to begin soon after arousal from


sleep. Individual spasms typically last for 1 to 5
seconds and occur in clusters, ranging from 2 to
100 spasms at a time.
Infantile spasms usually stop by age 5, but are
often replaced by other seizure types.
West Syndrome is characterized by infantile
spasms, hypsarrhythmia, and mental
retardation.

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Etiology
Cerebral malformations 35%, Perinatal insult
15%, Metabolic 15%,
Tuberous Sclerosis 10%
Treatment usually starts with AEDs, steroids,
ACTH, Vigabatrin, B6, Surgery (if lesions)
Prognosis depends on etiology. Worse
prognosis with symptomatic as many, 50%,
go on to have other types of seizures
Many develop mental retardation or delayed
development.

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Childhood epileptic encephalopathy (LennoxGastaut syndrome [LGS]) is a devastating


pediatric epilepsy syndrome
constituting 1-4% of childhood epilepsies
triad characterized by multiple types of
seizures, mental retardation or regression, and
characteristic EEG
abnormal EEG with generalized slow spike-andwave discharges (1.5-2 Hz)

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most common seizure types are tonicaxial, atonic, and absence seizures, but
myoclonic, generalized tonic-clonic, and
partial seizures can be observed. Seizures
often are resistant to therapy.
mean age at epilepsy onset is 3-5 years
(range, 1 d to 14 y)
60% have underlying cause (TS, NF,
perinatal insult) and 20% have history of
Infantile Spasms
diagnosis by history, PE, and EEG
treatment is difficult
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Landau-Kleffner Syndrome
onset 2-12 years
acquired aphasia, verbal auditory agnosia,
decreased spontaneous speech
difficulty understanding speech and child
stops talking
several seizure types (GTC, Myoclonic,
Absence)
neuropsychological disturbances in >50%
but intelligence is not affected
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sometimes the child is diagnosed with Autism or


being deaf
EEG is normal during wakefulness but during
sleep there is spike & wave mostly in parietal
and temporal lobes, sometimes electrical status
of sleep
treatment with AEDs and steroids shows good
control
recovery of language is variable and if onset is
before 6 years there is better outcome
less than 50% live independent lives

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LandauKleffner
EEG
Shows
S&W

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EEG
MRI brain
1st Unexplained seizure does not
necessitate AED treatment except:
Recognized epileptic syndrome with high
probability of recurrence.
Focal brain lesion.

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Absence Epilepsy
Juvenile Myoclonic Epilepsy
Benign Rolandic Epilepsy
Infantile Spasms
Lennox Gastaux

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Aura: A partial seizure experience


before the onset of a generalized
seizure; often described as a funny
feeling
Prodroma: Early clinical manifestations
that may occur hours to a few days
before the onset of a seizure (malaise,
headache, or depression)
Tonic phase: A state of muscle
contraction with increased muscle tone
(associated with loss of consciousness)

Clonic phase: Alternating contraction and


relaxation of muscles
Postictal phase: Time period immediately after
the end of seizure activity

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