(006) SEIZURES & EPILEPSY

DR. J. VIADO | 03/16/2022

OUTLINE DEFINITIONS
I. INTRODUCTION A. SEIZURE
A. Seizures
• An occasional excessive and disordered discharge of
B. Epilepsy nerve tissue (so seizure comes only from neuron or brain
C. Seizure vs Epilepsy cells)
D. Classification of Epileptic Seizure • Manifestation of transient hypersynchronous abnormal
II. SEIZURE DISORDERS neuronal behavior
A. Focal Seizures • Transient occurrence of signs and/or symptoms resulting
B. Generalized Seizures from abnormal excessive or synchronous neuronal activity
C. Epilepsy Syndromes (in Pediatrics) in the brain
III. REFERENCES • Paroxysmal disorder (It causes altered neurological
IV. TEST YOURSELF function secondary to abnormal cortical electrical
discharges of your brain or neuron)
I. INTRODUCTION • Altered neurological function
• Abnormal cortical electrical discharge
Learning Objectives • Beginning and an end Involuntary. (You cannot make
seizure of your own)
• Define what we mean by Seizures and Epilepsy • Seizures are grouped in several ways according to their
• Know the pathophysiology of seizures presumed etiology it can be Idiopathic (primary),
• Understand the different types of seizures symptomatic (secondary), site of origin, their clinical form
• Understand the different Epilepsy syndromes (generalized or focal), frequency, closely spaced
• Understand the causes of seizures sequence of status epilepticus) or by special
• Know what laboratory examinations should be requested electrophysiologic correlates.
in patient with seizure
• Know the emergency and long-term management of
seizures
• Know the management of Status Epilepticus
• Other issues

HISTORY TAKING

Chief complaints: “convulsion” (lay man’s term),

“agkissiw” (means seizure in Ilocano), “patangken” (means
stiffness in Ilocano) Figure 1. Homunculus

In getting a history of a patient who presents with seizure or Depending on the seizure type you can localize, especially if its
convulsion the following should asked if this is a real seizure or focal you can localize what area of the brain is affected. If its
not. generalized. Homunculus- Little brain mapping both for sensory
and motor cortex.
Questions:
• What was the patient doing just prior to the event? (Sitting, PATHOPHYSIOLOGY
running, eating)
• What happened during the event? (How does the patient
look like? was there upward rolling of the eyeball? was
there extension and flexion of extremities? was there
drooling of the saliva or is the patient just having blank
stare or is the patient have episodes of jerks)
• What was the patient’s condition just after the event? (Is
the patient febrile? was the patient tired? was the patient
sleep deprived?)
• Is it a seizure?
• If so, what kind?
• What caused it?
• What should be done?

Figure 2. Seizure Generation

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This is important in choosing the right anti-seizure drug. Seizure
generation is brought about now in the cellular level and in the
synaptic level.
At the cellular level, the interplay of cations plays a big role in
seizure generation. Cations, they are positive the anions are the
the negative. Positive ions are sodium, potassium and calcium.
On the other hand, if there’s influx of sodium in the cell, there is
also increase influx of water which causes neuronal swelling
causing increase tissue excitability producing seizure.
In patient with generalized epilepsy, drugs that acts on sodium
channel (carbamazepine and phenytoin). In calcium channels
specifically the t-type calcium channel, if there’s influx of calcium
(calcium going inside the cell) it causes cell hyperexcitability and
deep gray matters are involve in thalamic and cortical neurons
(there’s a firing in thalamic and cortical neurons producing
seizure). The t-type calcium channel, this is the pathophysiology
of patients with absence epilepsy. It’s a generalized absence
epilepsy, drug of choice is ethoxamide which acts to specific t-
type calcium channel. In K channel, if you have decreased
intracellular potassium it causes influx of calcium causing cell
hyperexcitability producing the firing of thalamic and cortical
neurons roducing seizure.
At the synaptic level, this is the connection of one neuron to
another, there’s an interplay of neurotransmitter (there’s a lot of
neurotransmitter but what we want specifically acts on gaba) the
one that acts on gaba which is inhibitory and glutamate which is
excitatory. If there’s decrease inhibitory there will be increase
excitatory. There’s hyperexcitable state producing seizure. This
is the mechanism of action of benzodiazepine (diazepam and
midazolam) acts on in the gaba neurotransmitter. In glutamate
which is excitatory, an increase excitatory concentration,
increase glutamate, there’s increase concentration of
hyperexcitable neurotransmitter producing hyperexcitable state
producing seizure.
B. EPILEPSY
• Seizure is a symptom; epilepsy is a disease. (don’t
diagnose patient with seizure because seizure is a
manifestation of epilepsy). By contrast, epilepsies
manifesting as seizures that begin locally and may evolve
into generalized tonic-clonic seizures
• Disorder characterized by the occurrence of at least 2
unprovoked seizures (means the patient is apparently well,
no fever, no metabolic imbalance, no CNS infection. So, if
the patient has 2 unprovoked seizure you can diagnosed a
patient having epilepsy)
• Is a disorder of the brain characterized by an enduring
predisposition to generate seizures and by the
neurobiologic, cognitive, psychological, and social
consequences of this condition?
• Epileptic seizures are almost by definition associated with
some abnormality in the EEG provided that it is being
recorded at the time of the seizure.
Epilepsy by Fisher et al.
• This is the specific definition of epilepsy, it’s the clinical/
practical definition which was published by international
league against epilepsy official report in 2014.
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• Epilepsy is a disease of the brain defined by any of the
following conditions:
i At least two unprovoked (or reflex) seizures occurring
>24 h apart (Ex. A month prior to consult the patient
has episodes of generalized seizure while sleeping,
no consult was done, bo medications taken, and few
hrs to consult while the patient is sleeping again, the
patient does episode of generalized seizure. Now,
that unprovoked, you can diagnosed the patient as
having epilepsy, because diagnosis of epilepsy is
clinical and based on the symptom of the patient.)
ii One unprovoked (or reflex) seizure and a probability
of further seizures similar to the general recurrence
risk (at least 60%) after two unprovoked seizures,
occurring over the next 10 years (Ex. patient has 1
unprovoked seizure but the patient has disability, no
history of hypoxia, cns infection, traumatic brain injury
even though there’s one episode, the general
recurrence risk for those patient is at least 60% on
having the second seizure, then you can treat the
patient and label them as having epilepsy)
iii Diagnosis of an epilepsy syndrome (Ex. Juvenile
myoclonic epilepsy, absence epilepsy west syndrome
etc.)
• Epilepsy is considered to be resolved for individuals who
had an age-dependent epilepsy syndrome but are now
past the applicable age or those who have remained
seizure-free for the last 10 years, with no seizure
medicines for the last 5 years. (the chance of recurrence is
like the normal population of around 1-4% having epilepsy)
• View issue TOC, Volume 55, Issue 4, April 2014, Pages
475-482.
• This is the specific definition of epilepsy, it’s the clinical/
practical definition which was published by international
league against epilepsy official report in 2014.
• Epilepsy is a disease of the brain defined by any of the
following conditions:
iv At least two unprovoked (or reflex) seizures occurring
>24 h apart (Ex. A month prior to consult the patient
has episodes of generalized seizure while sleeping,
no consult was done, bo medications taken, and few
hrs to consult while the patient is sleeping again, the
patient does episode of generalized seizure. Now,
that unprovoked, you can diagnosed the patient as
having epilepsy, because diagnosis of epilepsy is
clinical and based on the symptom of the patient.)
v One unprovoked (or reflex) seizure and a probability
of further seizures similar to the general recurrence
risk (at least 60%) after two unprovoked seizures,
occurring over the next 10 years (Ex. patient has 1
unprovoked seizure but the patient has disability, no
history of hypoxia, cns infection, traumatic brain injury
even though there’s one episode, the general
recurrence risk for those patient is at least 60% on
having the second seizure, then you can treat the
patient and label them as having epilepsy)
vi Diagnosis of an epilepsy syndrome (Ex. Juvenile
myoclonic epilepsy, absence epilepsy west syndrome
etc.)
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• Epilepsy is considered to be resolved for individuals who
had an age-dependent epilepsy syndrome but are now
past the applicable age or those who have remained
seizure-free for the last 10 years, with no seizure
medicines for the last 5 years. (the chance of recurrence is
like the normal population of around 1-4% having epilepsy)
• View issue TOC, Volume 55, Issue 4, April 2014, Pages
475-482.
C. SEIZURE DISORDER
• We no longer use this one. This is a diagnosis of seizure
in general.
• 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)
D. EPILEPTIC SYNDROME
• A disorder consisting of a cluster of signs and symptoms
plus its typical EEG
• Seizure of patient plus other concomitant comorbid
condition like cerebral palsy, inability to walk, deterioration
of language plus specific EEG finding. In EEG findings,
check what type of background activity, and epileptiform
discharges present in the patient.
• Is a disorder that manifests one or more specific seizure
types and has a specific age of onset and a specific
prognosis (depending on the syndrome they have)
• WS (West Sydrome), LKS (Landau Kleffner Syndrome),
LGS (Lennox-Gastaut Syndrome), JME (Juvenile
Myoclonic Epilepsy), DS, OS (Ohtahara Syndrome), ETC.
• The EPILEPSIES represent A group of diseases for which
Recurrent Seizures represent their Principal Manifestation.
. disorder other than the epilepsy.
E. SEIZURE VERSUS EPILEPSY
• A seizure is the event
• Epilepsy is the disease associated with spontaneously
recurring seizures
Figure 3. Tonic and Clonic Phase
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Incidence
• Approximately 4-10% of children experience at least 1
seizure in the first 16 year of life
• The cumulative lifetime incidence of epilepsy is 3%, and
more than half of the cases start in
• childhood
• The annual prevalence is 0.5-1%
Other definitions (Nelson)
• Acute symptomatic seizure- occur secondary to an
acute problem affecting the brain excitability such as
electrolyte imbalance
• Unprovoked seizure- one that is not an acute
symptomatic seizure
• Remote Symptomatic seizure- one that is considered
to be secondary to distant brain injury, such as an old
stroke.
• Reflex seizure- usually precipitated by asensory
stimulus such as flashing lights
• Epileptic encephalopathy- is an epilepsy syndrome
in which there is a sever EEG abnormality which is
thought to result in cognitive and other impairment in
the patient.
• Idiopathic epilepsy- old term; is an epilepsy
syndrome that is genetic or presumed genetic and in
which there is no underlying disorder affecting
development or other neurologic function.
• Genetic epilepsy – replaced idiopathic epilepsy; is an
epilepsy syndrome that is the direct result of a knwown
or presumed genetic defect (s) in which genetic defect
is not a causative of a brain structural or metabolic or
• Symptomatic epilepsy- old term; refers to an epilepsy
syndrome caused by an underlying brain disorder that
may or may not be genetic
• Structural/metabolic epilepsy- caused by a distinct
structural or metabolic entity that increases the risk for
seiziures and causes the epilepsy.
• Cryptogenic epilepsy/ presumed symptomatic
epilepsy- an epilepsy syndrome in which there is a
presumed underlying brain disorder causing the
epilepsy and affecting neurological function, but the
underlying disorder is not known;
• Unknown epilepsy- underlying cause of epilepsy is as
yet unknown
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F. CLASSIFICATION OF EPILEPTIC SEIZURES (Old

classification)

Figure 5. Generalized Seizures

(top) MRA (bottom) functional MRA occurring in both cortex

Figure 4. Classification of Epileptic Seizures I. Focal seizures

• Abnormal flow of electrical discharge from a specific or
single focus
G. NEW CLASSIFICATION • Originate within networks limited to one hemisphere
• ILAE classification of the epilepsies: Position paper of the • Formerly termed partial in which a focal or localized onset
ILAE Commission for Classification and Terminology can be discerned clinically or by EEG.
(Scheffer et al., 2017) • Focal seizures are further classified according to their
• Operational classification of seizure types by the additional features such as a specific subjective
• International League Against Epilepsy: Position Paper of experience aura), motor, autonomic, and most
the ILAE Commission for Classification and Terminology importantly, whether awareness or consciousness is
(Fisher et al., 2017). disturbed; the latter was formerly called partial complex
seizure.
• May be discretely localized or more widely distributed
H. GENERALIZED SEIZURES (because some seizure can go generalized tonic clonic)
• Originate at some point within and rapidly engage
bilaterally distributed networks
• Can include cortical and subcortical structures but not
necessarily the entire cortex (it occurs simultaneously left
and right involving both the cerebral cortex)
• Generalized seizures are of two types-convulsive and
nonconvulsive.
• The common convulsive type is the tonic-clonic (grand
mal) seizure. Less common i s a purely tonic, purely clonic,
or clonic-tonic-clonic generalized seizure.
• The typical nonconvulsive generalized seizure is the brief
lapse of consciousness or absence (petit mal); included
also under this heading are minor motor phenomena such • Figure 6. Focal Seizures. Present here is a middle
as brief myoclonic, atonic, or tonic seizures. temporal that goes bilateral temporal area

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Figure 7. Classification of Seizure Type Basic Version

New classification of seizure, they just change the word partial

to focal plus the word onset.

Figure 8. Classification of Seizure Types Expanded Version

Terms NO LONGER in use

• Complex partial
• Simple partial
• Partial
• Psychic
• Dyscognitive
• Secondarily generalized tonic-clonic

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FOCAL ONSET subserving the left upper extremity. after the seizure, the
patient looks very tired. This is an example of focal onset
aware of tonic clonic.

B. FOCAL TO GENERALIZED TONIC CLONIC

It started in left arm focal in onset, apparently after few seconds
it involves both hemisphere that’s why the seizure became
generalized tonic clonic

C. FOCAL ONSET WITH IMPAIRED AWARENESS

• Appears to be in a dream like state.
• Unaware or unresponsive to questioning
• May perform unusual actions such as picking of clothing’s,
grimacing, contorting to one side, chewing
• Feel confused for several minutes
• No recollection of the event
• Cardinal point “Impairment of consciousness”
• Limbic lobe is affected
Figure 10. Focal Seizures with Altered Consciousness • Characterized by affective and perceptual disturbance,
motor manifestations, and automatisms
• Automatisms
o Oro-alimentary
A. FOCAL AWARE o Chewing, lip smacking (most common)
You can localize where is the lesion, example you cannot control o Mimicry
your left hand and the basic lesion will be in the motor cortex on o Anger, joy
the left side of the brain because cerebral lesions are o Gestural
contralateral and cerebellar lesions are ipsilateral. o Clapping, scratching
o Ambulatory
Focal onset has many classifications and it depend on the area o Walking, riding a bicycle
of the brain that is affected, some pateints will have tonic clonic o Verbal automatism
extension of unilateral extremity, you can localize it already.
some will represent ringing of the ear that’s why psychiatrist
need neurologist to clear the patient if this is really a psychiatric
problem or some form of seizure or the patient has epilepsy.

• They signify a focal onset in the temporal lobe as reflected

by an aura that may be a hallucination or perceptual illusion
• It is a complete loss of control of thought and action, there
is a period of altered behavior and consciousness, for which
the patient is later found to be amnesic.

Figure 9: Focal Seizures

Focal Aware Video

The patient can’t control his hand (involuntary movement)
because there’s firing of neurons to right motor cortex
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Motor
• Tonic – clonic
• Clonic – action (shaking)
• Tonic – rigid (arms straighten forward)
• Myoclonic – nagugulat/surprised
• Myoclonic tonic clonic
• Myoclonic atonic
• Atonic
• Epileptic spasms

Non-motor
• Typical
• Atypical
• Myoclonic
• Eyelid myoclonic – just like eye blinking – type of
generalized onset seizure
Figure 11. EEG – Focal Onset with Impaired Awareness

There’s a specific area or focality of epileptiform discharges over A. GO: TONIC-CLONIC SEIZURES
the bilateral form to central area (epileptiform discharges only in I. Tonic phase (Protracted extension)
some part of brain) • Breathing is suspended and after some seconds the
skin and lips may become cyanotic. The pupils are
dilated and unreactive to light.
GENERALIZED ONSET (GO) • This is the tonic phase of the seizure and lasts for 10
to 20 s
GENERALIZED ONSET
• Begin – Begin throughout both hemispheres, more or less
simultaneously
• Do not have – Do not have localized onset
• Reflect – Reflect generalized disturbance of cortical
function

Figure 13. Tonic Phase. EEG: Generalized discharges all over

the tracing.

II. Clonic phase

• At first, there is generalized tremor, then violent flexor
spasms that come in rhythmic salvos and agitate the
entire body. The face becomes violaceous and
contorted by a series of grimaces, pulse is rapid, blood
pressure is elevated, pupils are dilated, and salivation
and sweating are prominent.

Figure 12. Classification of Seizure Types Expanded Version

• May cry out or gasp, fall down, become rigid

• Muscle may jerk, breathing becomes shallow
• May lose bladder and bowel control
• May drool, bite the tongue or lips and may turn blue
• Post ictal – maybe confused, drowsy, sleep for a while or
have a headache
Figure 14.Clonic Phase
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III. Post-ictal phase
• All movements have ended and the patient is
motionless and limp in a deep coma. The pupils now
begin to contract to light. Breathing may be quiet or
stertorous. This state persists for several minute.
• You can see that the patient is confused and then suddenly
she's okay and the EEG went back to normal again. so that
is your typical absence epilepsy.
• "It's just blank stares just like student looking at the class or
you guys looking at my or listening to my video without
actually understanding it, so sometimes, most of the
students like they have absence epilepsy or generalized
absence epilepsy"
• Electrical discharges associated with absence seizures
have a more stereotyped pattern of 3-per-second spike-
and-wave complexes that characteristically appear abruptly
in all leads of the EEG simultaneously and disappear
almost as suddenly at the end of the seizure.

Figure 15. Postictal Stupor

Ictus – event where the patient is in active seizure

Post-ictal -there is no longer epileptiform of discharges so the
patient looks so tired and unresponsive during that time.

B. GO: MYOCLONIC
There is a sudden jerking of the muscles specifically the right
hand that's why the patient actually was not able to hold the
spoon properly.
Characterized by a brusque, brief, muscular contraction, some
myoclonic jerks may be so small as to involve only one muscle
or part of a muscle; others are so large as to displace a limb on
one or both sides of the body or the entire trunk musculature

o Juvenile Myclonic Epilepsy- most common form of

idiopathic generalized epilepsy in older children and young
adults.

C. GO: ATONIC SEIZURES

• Atonic from the word itself atonic - loss of tone so you
expect there is a sudden drop attack because the patient
lost tone
• That is the attack, you can see that there is a sudden loss
of tone that's why he has helmet on the possibility of having
his head hurt is very high
• So atonic seizure is a form of generalized onset epilepsy
where in the patient has sudden loss of tone and this is one
of the hardest one to control among all the seizure types,
so the boy has to wear a helmet because he is very prone
to head trauma because of the sudden attack
D. GO: ABSENCE (NON-MOTOR) SEIZURE
Figure 16. Absence Seizures
If a patient comes into the emergency room or in their clinic
having the chief complaint of seizure, you ask the guardian or
the one who witnessed the event, how does it look like and then
by so doing you actually check if this is real seizure or not and if
this is really seizure you classify them on the new guidelines of
the international against epilepsy to focal onset, generalized
onset or unclassified.

• Absence (non-motor) seizure - this is a generalized onset

• Absence (non-motor) seizure where in the manifestation
are just blank stares
• During the attack, you can see the pathology on the EEG of
the patient with absence epilepsy. The findings are 3 Hz
spike and wave. You can see that during the attack, and
you can see that it would disappear after the attack.

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EPILEPSY SYNDROMES A. NEONATAL EPILEPSY

• Seizure syndromes presenting in neonates

• The most common seizure type in neonates is focal.
• In this example, the newborn is having focal clonic activity
of the left arm with eye deviation to the left. The EEG
shows electrographic seizures with rhythmic spikes
coming from the right hemisphere. The seizure in the right
hemisphere is responsible for both the left arm jerking and
eye deviation

Figure 17. Neonatal Focal Seizures

• The most common seizure type in neonates can be focal,
can be eye blinking only, and sometimes it is just cyanosis
or myoclonic jerk that occurs most of the time. Sometimes
they just have swarming movements, pedaling like riding a
bicycle only the lower extremities, they have a lot of type
of seizures.
• Any form of movements where in the mother is not
comfortable and comes to your clinic for consult, you have
to get a good history probably this patient is having a
seizure.
• Neonatal Seizure incidence:
o 57.5 per 1,000 in infants with birthweight (1,500g) and
o 2.8 per 1,000 in infants weighing between 2,500 and
3,999g

Table 1. Selected Epilepsy Syndromes by Age of Onset Figure 18. Spike example

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1. OHTAHARA SYNDROME
• Aka. Early Infantile epileptic encephalopathy
• Manifests tonic seizures and is usually caused by brain
malformation or syntaxin binding protein 1 mutations.
• Age of onset – birth
• Seizure types – they have a lot of seizures not just
generalized tonic-clonic because the myelination is not yet
fully developed. So they can have tonic seizures, they can
have myoclonic seizures, they can have infantile spasms
especially as they grow old within the first 2-3 months and
the associated findings based on the history of your
patient. Brief tonic seizures, myoclonic seizures, infantile
spasms
• Associated EEG patterns – suppression burst pattern
that can have evolution into hypsarrhythmia
• Hypsarrhythmia – it is very disorganized tracing of your
EEG.
• Common etiologies – structural abnormalities (cortical
dysplasia), gene mutations: ARX, STXBP1, CDKL5,
SCN2A, SPTAN1
• Now during at this point in time we're actually talking about
gene mutation, we're actually talking about sodium
channels, potassium channels as part of the laboratory
examinations. They even have epilepsy panel in some
country
• Treatment – pyridoxine, zonisamide, ACTH, prednisolone,
ketogenic diet
• Vitamin B6 which is a cofactor for your brain conduction
• Worst comes to worst if all medications doesn't work
anymore which usually is the case for Ohtahara then you
can go into ketogenic diet
• Prognosis – poorly controlled seizures, severe
developmental delay, shortened life span
• Prognosis is very poor in controlling seizures. They
actually are the group of patients who are
pharmacoresistant or have intractable type of epilepsy.
Figure 19. Suppression Burst Pattern
Typical EEG of Ohtahara syndrome. There are areas of
silence/normal wave and then suddenly there are burst of
sharpen slow wave/poly spike
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2. PYRIDOXINE DEPENDENT EPILEPSY
• There's another common epilepsy syndrome among
neonates and this one actually has a treatment, we call it
Vit. B6 dependent epilepsy / pyridoxine dependent
epilepsy
• Age of onset – usually first day of life or usually within the
first week but can be delayed
• Seizure types – myoclonic, clonic, generalized tonic clonic
• Associated EEG patterns – burst suppression or
multifocal spike waves with diffuse slowing pri treatment
• They respond to B6 that is why they know that this is
pyridoxine dependent epilepsy
• Common Etiologies – heterozygous and homozygous
mutations in ALDH7A1
• Treatment – treatment with pyridoxine or pyridoxal5-
phosphate, poorly responsive to other antiepileptic
medications pyridoxine 100 mg OD (up to 600mg/d)
• You already give all your anti-epileptic drugs that are
available intravenously and yet they still have seizures, so
they do not respond to other antiepileptic medications.
They will respond definitely to your pyridoxine or pyridoxal-
5- phosphate.
• Prognosis – mild to severe intellectual impairment
• If you catch a patient with this type of syndrome or epilepsy
early and you did not have a patient who went to status
epilepticus then the prognosis, they can have mild
intellectual impairment. But if its already late and then you
expect severe intellectual impairment because of the
damage that's being caused by your seizure that is actually
prolonged or super prolonged sometimes
B. SEIZURES OF INFANCY & EARLY CHILDHOOD
Epilepsy syndromes of infancy and early childhood
1. WEST SYNDROME/INFANTILE SPASM
• Parang nagugulat sila, Moro reflex
• It is like they're doing the Salaam attack, myoclonic with
the flexion/extension of the upper extremities and bowing
of the head.
• Consist of triad
o Infantile spasm that usually occur in clusters
o Developmental regression
o Hypsarrythmia in EEG
• Age of onset – 4-9 months of age
• Seizure types – epileptic spasms (Salaam attack)
• Associated EEG patterns – hypsarrhythmia
• Hysparrythmia – a high-voltage, slow, chaotic
background with multifocal spikes
• Common etiologies – various etiologies: CNS infection,
genetic, secondary to hypoxia, etc.
• Treatment – ACTH, vigabatrin, prednisolone, ketogenic
diet, surgical resection (if focal etiology)
• Prognosis – developmental delay, many will have
seizures later in life, can evolve to Lennox Gastaut
Syndrome or tuberous sclerosis
• During infancy they have west syndrome and when they
increase in age like 10-11, they can have Lennox Gastaut
Syndrome. The syndrome can evolve
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West Syndrome Common Etiologies • Seizure types – tonic (mostly nocturnal), atonic,
• Tuberous Sclerosis (10-30%) myoclonic, atypical absence, generalized tonic clonic,
• Tuberous sclerosis is the most common it's actually a focal
neurocutaneous syndrome they have lesions in the skin • Associated EEG patterns – generalized 1-2 hz (happens
and lesions also in the brain. within one second) slow spike and wave, generalized
• Perinatal (15-25%) slowing, paroxysmal fast activity (recruiting rhythm) during
o Fetal infections sleep
o HIE (Hypoxic-ischemic encephalopathy) /perinatal • If you have a patient that presents with seizure definitely
brain injury request for an EEG to actually check if there's a specific
o Hypoglycemia syndrome for your patient
• Brain malformations • Common etiologies – variety of etiologies, proceeded by
• Metabolic abnormalities infantile spasms in 9-40% of cases
• Pyridoxine deficiency • Some, not all, Ohtahara syn developes West syn, then
• Chromosomal Abnormalities progress to LG Syndrome.
• Trisomy 21 • Treatment – felbamate, clobazam, rufinamide, topiramate,
• ARX zonisamide, ketogenic diet, valproate, levetiracetam, VNS,
• TSC1 corpus callosotomy, focal cortical resection (if there is a
• TSC2 focus)
• SCN2A • Prognosis – moderate to severe intellectual impairment,
• ETC. usually correlates with etiology and seizure control

Figure 20. Hypsarrhythmia Figure 21. Hypsarrhythmia

• It is disorganized. There are big, small, and like runs.

• The odd number are the left and the even number are the
right
• F=frontal, C=central, P=parietal, O=occipital
• Dark green line - 1 second from 1 line to another
• Usually 10 second tracing of EEG for monitoring
• We count the brain activity depending on the second or per
second and specific age group will have specific number of
brain activity which is normal for them.
• Non-definite background activity

2. LENNOX GASTAUT SYNDROME

• Consist of triad
o Developmental delay
o Multiple seizure types (atypical absences,
myoclonic astatic, and tonic)
o EEG finding Figure 22. Slow Spike and Wave
• Age of onset – 1-7 years of age

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C. CHILDHOOD EPILEPSY
SYNDROMES
1. BENIGN ROLANDIC EPILEPSY
• Aka. Benign childhood epilepsy with centrotemporal spikes
• Patients can outgrow this one
• Etiology: Unknown, although a family history of epilepsy
is common
• Age of onset: 4-10 years
• Seizures: Type: focal seizures, typically out of sleep
• Focal seizure is the usual seizure type, typically attack
during sleep.
• If it is not during sleep then it's not Benign Rolandic
Epilepsy
• Semiology: drooling, dysarthria, speech arrest, tingling or
clonic activity of unilateral face with spread to arm, may
progress to hemiclonic or generalized convulsive
• Typical history is they occur at night and early in the
morning
• Duration: self-limited, status epilepticus
uncommon
• Sometimes it only occurs once a year, twice a year that's
why some neurologists opted not to treat BRE because
probably they would just occur 1 every 2 years, 1 every 3
years to the time that they will already outgrow or actually
limit by itself.
• Frequency: typically low
• Interictal EEG: High amplitude centrotemporal spikes and
sharp waves with dramatic activation during sleep. Serial
EEGs may show shifting asymmetry of spike wave
discharges
• Sometimes called Benign Rolandic Epilepsy with
centrotemporal spikes (BRECS)
• Imaging: normal
• Treatment: pharmacoresponsive, may not require
preventative medications if seizures are infrequent.
• Clinical course: self-limited epilepsy with spontaneous
remission, usually by age 15-17 years. After 17 years it will
not occur anymore.
• Comorbidities: Normal cognition, although learning and
behavioral disorders can occur
Figure 23. Benign Rolandic Epilepsy – Centrotemporal Spikes
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2. CHILDHOOD ABSENCE EPILEPSY
• Etiology: presumed genetic
• Age of onset: 3-10 years, peak at 6-7years; onset before
age 3 years likely represents different epilepsy syndrome
• Common scenario here is like this, the mommy will come
to the clinic and telling you "doktora, the teacher of my
daughter complained that she always has episodes of
daydreaming in the class. She doesn't listen, she just
daydream, look outside, stop what she is doing and then
she'll be okay" feeling nila na nagloloko lang yung bata, it's
just an act out but when you get a history, it's probably a
patient with absence epilepsy and also they will have
deterioration in their school performance can be seen if
they deterioration or declining in the grades of this patient.
• Seizures: Type: generalized absence seizures, can be
provoked by hyperventilation in up to 90%; 3% will also
have generalized convulsive seizures
• When in doubt you do your hyperventilation test in your
clinic and you ask them to blow. "I ask my patient to blow
actually on a paper. I have a wheel in the clinic that when
is you blow it turns so it is like a play to them when I actually
want to elicit the absence attack. So, blow, blow, blow then
while talking to them in between and then suddenly they
will stop, and they will have the blank stare. • Very high
chances that you can pick up the seizure when you do your
hyperventilation test.
• Semiology: staring, behavioral arrest, unresponsiveness.
Infrequent associated automatisms, clonic jerks, loss of
postural tone.
• Duration: brief, approximately 10 seconds
• Frequency: high, hundreds per day if untreated
• Interictal EEG: generalized symmetric 3 Hz spike and
wave discharges with increased activation during
hyperventilation; a posterior delta rhythm that attenuates
with eye opening is seen in a minority of children
• Imaging: normal
• Treatment: pharmacoresponsive in 60-70%;
ethosuximide and valproic acid are most effective, valproic
acid has more reported side effects. Carbamazepine and
oxcarbazepine can precipitate absence status epilepticus.
• Drug of choice is ethosuximide then valproic acid. Next
choice are enzyme inducing antiepileptic drugs or anti-
seizure medications like your carbamazepine and
oxcarbazepine but sometimes they will precipitate the
patient to go into absence status epilepticus so you better
be careful in giving them.
• Clinical course: self-limited in 70%-90%. If seizures
continue in adolescence, 40% will have convulsive
seizures. Some may evolve to juvenile myoclonic epilepsy
(JME)
• Comorbidities: normal cognition, excellent cognitive
outcome, executive dysfunction is reported
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• Interictal EEG: generalized 4-6 Hz atypical spike and

Figure 24. EEG – Typical Absence Seizure 3Hz Spike and
Wave
This is a generalized onset. In one second there are 3 Hz spike
and waves
• Three types of absence seizures
o Typical absence seizure
o Atypical absence seizure- tone changes of
the head (head drop)
o Juvenile absence seizure –occur later age
polyspike and wave discharges, with photoparoxysmal
response that may precipitate myoclonic seizures in 30-
90%
• With photoparoxysmal response meaning when you do the
activation procedure of photic stimulation, they can have
myoclonic seizures during the EEG
• Imaging: normal
• Treatment: 80-90% are pharmacoresponsive. The
traditional treatment is valproic acid, but side effects may
limit its use, especially in women of reproductive age, and
other broad-spectrum AED’s are used. Carbamazepine,
Oxcarbazepine and Phenytoin can exacerbate seizures
• Do not use carbamazepine, oxcarbazepine, and phenytoin
in patients with juvenile myoclonic epilepsy
• Clinical course: spontaneous remission is rare
• Comorbidities: normal cognition, although executive
dysfunction is common

D. LATE CHILDHOOD ONSET EPILEPSY SYNDROMES

1. JUVENILE MYOCLONIC EPILEPSY

• Aka. Janz Syndrome
• Most common generalized epilepsy in young adults,
accounting for 5% of all epillepsies
• Common findings in teenagers
• Etiology: presumed genetic
• Age of onset: 12-18 years
• Seizures:
o The seizures are different but the primary Figure 25. EEG – Generalized Atypical Spike and Wave
seizure is the myoclonic
o Myoclonic: sudden jerk, lasting <1 second, no clear
associated loss of awareness, can happen multiple
times per day, especially in the morning, with strobe
lights or computer/mobile games like mobile legends
and also too much anime that changes in the lighting
can manifest or can trigger patient with juvenile
myoclonic epilepsy. So, if you have patients like this
better advise them to actually limit to 1 and a half
hours a day exposure on those type of an internet
gaming etc. Occur in all JME patients but may not be
recognized initially as seizures. A history of sudden
jerks, clumsiness, or jitteriness, especially in the
morning should be questioned.
o Generalized tonic clonic: often begin a repetitive
myoclonic seizure that culminate in a generalized
bilateral convulsion. Typically, self-limited and
infrequent, although nearly all JME patients will have
at least one. Often the first seizure recognized.
o Absence: staring, behavioral arrest, Figure 26. EEG – Myoclonic Seizure
unresponsiveness. May not be previously recognized
and a history of staring spells should be questioned.
Occur in approximately 20% of patients with JME.

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II. REFERENCES
Viado, J. (2022, March 16). Seizures and Epilepsy

Anschel, David J.Neurology Pretest Self-assessment and Review. 6th Edition, McGraw-Hill

Robert M. Kliegman. Nelson Textbook of Pediatrics 20th Ed. 2016, Elsevier, Inc.

III. TEST YOURSELF

1. A 9-year-old boy is brought to your clinic by his parents because he has begun to have episodes of eye fluttering lasting several
seconds. Sometimes he loses track of his thoughts in the middle of a sentence. There was one fall off a bicycle that may have been
related to one of these events. There are no other associated symptoms, and the episodes may occur up to 20 or more times per
day. The boy’s development and health have been normal up until this point. He did have two head injuries as a young child: the first
when he fell off a tricycle onto the ground, and the second when he fell off of a playset onto his head. Both episodes resulted in a
brief loss of consciousness and he did not think clearly for part of the day afterward, but had no medical intervention. Which of the
following tests is most likely to confirm this patient’s diagnosis?
a) Brain CT scan
b) Brain MRI
c) Electroencephalogram
d) Lumbar puncture
e) Nerve conduction study

2. A 19-year-old right-handed man who carries the diagnosis of epilepsy is seen in the urgent care clinic. He had been healthy until
about age 12, when he began to have episodes of eye fluttering lasting several seconds. Sometimes he would lose track of his
thoughts in the middle of a sentence. There was one fall off of a bicycle that may have been related to one of these events. He has
been treated with valproic acid. At one point he was off all medications, but the seizures returned. He is now at the end of his first
semester of college and came in today because he had a witnessed generalized tonic-clonic seizure this morning. He had had only
about 2 hours of sleep the night before because he was studying for a final exam. Which of the following is the most appropriate
thing to tell this patient?
a) “I know that you faked this seizure to avoid taking a test.”
b) “Lack of sleep may have contributed to triggering this seizure.”
c) “You can expect to have tonic-clonic seizures on a regular basis from now on.”
d) “Your seizures are getting worse and there is nothing we can do about it.”
e) “You should take the next semester off to recover and get extensive testing.”

3. A 56-year-old man with epilepsy is brought into the emergency room. He has been having continuous generalized tonic-clonic
seizures for the past 30 min. He is treated with 2 mg of intravenous lorazepam. Most physicians recommend using a high dose of
intravenous benzodiazepine as part of the management of status epilepticus because it has which of the following qualities?
a) Ability to suppress seizure activity for more than 24 h after one injection
b) Lack of respiratory depressant action
c) Rapid onset of action after intravenous administration
d) Lack of hypotensive effects
e) Lack of dependence on hepatic function for its metabolism and clearance

4. A 34-year-old woman is having her medications tapered in the epilepsy monitoring unit. She has a convulsive seizure that does not
stop after 5 min, even after she receives a lorazepam injection. A second intravenous drug is given. Infusing which of the following
antiepileptic drugs at more than 50 mg/min in an adult may evoke a cardiac arrhythmia?
a) Carbamazepine
b) Diazepam
c) Phenobarbital
d) Clonazepam
e) Phenytoin

5. A 44-year-old man presents with left arm shaking. Two days ago, the patient noticed left arm paresthesias along the lateral aspect
of his left arm and left fourth and fifth fingers while he was reading. He thinks he may have been leaning on his left arm at the time;
the symptoms resolved after 30 seconds. This morning, he noted the same feelings, lasting a few seconds, but then his fourth and
fifth fingers started shaking rhythmically, and the shaking then spread to all of his fingers, his hand, and then his arm up to his elbow.

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This episode lasted a total of 30 seconds. He denies any strange smells or tastes, visual changes, or weakness. Afterward, his
fingers felt locked in position for a few seconds. Then he felt as if he did not have control of his hand and had difficulty donning his
socks. He and his wife decided to drive to emergency room, and in the car he had trouble putting his seat belt latch into its socket.
Examination and routine labs are normal. Which of the following is the most appropriate next action?
a) Discharge the patient to follow up in clinic in 2 weeks
b) Obtain a brain MRI
c) Obtain an electroencephalogram
d) Obtain an orthopedic consult
e) Order electromyography and nerve conduction studies

6. A 31-year-old right-handed woman has a history of alcohol abuse requiring detox. Currently, she says she is drinking about nine
beers 3 days per week. She drank five glasses of wine and three beers 5 days ago. Last night, she had 10 beers. This morning, she
awoke feeling well. She was speaking with her fiancé, went to the bathroom, and got back into bed. She had no headache, fever,
chills, nausea, vomiting, or pain. Suddenly her body became stiff with arms flexed for a few seconds, followed by rhythmic jerking of
both arms. Her legs were shaking, but less so. Her eyes were open, and she was foaming at the mouth. After 1 min, this stopped,
and she initially did not recognize her fiancé or his sister. She slowly returned to a normal level of consciousness over a 10-minute
period. She remembers events just prior to the episode, and she remembers being in the car on the way to the hospital. Her only
medication is a multivitamin. She denies illicit drugs. Her examination is entirely normal. Routine labs and a brain MRI are normal.
Which of the following is the most likely underlying cause of her condition?
a) Autoimmune
b) Genetic
c) Infectious
d) Neoplastic
e) Toxic/metabolic

7. A 4-year-old boy has the onset of episodes of loss of body tone, with associated falls, as well as generalized tonic-clonic seizures.
His cognitive function has been deteriorating. EEG shows 1.5- to 2-Hz spike-and-wave discharges. Which of the following is the most
likely diagnosis?
a) Landau-Kleffner syndrome
b) Lennox-Gastaut syndrome
c) Juvenile myoclonic epilepsy
d) Mitochondrial encephalomyopathy
e) Febrile seizures

8. A 27-year-old man begins to experience infrequent episodes of nausea, warmth rising through his body, and an unusual odor like
rotting fish. His girlfriend notices that afterward he may develop twitching of the left side of his face and an inability to speak for
several minutes. Afterward the man appears dazed and cannot remember what has occurred. He has otherwise been well. Magnetic
resonance imaging (MRI) of his brain is most likely to show a lesion in which of the following areas?
a) Left occipital lobe
b) Right frontal lobe
c) Cribriform plate
d) Uncus
e) Left parietal lobe

9. An 18-year-old girl riding on the back of her boyfriend’s motorcycle without a helmet is brought in with a left frontal skull fracture and
cortical contusion. GCS is 10. She is admitted to the intensive care unit. She has had no seizures. Which of the following is true
regarding anticonvulsant therapy in this case?
a) It is contraindicated due to risk of rash
b) It is best achieved using phenobarbital
c) It is likely to cause increased cerebral edema
d) It is indicated to reduce the incidence of late posttraumatic epilepsy
e) It is indicated to reduce the incidence of early posttraumatic seizures

10. A patient with intractable complex partial seizures due to cortical dysplasia undergoes left temporal lobectomy. He is most likely to
develop which of the following problems after surgery?
a) Right superior quadrantanopsia
b) Right inferior quadrantanopsia
c) Right homonymous hemianopsia
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d) Right hand weakness

e) Aphasia
ANSWER KEY

1. The answer is c. This is a common presentation for primary generalized epilepsy of childhood. An electroencephalogram showing
the classic 3-Hz spike-and-wave pattern would confirm this diagnosis. Brain MRI and CT are useful for evaluating brain anatomy.
Anatomic problems can cause seizures, but these tests will not provide any information about brain electrical activity. Lumbar
puncture is useful for measuring cerebrospinal fluid pressure and looking for central nervous system inflammation or infection. Central
nervous system inflammation or infection may cause seizures. Nerve conduction study is useful to evaluate peripheral nerve injuries
such as nerve entrapment.
2. The answer is b. Lack of sleep is a common seizure trigger. There is no reason to believe that the patient faked the seizure. It is
impossible to predict his future seizure course based on this one event; having one seizure does not necessarily mean that his
seizures are getting worse, and even if they are, many treatments are available. There is no reason for the patient to take a prolonged
leave of absence from school because of one seizure. This may even have detrimental psychological consequences
3. The answer is c. Until recently, the most popular benzodiazepine for use in status epilepticus was diazepam (Valium), which has a
rapid onset of action but is cleared relatively quickly. Because of this property, patients needed additional medications, such as
phenytoin, to protect them from recurrent seizure activity as early as 20 min after diazepam injection. A longer-acting benzodiazepine,
lorazepam (Ativan), has the advantage of acting rapidly like diazepam but being cleared more slowly from the brain.
4. The answer is e. Rapid infusion of phenytoin may produce a cardiac arrhythmia or hypotension. Phenytoin should not be administered
at rates greater than 50 mg/min in adults or 1 mg/(kgmin) in children to reduce the chances of this reaction occurring. Thus, it usually
requires approximately 20 min to administer a 1000- to 1500-mg standard loading dose of phenytoin in an emergent setting such as
status epilepticus. Fosphenytoin, a water-soluble prodrug of phenytoin, has the advantage of causing fewer infusion site reactions.
It can be given at doses of up to 150 mg/min in an adult, with risks of cardiac dysrhythmia similar to those of phenytoin. Another
advantage of fosphenytoin is that it can be administered intramuscularly when intravenous access is problematic. Carbamazepine is
not administered intravenously at all. Rapid infusion of phenobarbital may produce hypotension or respiratory arrest, but is much
less likely to depress cardiac activity. Diazepam and clonazepam are safer than phenobarbital, but rapid infusion of excessively high
doses may depress blood pressure and other autonomic functions.
5. The answer is b. This history is typical of a simple partial seizure. A focal brain lesion must be ruled out. It would be wrong to
discharge the patient to follow up in clinic in 2 weeks without at least a CT scan and preferably an MRI. Although he probably had a
seizure, obtaining an electroencephalogram at this point will not be as helpful as an MRI. This is unlikely to be a peripheral nerve
problem, and therefore an orthopedic consult or electromyography and nerve conduction studies are not indicated.
6. The answer is e. This is a typical example of alcohol withdrawal seizure. The greatest risk for alcohol withdrawal seizures occurs
within the first day after drinking cessation, in contrast to delirium tremens, which usually occurs within 2 to 4 days of drinking
cessation. There is no evidence of an autoimmune process in this patient. Rasmussen encephalitis is an example of a seizure
disorder thought to be of autoimmune etiology. There are many examples of genetically transmitted epilepsies, which usually present
during childhood. Infections such as meningitis, brain abscess, or encephalitis can cause seizures. Signs of these include meningeal
signs, fever, and MRI findings. If this patient had a brain tumor, you might expect a history of headache due to increased intracranial
pressure. Additionally, the exam and MRI would likely be abnormal.
7. The answer is b. Lennox-Gastaut syndrome is characterized by mental dysfunction, multiple seizure types and 1- to 2-Hz generalized
spike-wave discharges on EEG. It is often difficult to control the seizures that develop in children with this syndrome. Many affected
children have a history of infantile spasms (West syndrome). Infants and children with infantile spasms exhibit paroxysmal flexions
of the body, waist, or neck and usually have a profoundly disorganized EEG pattern called hypsarrhythmia.
8. The answer is d. Many patients with complex partial seizures have a preseizure phenomenon (the aura) that alerts them to an
impending seizure. This patient’s aura includes an olfactory hallucination, which is usually associated with lesions of the mesial
temporal lobe, particularly the uncus or parahippocampal gyrus. Diseases that can affect that region include tumors, trauma, and
mesial temporal sclerosis.
9. The answer is e. There is evidence that prophylactic phenytoin reduces the incidence of seizures after head injury. Because early
posttraumatic seizures may lead to increased morbidity and prolonged hospital stays, it is reasonable in some situations to treat
patients prophylactically. There is no evidence that prophylactic treatment reduces the long-term risk of developing posttraumatic
epilepsy
10. The answer is a. The most common complication of temporal lobectomy is a visual field defect due to interruption of fibers from the
optic tracts passing over the temporal horn of the lateral ventricles. Superior quadrantanopsia is more common than hemianopsia.
Some deficits may improve if the injury does not completely damage the nerves. Language deficits, particularly dysnomia, occur less
frequently. Hemiparesis is uncommon.

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