Review of Clinical Electroencephalography

Chapter 9
Electroencephalography in common
epileptic syndromes
Saturday, March 13rd 2021

Presentan : Desby Juananda

Moderator : dr. Desin Pambudi Sejahtera, M.Sc., Sp.S(K)
 Epilepsy Syndrome
• Childhood and Juvenile Absence Epilepsy • West’s Syndrome
• Benign Childhood Epilepsy • Lennox-Gastaut syndrome
• Benign Epilepsy of Childhood with • The Syndrome of Continuous Spike and
Centrotemporal Spikes (BECTS) or Benign Wave Discharges During Slow Sleep
Rolandic Epilepsy (BRE)
• Benign Partial Epilepsy of Childhood with (CSWS) [electrical status epilepticus
Occipital Paroxysms (BPEOP) during slow sleep]
• Cryptogenic or Symptomatic Focal • The Syndrome of Acquired Epileptic
Epilepsies Aphasia (Landau-Kleffner syndrome)
• Medial temporal lobe epilepsy
• Lateral temporal lobe epilepsy
• Frontal Lobe Epilepsy
• Juvenile myoclonic epilepsy (JME)
• Epilepsy with Generalized Tonic-Clonic
Seizures on Awakening

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 Epilepsy Syndrome
The Classification of epilepsy syndromes is based on :
• Localization-related & generalized epilepsies
• Localization-related epilepsies : focal IEDs, the location à corresponds
approximately to the area of seizure onset
• Generalized epilepsies : generalized IEDs
• Idiopathic & symptomatic epilepsies
• Idiopathic epilepsies : normal or near-normal background activity
• Symptomatic epilepsies :
• Focal, multi focal, or diffuse abnormalities of background activity
• Persistent focal voltage attenuation, especially of faster frequencies, or
polymorphic delta activity
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 Childhood & Juvenile Absence Epilepsy
• Childhood absence epilepsy (CAE) :
• The ages : 3 to 5 years (remits
between 10 to 12)
• Absence seizures > GTCS
(remission by late adolescence)
• Juvenile absence epilepsy (JAE) :
• The ages : 10 to 12 years (or later)
• Absence seizures < GTCS
• EEG features of CAE and JAE are
also broadly similar.

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Childhood & Juvenile Absence Epilepsy
EEG Findings :
• A normal background for age
• 3-Hz generalized spike and wave discharges
(SWC)
• Frequency : 4 Hz (the onset) and 2.5 Hz (the end)
• Duration : 3-25 seconds
• The repetition rate is faster at onset in JAE than
in CAE
• Polyspikes are seen more often in JAE
• Typically, an initial positive component is
followed by one or more negative components
and then a negative slow wave
• Frontally dominant
• Discharges are not truly bisynchronous
• Eye opening does not alter the discharges
11/03/21 Department of Neurology FKKMK UGM - Dr. Sardjito Hospital
• Occipital intermittent rhythmic delta
discharges (OIRDA)
• Favorable prognostic indicator (CAE > JAE)
• High-voltage OIRDA (15%-38%)
• Strongly age related : 6 and 10 y.o (70%), > 15
years (rare)
• Hyperventilation à ↑ 3-Hz spike-wave
bursts in CAE (50%-80%), OIRDA +
• Photic stimulation à ↑ 3-Hz spike-wave
bursts (18%)
• Absence should be differentiated from
“atypical absence seizures” (e.g LGS) à EEG
shows a less abrupt onset and offset,
background is slow, and duration of
discharges is shorter.
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 Faster at onset OIRDA

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 OIRDA POLYSPIKE

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Benign Childhood Epilepsy
Benign Epilepsy of Childhood with
Centrotemporal Spikes (BECTS) or Benign
Rolandic Epilepsy (BRE)
• Idiopathic, localization-related epilepsy syndrome
• The most common forms of childhood epilepsy
(16%-24%)
• Characterized by two defining features :
• Clinical : stereotyped partial seizures à unilateral
paresthesias of the tongue, lips, inner cheeks, and
gums, accompanied by unilateral tonic or clonic activity
of the facial and pharyngeal/laryngeal muscles, speech
arrest (anarthria), and excessive salivation
• Electrographic : interictal EEG à central-mid temporal
spikes and otherwise normal background activity
• Onset of seizures : 4-10 y.o
• Seizures occur exclusively during sleep (80%)
11/03/21 Department of Neurology FKKMK UGM - Dr. Sardjito Hospital
EEG Findings :
• Interictal EEG : central (C3-C4) mid temporal (T3-
T4) epileptiform discharges à stereotyped,
diphasic or triphasic sharp waves (100-300 µV),
usually followed by an after going slow wave
• Unilateral discharges are more common
• Hyperventilation/photic stimulation/eye opening
does not block the discharges
• The EEG discharge disappears at late adolescence
(at around age 15 years)
Note :
There is an increased incidence of generalized spike-
and-wave discharges (typical absence seizure) in BRE
à it may be difficult to identify the specific clinical
syndrome “benign rolandic epilepsy or childhood
absence epilepsy?”
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 Generalized spike-and-
wave discharges (3 Hz
spike and wave)

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 Seizures occur exclusively during sleep

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Benign Childhood Epilepsy
Benign Partial Epilepsy of Childhood with
Occipital Paroxysms (BPEOP)
• BPEOP is analogous to BRE; subtypes à
early-onset variant and late-onset
variant
• Neurologically, CT & MRI à normal; boys
and girls are equally affected
• Late-onset variant (15 months -17 y.o;
peak 7- 9 y.o)
• Ictal : begin with visual symptoms
(amaurosis, phosphenes, illusions, or
hallucinations), typically brief, lasting only
seconds, without alteration in
consciousness
• Post ictal : severe diffuse headache, nausea
and vomiting
• Early-onset variant (3-5 y.o)
• Ictal : lack the characteristic visual
phenomena, stereotyped seizures : lateral
gaze deviation and ictal vomiting, with a
varying degree of alteration in
consciousness, exclusively nocturnal,
typically prolonged (5-10 min or longer) à
status epilepticus
• Prognosis is universally excellent (30% of
patients experience only a single seizure,
seizures occur infrequently)
• Duration of the disease : 1 to 2 years, and
nearly become seizure free by age 12
• It is two to three times less common that
BECTS; the early-onset variant accounts
for most cases

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Benign Childhood Epilepsy
EEG Findings :
• EEG findings are indistinguishable in
the two BPEOP variants
• Interictal EEG : normal background
activity, occipital epileptiform
discharges à diphasic spike or sharp
wave with a high-voltage (200-300
μV) surface-negative peak followed by
a low-voltage surface-positive peak
and an after going surface negative
slow wave
13/03/21 Department of Neurology FKKMK UGM - Dr. Sardjito Hospital
• Typically blocked with eye opening;
hyperventilation/photic stimulation
usually has no effect on epileptiform
activity
• Other epileptiform abnormalities à
generalized spike-wave or central mid
temporal discharges and frontal
discharges
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 Discharges blocked with eyes opening

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Cryptogenic or Symptomatic Focal
Epilepsies
Medial Temporal Lobe Epilepsy
• The most common of focal epilepsies
• It typically associates a history of complicated
febrile seizures followed by a period of
latency before onset of epilepsy in the peri-
pubertal period, and hippocampal sclerosis
• Seizures may be simple, simple followed by
complex, or complex from onset; mostly
occurs during sleep
• Duration : 30 seconds to 2 minutes
• Seizures occurs mainly during waking and
particularly at awakening
• Typically drug-resistant but may respond to
surgical treatment
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• Ictal : impairment of consciousness (preceded
by an aura), epigastric feeling (nausea), déjà-
vu or jamis-vu, staring, oral automatisms
(chewing, lip smacking), vocal and verbal
automatisms, simple gestural automatisms,
autonomic signs (pallor, flushing, tachycardia,
breathing irregularities, mydriasis, bleching)
• Post ictal : temporal and spatial disorientation
and language deficit (if the dominant side is
involved)
• Etiology : hippocampal sclerosis, but other
causes may be found: dysplasias, tumors
(ganglioglioma, low grade tumors), AVM, and
a history of meningitis or encephalitis; some
cases are cryptogenic
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Cryptogenic or Symptomatic Focal
Epilepsies
EEG Findings : • Ictal EEG : precise organization with
• Interictal EEG : spikes and spike- flattening over the anterior and
waves over the anterior and middle middle temporal region followed by
temporal leads, theta activity and rhythmic slow spikes at 6-7 Hz à the
even slower waves spikes become progressively less
rhythmical and fade into slow wave
• The frequency and morphology of over the same territory
the abnormalities is determined by
the etiology; hippocampal sclerosis
à spikes and spike-waves often occur
in pairs or triplets, but they are not
necessarily frequent

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Cryptogenic or Symptomatic Focal
Epilepsies
Lateral Temporal Lobe Epilepsy
• Seizure originating in the external
temporal cortex may or may not
propagate to the limbic structures
• The seizures are simple or complex and
rarely generalized
• Ictal : simple auditory hallucinations,
complex auditory hallucinations or
illusions, auditory dreamy state and
language deficits (if the dominant
temporal lobe is involved); complex
seizures without aura à behavioral
arrest and non-oral automatisms
• Seizures are seen during waking but also
during sleep
11/03/21 Department of Neurology FKKMK UGM - Dr. Sardjito Hospital
EEG Findings :
• Interictal EEG : spikes, spike-waves, slow
spikes, polyspikes, polyspike-waves, fast
rhythms and theta activities over middle
and posterior temporal leads, often
frequent and high-voltage
• Ictal EEG : a flattening followed by fast
recruiting rhythms over the middle and
posterior temporal region, with rapid
diffusion followed by rhythmic slow
waves that may be faster, slower or
similar (6-7Hz) to those found in mesial
temporal seizures
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Cryptogenic or Symptomatic Focal
Epilepsies
Frontal Lobe Epilepsy
• It is the second most common
localization-related epilepsy
• Seizure symptoms are heterogeneous
à large size of the frontal lobe with
many functional and anatomical
divisions
• ILAE commission classified the frontal
lobe epilepsies by anatomical areas
that produce relatively characteristic
seizure symptoms : supplementary
motor, cingulate, anterior
frontopolar, orbitofrontal,
dorsolateral, opercular, and motor
cortex
11/03/21 Department of Neurology FKKMK UGM - Dr. Sardjito Hospital
• Frontal lobe seizures features :
• Early and prominent motor
manifestations, including clonic activity,
asymmetrical tonic posturing, or complex
semipurposeful, repetitive movements
that often involve the legs (e.g., bicycling)
• Short duration with minimal or no
postictal confusion
• Occurrence in clusters
• Frequent secondary generalization
• Predilection for occurring at night
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Cryptogenic or Symptomatic Focal
Epilepsies
Three manifestations are especially correlated
with frontal lobe epilepsy :
• Supplementary motor area seizures à
sudden asymmetrical tonic posturing of the
limbs, usually with one arm extended upward,
and contralateral head and eye deviation;
consciousness may or may not be impaired
• Complex partial seizures à prominent motor
activity, such as vigorous rocking, bicycling,
circling, or vocalization; minimal or no
impairment in consciousness; and no
postictal confusion à often suspected as
nonepileptic psychogenic seizures
• Simple partial motor clonic seizures à
arising from regions within or adjacent to the
primary motor cortex
11/03/21 Department of Neurology FKKMK UGM - Dr. Sardjito Hospital
EEG Findings :
• Interictal EEG : EEG is often normal or non
diagnostic à relatively inaccessible to scalp
EEG recording
• Medial frontal epilepsy à epileptiform
discharges are not identified on scalp EEG
recordings
• Secondary bilaterally synchronous discharges à
parasagittal epileptogenic lesions
• Focal epileptiform discharges (highest voltage at
or adjacent to the vertex)
• Orbi frontal foci à high-voltage (up to 300μV),
sharply contoured slow waves that are broadly
distributed over the frontal regions but maximal
at F3-F4 and Fp1-Fp2
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Cryptogenic or Symptomatic Focal
Epilepsies
Ictal EEG : • EEGs sometimes show only diffuse,
• The ictal EEG is nonlocalizing in more bilateral frontal voltage attenuation
than half the patients with FLE à no followed by bilateral frontal or diffuse
electrographic correlate to be seen in rhythmic theta or delta activity à
scalp electrodes orbital frontal seizures
• Prominent motor activity of many
frontal lobe seizures produces large
amounts of muscle and movement
artifact

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Juvenile Myoclonic Epilepsy (JME)
• Inherited disorder (40%-positive family • Absence seizures (30-40%) à infrequent,
history), equal distribution between sexes, brief, and not associated with automatisms
appears around puberty (12-18 y.o) à may be the first manifestation of the
disorder
• JME is frequently under-diagnosed and
under-appreciated; myoclonic jerks appear 2- • Photosensitivity (25–42%); female
3 yrs before the first generalized T-C seizure predominance à generalised spikes, spike
and wave or polyspike and wave in response
• JME à multiple seizure types : myoclonic to intermittent light stimulation (ILS)
seizures (100%) with short, bilateral, single or
repetitive arrhythmic, irregular jerks,
predominantly in the shoulders and arms, it
mostly occurs after awakening from sleep
• Myoclonus is especially marked in the setting
of fatigue and sleep deprivation
• GTCS (90%–95%) à a few minutes,
generalised mild to moderate myoclonus of
increasing frequency and intensity

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Juvenile Myoclonic Epilepsy (JME)
EEG Findings :
• Interictal EEG : JME is characterized by two main
features:
• Normal or near-normal background activity, with a
well-modulated alpha rhythm
• Spontaneous bursts of generalized, bisynchronous
epileptiform discharges.
• Polyspikes and polyspike-wave discharges (not
pathognomonic), consists of a burst of generalized
bisynchronous, maximal voltage in the frontal and
central regions, followed by high-voltage, irregular
2- to 5-Hz slow waves with intermixed spikes
• Spike-wave and polyspike-wave discharge are
"fast” à the repetition rate is higher than the 3-Hz
(3.5 to 6 Hz)
• Hyperventilation/photic stimulation (10-20 Hz)
generally activates epileptiform
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• Ictal EGG : myoclonic seizures à polyspike or
polyspike-wave bursts; polyspikes are of medium
to high voltage, maximally expressed over the
frontal regions, and followed by high-voltage, 1- to
3-Hz rhythmic slow waves
• Extremely brief jerk ("lightning-like") à EEG
discharge is typically 1 to 2 seconds in duration and
may last as long as 4 seconds
• Absence seizures à generalized, irregular (2.5- to
4-Hz) spike and polyspike-waves, several seconds
and interrupted with discontinuities lasting 1
second or less; a classical 3-Hz spike-wave pattern
is uncommon
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 Polyspike and wave discharges Spike and wave discharges

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 Spike-wave discharges Polyspike-wave discharges Polyspike-wave discharges

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 Spike- and polyspike-waves Photoparoxysmal response in JME

EMG leads
(deltoid
muscles)

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Epilepsy with Generalized Tonic-Clonic
Seizures on Awakening
• Idiopathic generalized epilepsy, less
common than JME
• Onset : second decade (range: 5-25
y.o); slight male predominance;
family history of epilepsy is
frequent
• GTCS occur within one hour of
spontaneous morning awakening
at the end of the nocturnal sleep
period (90%), or during
spontaneous or provoked
intermediate awakenings
13/03/21 Department of Neurology FKKMK UGM - Dr. Sardjito Hospital
• Seizures are rare; elicited by sleep
deprivation or excessive
consumption of alcohol on the
previous evening
• Prognosis is usually good, as
seizures remain rare spontaneously
and treatment is effective
EEG Findings :
• Background is normal
• Rare generalized spike-waves and
rare fast polyspike-waves(2-4Hz)
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West’s Syndrome
• One of the most frequent epileptic
syndrome in infants (2.9-4.9 per 100,000)
• This encephalopathy is characterized by a
triad: 1) Infantile spasms, 2)
developmental retardation, 3)
hypsarrhythmia on EEG
• Onset : the first year of life (4-7 months)
• It is associated with neurological
abnormalities arising from a diverse array
of structural, metabolic, and genetic
disorders (90%) or
cryptogenic/idiopathic (10-15%)
12/03/21 Department of Neurology FKKMK UGM - Dr. Sardjito Hospital
• Spasms :
• Brief, symmetric, bilateral tonic
contractions of muscles of trunk, neck and
limbs (flexor spasms) à followed by crying
• Mixed (flexor-extensor), asymmetric or
unilateral, and in the two latter cases often
contralateral to a brain lesion
• Spasms occur in series, often at awakening
or at sleep onset
• Etiologies : neurocutaneous syndrome
(tuberous sclerosis), malformations, pre-,
peri-, or postnatal encephalopathies, as
well as metabolic, degenerative, or
chromosomal diseases, or prenatal
infections
• Prognosis is in part related to the etiology
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West’s Syndrome
EEG findings :
• Interictal EEG : "hypsarrhythmia”
(75%) à onset of the spasm,
background is disorganized, with
high-voltage, asynchronous and
arrhythmic slow waves, in
association with asymmetric and
multifocal spikes and polyspikes
• It is seen in waking and is
fragmented during sleep; occur
selectively during somnolence
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• Electrodecremental response à a
marked suppression of the
background that lasts for the
duration of the spasm
• Surface EMG à brief tonic
muscular contraction
• Focal seizures can initiate the
series of spasms
• EEG is useful in judging successful
treatment of West syndrome, e.g.,
treatment with ACTH or vigabatrin
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 Electrodecremental episodes
Multifocal spikes

Slowing

Multifocal spikes

Slowing

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 Hypsarrhythmia Hypsarrhythmia

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 Flexor spasm

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Lennox-Gastaut Syndrome
• The Lennox-Gastaut syndrome (LGS) triad
: 1) Severe generalized epilepsy, 2)
Mental retardation, 3) EEG pattern of
slow-spike and-wave discharges
• Onset : 1-8 y.o (most cases begin 2-5 y.o)
• ILAE à the generalized cryptogenic or
symptomatic epilepsies, criteria :
• High seizure frequency; tonic, atonic, and
atypical absence seizures; myoclonic,
generalized tonic-clonic, and partial
seizures à patients with LGS have multiple
seizure types, and at least one episode of
status epilepticus occurs in the majority
• Mental retardation, in general; behavioral
disorders, not accompanied by cognitive
impairment, should be sufficient for
diagnosis
12/03/21 Department of Neurology FKKMK UGM - Dr. Sardjito Hospital
• EEG à abnormal background activity with
diffuse sharp slow waves that have a
repetition rate <3 Hz; multifocal spikes or
sharp waves and, during sleep, frequent
bursts of 10 Hz and faster frequencies
• Tonic, atonic, and myoclonic seizures can
all result in the characteristic "drop
attacks"
• LGS cannot be attributed to a single
cause or common pathological substrate
à diverse prenatal, perinatal, and
postnatal disorders have been implicated
(2/3 symptomatic & 1/3 cryptogenic)
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Lennox-Gastaut Syndrome
EEG Findings : • Background slowing à diffuse
abnormalities of background activity
• Interictal EEG : occur (90%), moderate to severe, and
• Slow spike and wave pattern (SSW) correlated with the degree of
à bilateral, symmetrical and highest cognitive impairment
voltage in frontocentral with • Ictal EEG : Electrographic
frequency of 1.5-2.5 Hz
• Paroxymal fast activity (PFA) or accompaniment varies with the
polyspike discharges à diffuse, seizure type
bilaterally synchronous bursts
activity (15-20 Hz) that last several
seconds, highest voltage in the
frontal areas, the frequency : 7-30
Hz, the voltage : 25-250 μV, the
duration : 2-12 seconds, only during
REM sleep

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 Generalized sharo- and • Diffuse backgroud delta frequency (1,5-2 Hz)
slow-wave complex • Bilateral synchronous slow-spike-and-wave
(SSW)

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 SSW discharge (2 Hz frequency) Paroxysmal fast activity (PFA)

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 Generalized PFA & Ictal EEG of tonic seizure
electrodecrement LGSà bilateral synchronous
16-20 Hz activity

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The Syndrome of Continuous Spike and Wave Discharges
During Slow Sleep (CSWS) [Electrical Status Epilepticus During
Slow Sleep]
• Patry et al à "subclinical electrical
status epilepticus induced by sleep”
• CSWS à rare condition (<0.5% of
childhood epilepsies); age at onset 1-
12 y.o. (peaks 5-7 y.o), less
pronounced cognitive regression,
psychomotor impairment, and
epilepsy (focal or generalized seizures
including atypical absences and falls),
boys are more concerned (63%)
• Abnormal neuroimaging à
polymicrogyria, unilateral or diffuse
cortical atrophy or porencephalia.
• Simple partial motor and generalized
tonic-clonic seizures predominate.
• Patients have a significant decline in
IQ with deterioration in language,
temporospatial disorientation,
impaired memory, and reduced
attention span
• Behavioral changes such as
aggressiveness or, rarely, psychosis
also occur

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The Syndrome of Continuous Spike and Wave Discharges
During Slow Sleep (CSWS) [Electrical Status Epilepticus During
Slow Sleep]
EEG Findings : • The discharges can ever disappear,
• Bursts of diffuse spike-waves (2-3 Hz) and focal, frontally predominant
à associated with absences, during changes can be discerned
waking • The EEG becomes progressively
• CSWS appear as soon as the child normal after remission of epilepsy
falls asleep
• Discharges can be asymmetric

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The Syndrome of Acquired Epileptic Aphasia
(Landau-Kleffner Syndrome)
• Landau and Kleffner à acquired aphasia
associated with epileptiform activity on
EEG ("epileptic aphasia")
• It occurs in previously healthy children
(3-9 y.o., peak incidenceà 5-7 y.o. and
never after age 12); boys are affected
twice as often
• The first indication of aphasia is verbal
auditory agnosia, but language function
continues to deteriorate; some children
become mute and do not respond even
to nonverbal sounds
• Hyperactivity and personality changes
appear as the aphasia worsens
12/03/21 Department of Neurology FKKMK UGM - Dr. Sardjito Hospital
• Seizures (70%) à infrequent, partial
motor, atypical absence, generalized
tonic-clonic, and atonic seizures
• Treatment with antiseizure drugs does
not clearly affect aphasia, EEG findings, or
prognosis
• About 2/3 of children have residual
language impairment; cognitive and
behavioral abnormalities are less
frequent consequences
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The Syndrome of Acquired Epileptic Aphasia
(Landau-Kleffner Syndrome)
EEG Findings :
• High-voltage multi focal spikes and
spike-wave discharges singly and
repetitively, the posterior
temporal regions preferentially,
unilateral or bilateral (diffuse and
bisynchronous and either
symmetrical or asymmetrical)
• During REM sleep, the slow spike-
wave pattern fragments and focal
or multi focal discharges appear in
a pattern similar to that seen in the
waking state
12/03/21 Department of Neurology FKKMK UGM - Dr. Sardjito Hospital
• Seizure remit and the EEG
normalizes in nearly all patients by
the end of adolescence, but some
degree of language dysfunction
persists in the majority
• Most patients show improvement,
but the degree of recovery is
variable and unpredictable; some
patients remain profoundly
impaired
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• Mild slowing background activity Continuous spike-and
• Infrequent right midtemporal spikes wave discharges

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 Thank You

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