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EEG in Common Epilepsy Syndromes

Updated: Sep 18, 2017
Author: Raj D Sheth, MD; Chief Editor: Selim R Benbadis, MD more...

Role of EEG in Epilepsy Syndromes

Electroencephalography (EEG) is an essential component in the evaluation of epilepsy. The EEG
provides important information about background EEG and epileptiform discharges and is required
for the diagnosis of specific electroclinical syndromes. [1] Such a diagnosis carries important
prognostic information, guides selection of antiepileptic medication, and suggests when to
discontinue medication. Neurologic examination and imaging in the essential idiopathic, typically
genetic, epilepsies are usually normal. [2]

EEG background frequencies and epileptiform discharges

Following a seizure (ie, during the postictal period) the EEG background may be slow. However,
interictal background EEG frequencies that are slower than normal for age usually suggest a
symptomatic epilepsy (ie, epilepsy secondary to brain insult). Normal background suggests primary
epilepsy (ie, idiopathic or possibly genetic epilepsy). Thus, EEG background offers important
prognostic and classification information.

Epileptiform discharges help clinicians to separate generalized from focal (ie, partial) seizures.

Epilepsy syndromes

Epilepsy syndromes include symptomatic, cryogenic, and idiopathic epilepsy. Symptomatic

epilepsy is defined as seizures resulting from an identifiable cerebral disorder. Cryptogenic epilepsy
consists of seizures that occur without an identifiable cause in a patient with cognitive impairment
or with neurologic deficits (eg, Lennox-Gastaut syndrome (LGS), infantile spasms [see the first
image below], and myoclonic astatic epilepsy of Doose.)

Idiopathic epilepsy consists of seizures that occur without an identifiable cause in a patient with
entirely normal findings on neurologic examination and of normal intelligence (eg, benign partial
epilepsy of childhood with centrotemporal spikes [BECTS], benign partial epilepsy of childhood with
occipital paroxysms [BPEOP], and juvenile myoclonic epilepsy [see the second image below]).

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Electroencephalogram demonstrating hypsarrhythmia in infantile spasms. Note the chaotic high-amplitude

background.
View Media Gallery

Electroencephalogram demonstrating polyspike and wave discharges seen in juvenile myoclonic epilepsy.
View Media Gallery

EEG characteristics of these specific electroclinical epilepsy syndromes are discussed in this
article. Roles of EEG in temporal lobe epilepsy and frontal lobe epilepsy, among others, are not
addressed here.

For more information, see the following:

Epilepsy and Seizures

Benign Childhood Epilepsy
First Adult Seizure
First Pediatric Seizure
EEG in Status Epilepticus
Epileptiform Normal Variants on EEG
EEG Video Monitoring

Neonatal Seizures
Generalized seizures are rare in neonates. Many of the so-called subtle, generalized tonic, and
multifocal myoclonic seizures do not have an electroencephalographic (EEG) correlate. These
movements in the severely affected infant may represent brain stem release phenomena. Focal
seizures, particularly clonic seizures, are highly associated with EEG changes. Thus, EEG plays a
crucial role in the evaluation of neonatal seizures. The EEG changes significantly with gestational
age; therefore, calculation of gestational age and familiarity with age-specific norms is crucial in
interpretation of the EEG in infants.

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Two well-defined EEG seizure patterns are seen in neonates, as follows:

Seizures with focal low-frequency electrographic correlates: These patterns may occur at
1-1.5 Hz frequency and are generally seen in severe cerebral insults, such as severe
hypoxic-ischemic encephalopathy.
Seizures with focal high-frequency electrographic correlates: These patterns typically evolve
over 10-20 seconds and are usually seen with focal cerebral insults, such as strokes. Strokes
in the neonate, unlike in the older individual, are typically associated with porencephalic cysts.
Porencephalic cysts result from strokes that involve large portions of the cerebral
parenchyma (ie, loss of both gray and white matter leading to a communication between the
subarachnoid space and the cerebral ventricles).

For more information, see Benign Neonatal Convulsions.

Infantile Spasms and West Syndrome

West syndrome is a triad of infantile spasms, developmental retardation or regression, and
hypsarrhythmia on electroencephalogram (EEG). The syndrome presents in infants aged between
6 and 18 months.

The presence of a hypsarrhythmic EEG confirms the diagnosis of infantile spasms (see the
following image). EEG patterns may evolve over a time period; they initially appear in the sleep
EEG record and subsequently present during the awake state. Hypsarrhythmia is seen in 75% of
patients with West syndrome.

Electroencephalogram demonstrating hypsarrhythmia in infantile spasms. Note the chaotic high-amplitude

background.
View Media Gallery

Hypsarrhythmia consists of diffuse giant waves (high voltage, >400 microvolts [µV]) with a chaotic
background of irregular, multifocal spikes and sharp waves and very little synchrony between the
cerebral hemispheres. During sleep, the EEG may display bursts of synchronous polyspikes and
waves. A pseudoperiodic pattern may be evident. Persistent slowing or epileptiform discharges in
the hypsarrhythmic background may be present and may represent an area of focal dysfunction.
Several variations to the hypsarrhythmic pattern, which are referred to as hypsarrhythmic variants,
may be noted.

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Clinical spasms are associated with a marked suppression of the background that lasts for the
duration of the spasm. This characteristic response is called the electrodecremental response (see
the image below).

Electroencephalogram demonstrating hypsarrhythmia. Note the electrodecremental response that is

associated with a spasm in infantile spasms (ie, West syndrome).
View Media Gallery

EEG is useful in judging successful treatment of West syndrome. Typically, shortly after treatment
with adrenocorticotropic hormone (ACTH) or vigabatrin is initiated, the spasms stop and
hypsarrhythmia disappears.

Hypsarrhythmia rarely persists beyond the age of 24 months. It may evolve into the slow spike and
wave discharges seen in Lennox-Gastaut syndrome.

Lennox-Gastaut Syndrome
Lennox-Gastaut syndrome (LGS) is a childhood (onset, age 3-5 y) epileptic encephalopathy that
manifests with atonic seizures, tonic seizures, and atypical absence seizures associated with
mental retardation and a characteristic electroencephalographic (EEG) pattern. Infantile spasms
and West syndrome frequently transform into Lennox-Gastaut syndrome. However, unlike West
syndrome, Lennox-Gastaut syndrome tends to be a lifelong epileptic encephalopathy.

The EEG in affected patients shows an abnormally slow background and diffuse slow spike and
slow wave (< 2.5 Hz) activity (see the images below). The slow spike and wave activity serves to
differentiate (poor prognosis) Lennox-Gastaut syndrome from benign absence epilepsy, in which
diffuse 3-Hz spike and wave activity is seen, and from some of the more benign myoclonic types of
epilepsy characterized by fast spike and wave (>2.5 Hz) activity, which carries a dramatically better
prognosis than Lennox-Gastaut syndrome. Many other epilepsy syndromes overlap with Lennox-
Gastaut syndrome, however, including myoclonic astatic epilepsy of Doose and other severe
myoclonic epilepsies.

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Slow (&lt; 2.5 Hz) electroencephalographic spike and wave discharges associated with atypical absence
seizures (ie, Lennox-Gastaut syndrome).
View Media Gallery

Slow (&lt; 2.5 Hz) electroencephalographic spike and wave discharges in atypical absence epilepsy (ie,
Lennox-Gastaut syndrome).
View Media Gallery

EEG features of Lennox-Gastaut syndrome may be divided into interictal and ictal.

Characteristic interictal EEG features consist of background slowing and diffuse slow spike and
wave activity that last from several minutes to a near continuous state. The duration of the
epileptiform discharges tends to correlate with epilepsy control, with shorter durations occurring in
patients with better control of seizures. Spikes, or more commonly sharp waves, are typically 200
milliseconds in duration and are followed by slow waves. Polyspike discharges are seen in those
epilepsy variants with prominent myoclonic seizures or during non–rapid eye movement (REM)
sleep.

Ictal EEG features have varying electrographic accompaniment with the seizure type.

For more information, see Lennox-Gastaut Syndrome.

Childhood Absence Epilepsy

Childhood absence epilepsy (CAE) presents between ages 3 and 5 years and usually remits by
ages 10-12 years. Unlike juvenile absence epilepsy, childhood absence epilepsy is usually not
associated with tonic-clonic seizures. Electroencephalography (EEG) shows a normal background
for age and 3-Hz generalized spike and wave discharges (see the following image).

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Typical 3-Hz electroencephalographic spike and wave discharges seen in absence epilepsy.
View Media Gallery

The frequency of the spike-wave complexes is usually 4 Hz at the onset of the absence seizures
and may slow to 2.5 Hz at the end of a seizure. [3] Typically, an initial positive component is
followed by one or more negative components and then a negative slow wave. They are frontally
dominant (see the image below). The duration of the discharges is typically 3-25 seconds.

Electroencephalogram demonstrating absence epilepsy. Anteriorly dominant, typical 3-Hz spike and wave
discharges.

View Media Gallery

The discharges are not truly bisynchronous; usually a millisecond difference is noted between the
left and right cerebral hemispheres. Eye opening does not alter the discharges. However, the
discharges are state dependent; their frequency increases with non–rapid eye movement (REM)
sleep, although the duration of the discharges is reduced. During REM sleep, the frequency of
discharges resembles that seen in wakefulness. Some patients display occipital intermittent
rhythmic delta discharges (OIRDA), which is thought to be a favorable prognostic indicator.

Generalized discharges in childhood absence epilepsy are ictal in nature. They may be so brief that
no obvious clinical movements are seen, although typically minor eyelid fluttering or subtle,
rhythmic contractions of the mouth are seen. These minor motor accompaniments occur in 85% of
patients with absence epilepsy.

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Absence status epilepticus vs childhood absence epilepsy

Absence status epilepticus occurs in about 10% of patients with childhood absence epilepsy.
Typically, a child with staring spells is misdiagnosed as having partial complex seizures and is
treated with carbamazepine. In fact, carbamazepine can precipitate absence status, which is a
nonconvulsive status epilepticus in which patients appear to be in a "twilight state." They are able
to answer questions intermittently, although at times they are confused. EEG is crucial in the
diagnosis; it shows near-continuous generalized spike and wave discharges.

Absence seizure vs atypical absence seizure

Absence should be differentiated from atypical absence seizures, which usually are seen in
patients with Lennox-Gastaut syndrome. The EEG in atypical absence seizures shows a less
abrupt onset and offset than in typical absence seizures. Furthermore, the EEG background is
slow, and the duration of discharges is shorter.

For more information, see Absence Seizures.

Benign Partial Epilepsies

Two benign partial epilepsy syndromes of childhood have been well defined: (1) benign rolandic
epilepsy (BRE), also called benign partial epilepsy of childhood with centrotemporal spikes
[BECTS] and (2) benign partial epilepsy of childhood with occipital paroxysms (BPEOP). Other less
well-defined syndromes include frontal and parietal partial epilepsy syndromes.

Benign rolandic epilepsy

Patients with benign rolandic epilepsy are typically aged 3-10 years. They may present with a
history of orobuccal numbness on one side of the mouth or with a tingling sensation on one side of
the face. These seizures are associated with preserved mentation and thus are simple partial
seizures. During sleep, patients may have generalized tonic-clonic convulsions.

The electroencephalographic (EEG) features of benign rolandic epilepsy include frequent spike and
wave discharges in the centrotemporal region (see the image below). The electrical field of
epileptiform discharges is not distributed widely. Frequently, the dipole is located tangentially, with
positivity in the frontal regions. [4] The negative pole is 150-300 microvolts (µV), and the entire spike
and wave complex lasts for 80-120 milliseconds. Characteristically, the spike is triphasic and
blends into the after-coming slow wave.

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Benign rolandic epilepsy associated with typical centrotemporal electroencephalographic spikes.

View Media Gallery

Commonly, epileptiform discharges occur in runs, and they may be bilateral in 30% of patients;
when they occur bilaterally, the discharges are independent and asynchronous. Unilateral
discharges are more common. Activating movements or eye opening does not block the
discharges. Sleep, however, has a prominent activation on the epileptiform discharges (see the
following image). Non–rapid eye movement (REM) sleep, in particular, may show a 400-500%
increase in the spike-wave index. Over time, the epileptiform discharges decrease, and they finally
disappear around age 15 years. At times, the EEG, in addition to displaying centrotemporal spikes,
can show generalized or multifocal spike wave discharges. [5]

Electroencephalogram demonstrating benign rolandic epilepsy. Note the characteristic spike and waves seen
in drowsiness.
View Media Gallery

Benign rolandic epilepsy appears to be a dominantly inherited condition with variable penetrance.
[4] This condition is a syndromic diagnosis, with the EEG forming an important component of the
diagnosis. However, epileptiform discharges in the rolandic region do not necessarily mean that the
patient has benign rolandic epilepsy.

Benign partial epilepsy of childhood with occipital paroxysms

Gastaut described a partial epilepsy that was analogous to benign rolandic epilepsy, although the 2
syndromes have important differences. [6] In BPEOP, for example, epileptiform discharges are
located in the posterior head region, most prominently in the occipital region. Typical phenomena
include interictal high-voltage (200-300 µV) EEG spike and wave complexes occurring in runs with
a degree of rhythmicity and a frequency of 1-3 Hz. Typically, they are blocked or prominently
attenuated with eye opening. The complexes may be unilateral or bilateral and may occur
independently on each side.

As in benign rolandic epilepsy, the occipital spike-wave index is activated prominently with non-
REM sleep. Generalized spike and wave discharges may be present in 10% of children. Unlike
benign rolandic epilepsy, which remits in most patients by age 16 years, BPEOP may persist in
20% of patients after age 20 years.

For more information, see Benign Childhood Epilepsy.

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Juvenile Myoclonic Epilepsy

Juvenile myoclonic epilepsy (JME) is the most common epilepsy syndrome presenting with
generalized tonic-clonic seizures in a patient aged 12-30 years who is otherwise neurologically
normal. The imaging findings are normal. This condition may account for as many as 10% of all
patients with epilepsy. In susceptible persons, sleep deprivation often precipitates seizures.

Typically, the patient may experience myoclonic jerks in the morning, although many patients do
not mention that they are having myoclonic seizures until asked specifically about body jerks.

Approximately 15% of patients have associated juvenile absence epilepsy or generalized tonic-
clonic seizures upon awakening. Often, the diagnosis is not made in a definitive fashion, which is
unfortunate, as a correct diagnosis helps guide management. This, in turn, affects prognosis,
because the drugs used in this entity differ from those used in most other seizure types. [7]

The interictal electroencephalogram (EEG) shows a normal background with frequent generalized
polyspike and wave discharges that may be anteriorly dominant or diffuse (see the following
images. By definition, polyspike and wave discharges have at least 3 spikelike components in
them. [8]

For more information, see Juvenile Myoclonic Epilepsy.

References

1. Nolan MA, Redoblado MA, Lah S, et al. Memory function in childhood epilepsy syndromes. J
Paediatr Child Health. 2004 Jan-Feb. 40(1-2):20-7. [Medline].

2. Urbach H. Imaging of the epilepsies. Eur Radiol. 2005 Mar. 15(3):494-500. [Medline].

3. Loiseau P, Duche B, Pedespan JM. Absence epilepsies. Epilepsia. 1995 Dec. 36(12):1182-6.
[Medline].

4. Legarda S, Jayakar P, Duchowny M, et al. Benign rolandic epilepsy: high central and low
central subgroups. Epilepsia. 1994 Nov-Dec. 35(6):1125-9. [Medline].

5. Beydoun A, Garofalo EA, Drury I. Generalized spike-waves, multiple loci, and clinical course
in children with EEG features of benign epilepsy of childhood with centrotemporal spikes.
Epilepsia. 1992 Nov-Dec. 33(6):1091-6. [Medline].

6. Andermann F, Zifkin B. The benign occipital epilepsies of childhood: an overview of the

idiopathic syndromes and of the relationship to migraine. Epilepsia. 1998. 39 suppl 4:S9-23.
[Medline].

7. Panayiotopoulos CP, Tahan R, Obeid T. Juvenile myoclonic epilepsy: factors of error involved
in the diagnosis and treatment. Epilepsia. 1991 Sep-Oct. 32(5):672-6. [Medline].

8. Janz D. Juvenile myoclonic epilepsy. Epilepsy with impulsive petit mal. Cleve Clin J Med.
1989. 56 suppl pt 1:S23-33; discussion S40-2. [Medline].

9. Benbadis S. The differential diagnosis of epilepsy: a critical review. Epilepsy Behav. 2009

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May. 15(1):15-21. [Medline].

10. Benbadis SR, Chen S, Melo M. What's shaking in the ICU? The differential diagnosis of
seizures in the intensive care setting. Epilepsia. 2010 Nov. 51(11):2338-40. [Medline].

Media Gallery

Electroencephalogram demonstrating hypsarrhythmia in infantile spasms. Note the chaotic

high-amplitude background.
Electroencephalogram demonstrating hypsarrhythmia. Note the electrodecremental response
that is associated with a spasm in infantile spasms (ie, West syndrome).
Slow (&lt; 2.5 Hz) electroencephalographic spike and wave discharges associated with
atypical absence seizures (ie, Lennox-Gastaut syndrome).
Slow (&lt; 2.5 Hz) electroencephalographic spike and wave discharges in atypical absence
epilepsy (ie, Lennox-Gastaut syndrome).
Typical 3-Hz electroencephalographic spike and wave discharges seen in absence epilepsy.
Electroencephalogram demonstrating absence epilepsy. Anteriorly dominant, typical 3-Hz
spike and wave discharges.
Benign rolandic epilepsy associated with typical centrotemporal electroencephalographic
spikes.
Electroencephalogram demonstrating benign rolandic epilepsy. Note the characteristic spike
and waves seen in drowsiness.
Electroencephalogram demonstrating polyspike and wave discharges seen in juvenile
myoclonic epilepsy.
Electroencephalogram demonstrating polyspike and wave response produced by photic
stimulation.

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Contributor Information and Disclosures

Author

Raj D Sheth, MD Chief, Division of Pediatric Neurology, Nemours Children's Clinic; Professor of
Neurology, Mayo College of Medicine; Professor of Pediatrics, University of Florida College of
Medicine

Raj D Sheth, MD is a member of the following medical societies: American Academy of Neurology,
American Academy of Pediatrics, American Epilepsy Society, American Neurological Association,
Child Neurology Society

Disclosure: Nothing to disclose.

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Specialty Editor Board

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical
Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Received salary from Medscape for employment. for: Medscape.

Norberto Alvarez, MD Assistant Professor, Department of Neurology, Harvard Medical School;

Consulting Staff, Department of Neurology, Boston Children's Hospital; Medical Director, Wrentham
Developmental Center

Norberto Alvarez, MD is a member of the following medical societies: American Academy of

Neurology, American Epilepsy Society, Child Neurology Society

Disclosure: Nothing to disclose.

Chief Editor

Selim R Benbadis, MD Professor, Director of Comprehensive Epilepsy Program, Departments of

Neurology and Neurosurgery, Tampa General Hospital, University of South Florida Morsani College
of Medicine

Selim R Benbadis, MD is a member of the following medical societies: American Academy of

Neurology, American Academy of Sleep Medicine, American Clinical Neurophysiology Society,
American Epilepsy Society, American Medical Association

Disclosure: Serve(d) as a director, officer, partner, employee, advisor, consultant or trustee for:
Acorda, Cyberonics, Eisai, Lundbeck, Sunovion, UCB, Upsher-Smith<br/>Serve(d) as a speaker or
a member of a speakers bureau for: Cyberonics (Livanova), Eisai, Lundbeck, Neuropace,
Sunovion, UCB<br/>Received research grant from: Acorda, Cyberonics, GW, Lundbeck, Sepracor,
Sunovion, UCB, Upsher-Smith.

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