NeuroLogic: Seizure Management

Seizure Management

Welcome

Start here

EEG Fundamentals taught you to read the record. This course teaches what to do for the patient: how seizures and epilepsy are evaluated and treated, and the evidence behind each step.

About 4 hours 55 minutes28 short lessons · read in order or choose a topic from Contents

Each lesson explains the reasoning, shows the trial or guideline that supports it, links back to the electroencephalography (EEG) lesson it builds on, and ends with a quick check. It is written for neurology residents; fellows can use single lessons for review.

How to read the lessons

  • Green: what to do
  • Red: harm, what to avoid, a contraindication
  • Blue: a number worth remembering
  • Highlighted: the point most often examined

The hand-drawn figures are the lecture's drawings. Each draws itself the first time you reach it; Play draws it again, and the numbered buttons show one part at a time.

This course gives no dosing advice

It teaches how to choose and use treatment, not how much to give. Doses, titration and administration depend on the patient and change over time, so take them from your institution's order sets and a current drug reference when you prescribe. Where a study reports risk by dose, as the pregnancy registries do, the dose bands are shown as evidence, not as advice.

Start the course

By the end, you can

  • Decide whether a first event was a seizure, whether it was provoked, and whether treatment should start.
  • Read a seizure's signs for where it starts, find its cause, and recognize functional seizures.
  • Choose a first antiseizure medication from the seizure type, the epilepsy and the patient, and know when drug levels and interactions matter.
  • Run the treatment of convulsive status epilepticus against the clock, and act on what continuous EEG shows afterwards.
  • Recognize drug-resistant epilepsy early and explain what the epilepsy center can offer.
  • Write a rescue plan for seizure clusters, and talk with patients about sudden unexpected death in epilepsy and everyday safety.
  • Counsel women with epilepsy about contraception, pregnancy and the hormonal changes of a lifetime.

Course topics

  1. The first seizureEvaluation, and when to treat
  2. DiagnosisSemiology, the cause, functional seizures, seizures early in life
  3. Antiseizure medicationsChoosing, mechanisms, profiles, levels
  4. Status epilepticusThe clock, treatment, continuous EEG
  5. Drug-resistant epilepsyReferral, evaluation, surgery and devices
  6. Stopping treatmentWhen and how to discuss it
  7. Living with epilepsyClusters and rescue medicines, sudden death, safety
  8. Women's health and hormonesCycle, contraception, pregnancy, menopause

Written for teaching by the NeuroLogic team from primary studies and guidelines, cited in each lesson. Reviewed October 2026. Use local protocols and current prescribing information for patient care.

Evaluating a first seizure

Clinical reasoning

A first event raises three questions, in order: was it an epileptic seizure, was it provoked, and what does the workup show? The answers decide whether this is epilepsy and whether to treat.

1. Was it an epileptic seizure?

Most of the answer comes from the history, ideally from someone who saw the event. Transient ischemic attack, migraine aura, parasomnias and hypoglycemia can all be mistaken for seizures. Two mimics deserve particular attention:

Induced syncope in healthy volunteers (Lempert 1994) and eye closure in one video-electroencephalography series (Chung 2006).

Convulsive syncope

  • When syncope was induced in healthy volunteers, 38 of 42 with complete syncope (90%) had myoclonic jerks, usually multifocal and arrhythmic; 79% had other movements such as head turns or oral automatisms.
  • The eyes usually stayed open, and the whole episode lasted about 12 seconds.
  • Jerking does not make an event epileptic.
  • In 539 people with loss of consciousness and a known diagnosis (102 with epileptic seizures, 437 with syncope), a point score built only from the history separated seizures from syncope with 94% sensitivity and 94% specificity.

Sources: Lempert 1994; Sheldon 2002.

Functional/dissociative seizures

  • The term proposed by the International League Against Epilepsy (ILAE) task force in 2025 for what were called psychogenic nonepileptic seizures.
  • In one video-electroencephalography (video-EEG) series, 96% of patients with functional seizures kept their eyes closed, while 97% of those with epileptic seizures had them open.
  • Pooled across studies the sign is less reliable, especially in blinded studies, and witnesses' accounts of the eyes are often wrong.
  • Video-EEG of a typical event settles the question.

Sources: Hingray 2025; Chung 2006; Brigo 2013; Syed 2011.

2. Was it provoked?

An acute symptomatic, or provoked, seizure happens at the time of an acute brain insult or systemic disturbance. It is treated by treating the cause, and it does not count toward a diagnosis of epilepsy. The International League Against Epilepsy (ILAE) definitions, written for epidemiological research, with metabolic cutoffs chosen to favor specificity, include, among others (Beghi 2010):

  • within 7 days of a stroke, traumatic brain injury, intracranial surgery or anoxic encephalopathy, or during an active infection of the central nervous system;
  • 7 to 48 hours after the last drink in alcohol withdrawal in someone with chronic alcohol use, or during withdrawal from benzodiazepines or barbiturates; also intoxication or exposure to a drug that provokes seizures;
  • with a severe metabolic disturbance in blood drawn within 24 hours: glucose below 36 mg/dL, or above 450 mg/dL with ketoacidosis; sodium below 115 mmol/L; calcium below 5 mg/dL; magnesium below 0.8 mg/dL; urea nitrogen above 100 mg/dL; or creatinine above 10 mg/dL.
When a seizure counts as acute symptomatic, from the ILAE definitions (Beghi 2010). One unprovoked seizure a month or more after a stroke is the ILAE report's example of epilepsy after a single seizure (Fisher 2014).

A seizure with a milder metabolic disturbance does not meet the definition of acute symptomatic, but the report does not class it as unprovoked either: it falls into an "unknown" category that does not count toward epilepsy. A seizure months or years after a stroke or brain injury is not provoked: it is a remote symptomatic seizure, and it may already mean epilepsy, as the next lesson explains.

3. The workup

Yields after a first seizure in adults (Krumholz 2007), the timing of the EEG (King 1998) and emergency imaging (Harden 2007).

EEG

  • A routine EEG showed epileptiform abnormalities in about 23% of adults after a first seizure, and these predicted recurrence.
  • Timing matters: in 300 consecutive adults and children, an EEG within 24 hours found epileptiform abnormalities in 51%, against 34% when it was done later.
  • With the EEG, a syndrome could be diagnosed in 77% instead of 47%.

Sources: Krumholz 2007; King 1998.

Imaging

  • Computed tomography (CT) or magnetic resonance imaging (MRI) was significantly abnormal in about 10%.
  • In the emergency department, a noncontrast CT changes acute management in 9 to 17% of adults with a first seizure.
  • An MRI with an epilepsy protocol should follow soon after a first seizure, if resources allow; it is not routinely needed for typical genetic generalized or self-limited syndromes.

Sources: Krumholz 2007; Harden 2007; Bernasconi 2019.

Laboratory tests, lumbar puncture and toxicology

  • Guided by the clinical picture rather than done routinely.
  • Laboratory tests were abnormal in up to 15%, but the abnormalities were minor and did not cause the seizure.
  • Fever or other signs of infection predicted abnormal cerebrospinal fluid.

Source: Krumholz 2007.

The 2007 American Academy of Neurology parameter on evaluating a first seizure (the source of the 23% and 10% yields) was retired in 2018; its figures remain the clearest summary of what each test finds. The emergency neuroimaging review (Harden 2007) was reaffirmed in 2025.

Key points

  • Brief myoclonic jerks are common in syncope; the history, and video-EEG when needed, decide.
  • A provoked seizure has a cause to treat and does not by itself mean epilepsy.
  • An early EEG and an epilepsy-protocol MRI give the most information.

Quick check A 45-year-old man with alcohol use disorder stops drinking abruptly and has a generalized tonic–clonic seizure 30 hours after his last drink, with tremor and diaphoresis. Computed tomography of his head is normal. How is this seizure classified?

Sources: as cited. Seizure terms follow the ILAE classification as updated in 2025 (Beniczky 2025).

When a first seizure becomes epilepsy, and when to treat

Evidence

After one unprovoked seizure, the question is how likely a second one is. That probability decides both the diagnosis of epilepsy and the case for starting treatment.

The ILAE definition of epilepsy

The International League Against Epilepsy (ILAE) practical definition says a person has epilepsy after any of (Fisher 2014):

  1. at least two unprovoked (or reflex) seizures more than 24 hours apart;
  2. one unprovoked (or reflex) seizure and a probability of further seizures similar to the risk after two unprovoked seizures, at least 60%, over the next 10 years;
  3. the diagnosis of an epilepsy syndrome.

The 60% is a guide, not a sharp line; after two unprovoked seizures the risk of more is about 60 to 90%. The report's example for the second criterion is a single unprovoked seizure at least a month after a stroke, and it warns that one seizure with a lesion, or with epileptiform discharges, does not automatically qualify. Seizures clustered within 24 hours count as one. Epilepsy is resolved in someone who has outgrown an age-dependent syndrome, or who has been seizure-free for 10 years and off medication for the last 5.

How likely is a second seizure?

Level A, B, C: the American Academy of Neurology grades each guideline recommendation by the evidence behind it, from A (the strongest) to C (the weakest).

After a first unprovoked seizure in an adult, the risk of another is highest early: 21 to 45% within 2 years (Level A). These figures come from cohorts that mixed treated and untreated people, and the risk appeared lower in those treated (Krumholz 2015). After a second unprovoked seizure, about three-quarters of people had another within 4 years (Hauser 1998).

Four features raise the risk after a first seizure

  • A prior brain insult such as stroke or trauma (Level A).
  • An electroencephalogram (EEG) with epileptiform abnormalities (Level A).
  • A significant abnormality on brain imaging (Level B).
  • A nocturnal seizure (Level B).

Source: Krumholz 2015.

Range across cohorts in the American Academy of Neurology and American Epilepsy Society guideline (Krumholz 2015); second and third seizures in one cohort (Hauser 1998); the threshold from the ILAE practical definition (Fisher 2014).

What immediate treatment does, and does not, do

Evidence

First Seizure Trial (FIRST): immediate or deferred treatment after a first tonic–clonic seizure, 397 people aged 2 to 70
At 2 years, 25% of the treated group had relapsed against 51% of the untreated (FIRST Group 1993). In longer follow-up of an enlarged cohort of 419, the chance of reaching 1 or 2 years without seizures was the same whether treatment began after the first seizure or after a relapse, and 42% of the deferred group had a recurrence during follow-up (Musicco 1997).
Multicentre trial for Early Epilepsy and Single Seizures (MESS): immediate or deferred treatment, 1,443 people
Immediate treatment delayed the next seizure and shortened the time to 2-year remission, but 76% and 77% were seizure-free between years 3 and 5 after randomization, and quality of life did not differ (Marson 2005).
Immediate treatment halved relapse in the first 2 years but did not change who was seizure-free years later (FIRST Group 1993; Marson 2005).

The American Academy of Neurology and American Epilepsy Society guideline sums it up: immediate treatment lowers the absolute risk of recurrence over the next 2 years by about 35%, the guideline's estimate (Level B), is unlikely to improve the chance of long-term remission (Level B), may not improve quality of life (Level C), and causes adverse effects in 7 to 31%, mostly mild and reversible (Level B) (Krumholz 2015).

Making the decision together

Treatment after a first seizure is a choice, not a rule. It weighs the individual recurrence risk against the burden of daily medication and its adverse effects, and it depends on what a second seizure would cost this person: driving, work, living alone, pregnancy plans. Many people choose to wait; others, especially those who drive for work, choose to start.

Key points

  • Epilepsy can be diagnosed after one unprovoked seizure when the risk of another is at least 60%; the ILAE report's example is a seizure a month or more after a stroke.
  • After a first unprovoked seizure, 21 to 45% recur within 2 years; a prior brain insult and an epileptiform EEG are the best-established predictors (Level A).
  • Immediate treatment reduces early recurrence but not the chance of long-term remission, so the choice is shared.

Quick check A 62-year-old has his first focal to bilateral tonic–clonic seizure 8 months after a left middle cerebral artery stroke. Nothing else provoked it. Does he have epilepsy by the ILAE practical definition?

Sources: as cited. The 2015 guideline was reaffirmed by the American Academy of Neurology in 2024.

What the first sign says: localizing and lateralizing signs

Clinical reasoning

A seizure's signs, read in the order they appear, show where the discharge is and where it goes. Before any test, they often tell which lobe, and which side, the seizures come from.

Describe the whole sequence

The International League Against Epilepsy (ILAE) seizure classification, updated in 2025, sorts a focal seizure by consciousness, preserved or impaired, judged from two things: whether the person recalls the seizure (awareness) and whether they respond normally during it (responsiveness). If either is impaired, consciousness is impaired. In its expanded version the seizure is then described by listing its signs in the order they appear, joined by arrows, because the sequence characterizes the epileptic network better than the first sign alone (Beniczky 2025).

The classification's own example of a focal impaired consciousness seizure (Beniczky 2025), the commonest aura in mesial temporal lobe epilepsy (French 1993) and the lateralizing value of dystonic posturing (Bleasel 1997).

Two rules sit behind localization. A sign shows where the discharge is when the sign appears, the symptomatogenic zone, which may lie some way from where the seizure began (the zones are set out in Presurgical evaluation). And primary cortex produces elementary symptoms while association cortex produces complex ones: among 20 surgically treated patients with visual auras, complex visual hallucinations never came from occipital lobe seizures, while elementary hallucinations, illusions and visual loss came from occipital, occipitotemporal and anteromedial temporal onsets alike (Bien 2000).

Where the first sign points

Mesial temporal

  • In 67 patients with proven mesial temporal onset, an abdominal visceral (epigastric) aura was by far the commonest, in 61%.
  • 81% had had convulsions in early childhood, most with fever; habitual seizures began at a mean age of 9 years.
  • Consciousness is then impaired, with oral and hand automatisms, as in the classification's example above.

Sources: French 1993; Beniczky 2025.

Supplementary motor area and sleep-related hypermotor epilepsy

  • Supplementary motor area seizures are short, stereotyped and tend to arise from sleep, often with tonic abduction of the arms at onset. They are easily mistaken for functional seizures, which are long, variable and arise from wakefulness.
  • Sleep-related hypermotor epilepsy replaced the name nocturnal frontal lobe epilepsy: the attacks are tied to sleep rather than the time of day, can arise outside the frontal lobe, and have characteristic motor features. The diagnosis has three levels of certainty: witnessed, video-documented and confirmed by video-electroencephalography (video-EEG).

Sources: Kanner 1990; Tinuper 2016.

Insula

  • With consciousness preserved: a sensation of laryngeal constriction and paresthesias, often unpleasant, over large areas of skin; then dysarthria and focal motor signs.
  • In stereo-electroencephalography of 50 patients with temporal lobe epilepsy whose seizures were suspected to involve the perisylvian cortex, 6 had this insular sequence. Seen at the start of seizures that otherwise look temporal, it points to the insula, which should be recorded directly before any decision on surgery.

Source: Isnard 2004.

Visual auras

  • Elementary hallucinations, illusions and visual loss are typical of occipital onset but do not exclude a temporal onset.
  • Complex (formed) hallucinations and tunnel vision point to an anteromedial temporal or occipitotemporal onset.

Source: Bien 2000.

Signs that tell the side

Some signs point to the hemisphere where the seizure starts. They differ in how often they appear, how well observers agree on them and how often they are right.

Lateralizing signs drawn for a left-hemisphere onset, from video-EEG series (Bleasel 1997; Kotagal 2000; Wyllie 1986; Leutmezer 1998; Gabr 1989; Kramer 1988; Marashly 2016).
SignSide of onsetHow reliable
Dystonic posturing of one armOppositeIn temporal lobe epilepsy, present in 35%, with a positive predictive value of 92% and good agreement between observers (Bleasel 1997)
Forced, sustained head and eye versionOppositePositive predictive value 100% in temporal and extratemporal seizures (Bleasel 1997). Version late in a convulsion is often toward the onset, in 12 of 27 such convulsions, so it does not lateralize (Wyllie 1986)
Figure-of-4: one elbow extended, the other flexed, as the seizure becomes bilateralOpposite the extended arm35 of 39 patients (Kotagal 2000); less reliable in a later blinded series (Marashly 2016)
Unilateral tonic posturing early in a temporal seizureNot reliablePositive predictive value 40%, with weak agreement between observers (Bleasel 1997)
Postictal nose wipingSame side as the handSeen in 51% of 76 patients with temporal lobe epilepsy; the hand was on the side of onset in 97% of patients (Leutmezer 1998)
Postictal aphasiaDominant temporal lobe92%, with dominance tested by intracarotid amobarbital (Gabr 1989)
Identifiable words during a seizure with impaired consciousnessNondominant temporal lobe83% (Gabr 1989)
Ictal vomitingRight temporal lobeAll 9 patients in one series (Kramer 1988)

Signs are stronger together. In a blinded review of 47 seizures that became bilateral, version, unilateral tonic or clonic activity, an asymmetric end to the clonic phase and Todd paresis each pointed to the correct side more than 80% of the time, while hand dystonia and the figure-of-4 were less reliable in that setting. When two or more reliable signs agreed, the side was correct in every patient, but no sign separated temporal from extratemporal onset (Marashly 2016).

Key points

  • Describe a seizure as its sequence of signs: the first sign shows the cortex the discharge reaches first, the sequence the network it spreads through.
  • An epigastric aura suggests mesial temporal onset; laryngeal constriction with widespread paresthesias suggests the insula.
  • Dystonia of one arm and forced version point to the opposite hemisphere, and postictal nose wiping to the same side; two reliable signs that agree were correct in every patient of one series.

Quick check A right-handed 34-year-old feels a rising sensation from his stomach, then stares. His wife sees lip smacking and fumbling, while his right arm stiffens in a twisted posture. Afterwards he wipes his nose with his left hand. Where do his seizures most likely begin?

Quick check On video-EEG, a seizure begins with forced, sustained turning of the head and eyes to the right. Both arms then stiffen, the right elbow extended and the left flexed, and a bilateral convulsion follows. As it ends, the head turns to the left. Which hemisphere does the seizure most likely start in?

Quick check A 30-year-old is being evaluated for temporal lobe surgery. Video-EEG shows that each seizure starts, with consciousness preserved, with a sensation of laryngeal constriction and unpleasant tingling over a large area of skin, followed by slurred speech. The scalp EEG suggests right temporal onset. What should happen before surgery?

Sources: as cited. Seizure terms follow the ILAE classification as updated in 2025 (Beniczky 2025).

Finding the cause: the scan, the genes and what changes treatment

Clinical reasoning

From the first seizure on, the question is not only whether this is epilepsy but what causes it. The cause often decides treatment: a lesion for surgery, a diet or a vitamin that treats the defect itself, immunotherapy, or a medication that must be avoided.

Six groups of causes

The International League Against Epilepsy (ILAE) classification of the epilepsies asks for the etiology at every step, from the first seizure on, in six groups chosen for their treatment consequences: structural, genetic, infectious, metabolic, immune and unknown. The groups are not exclusive: tuberous sclerosis complex is structural, which matters for surgery, and genetic, which matters for counseling and for mechanistic target of rapamycin (mTOR) inhibitors (Scheffer 2017).

The six etiology groups of the 2017 ILAE classification of the epilepsies, with examples it gives (Scheffer 2017), and how each is found (von Oertzen 2002; Smith 2022).
GroupWhat it meansExamplesHow it is found
StructuralAn abnormality on imaging that, with the clinical and electroencephalography (EEG) findings, is the likely cause; acquired, genetic or bothHippocampal sclerosis, malformations of cortical development, hypothalamic hamartoma, stroke, trauma, hypoxic–ischemic injuryMagnetic resonance imaging (MRI) with an epilepsy protocol: a subtle lesion needs one
GeneticThe epilepsy results directly from a known or presumed pathogenic variant, with seizures a core symptom. Genetic does not mean inherited: many variants arise de novoSCN1A in more than 80% of people with Dravet syndrome; a causative variant, most often de novo, in 30 to 50% of infants with severe developmental and epileptic encephalopathiesExome or genome sequencing or a multigene panel, with genetic counseling
InfectiousEpilepsy that results from an infection, not seizures during an acute infection. The commonest cause worldwideNeurocysticercosis, tuberculosis, human immunodeficiency virus, cerebral malaria, cerebral toxoplasmosis, congenital cytomegalovirus and Zika virusTests for the infection; it carries its own treatment
MetabolicA well-defined metabolic defect, most with a genetic basisPyridoxine-dependent epilepsy, glucose transporter 1 deficiency, porphyria, aminoacidopathies, cerebral folate deficiencyBiochemical and genetic tests; a specific treatment
ImmuneAutoimmune inflammation of the central nervous systemN-methyl-D-aspartate (NMDA) receptor encephalitis and leucine-rich glioma-inactivated 1 (LGI1) encephalitisAntibody testing; immunotherapy
UnknownNot found yet. How often a cause is found depends on how far the evaluation can goMany epilepsies, described by their clinical and EEG features alone, such as frontal lobe epilepsyRevisit the search as tests become available

Groups, definitions and examples from the classification (Scheffer 2017); glucose transporter 1 deficiency from its consensus statement (Klepper 2020).

The scan, and who reads it

The protocol and the reader both matter

  • In 123 consecutive candidates for epilepsy surgery, the sensitivity for a focal lesion was 39% for a standard MRI reported by a radiologist outside an epilepsy center, 50% when experts read the same scan, and 91% for an epilepsy-protocol MRI read by experts.
  • Outside reports of standard scans missed hippocampal sclerosis in 86% of cases.

Source: von Oertzen 2002.

A minimum epilepsy protocol

  • The ILAE Neuroimaging Task Force endorses structural MRI in new-onset epilepsy, generalized and focal alike, and sets a minimum protocol, HARNESS-MRI (harmonized neuroimaging of epilepsy structural sequences), built on three-dimensional acquisitions that most scanners can perform.
  • It also endorses computer-aided postprocessing to bring out subtle lesions.
  • A drug-resistant focal epilepsy with a "normal" standard MRI deserves an early epilepsy-protocol scan at a unit with epilepsy experience before it is called nonlesional.

Sources: Bernasconi 2019; von Oertzen 2002.

Who should have genetic testing

The National Society of Genetic Counselors guideline strongly recommends genetic testing for everyone with unexplained epilepsy, at any age: epilepsy not attributed to an acquired cause such as trauma or infection. First-tier testing is exome or genome sequencing, or a multigene panel of more than 25 genes, followed by chromosomal microarray, with exome or genome sequencing conditionally preferred over a panel. Tests should be chosen, ordered and interpreted by a qualified clinician with genetic counseling before and after (Smith 2022).

Counseling matters because a result changes more than the label. A de novo variant means the parents are unlikely to have epilepsy, but the patient may pass it on: with a dominant variant, each child has a 50% chance of inheriting it, though whether the child has epilepsy depends on its penetrance (Scheffer 2017).

Results that change treatment

Some results change the next prescription. The same drug class can harm one genetic epilepsy and treat another.

Six results that change treatment, from the Dravet syndrome consensus (Wirrell 2022), a series of KCNQ2 encephalopathy (Pisano 2015), consensus statements for glucose transporter 1 deficiency (Klepper 2020) and pyridoxine-dependent epilepsy (Coughlin 2021), the valproate label and the UK National Institute for Health and Care Excellence (NICE 6.3.6).
Gene and conditionWhat changesWhy
SCN1A loss of function (the Nav1.1 sodium channel): Dravet syndromeAvoid sodium-channel blockers for maintenance therapyThey worsen seizures: lamotrigine did so in 17 of 21 children (Guerrini 1998), and the consensus advises against them (Wirrell 2022; see Medicines for epilepsy syndromes)
KCNQ2 (a potassium channel subunit): neonatal-onset encephalopathyA sodium-channel blocker often worksOf 15 infants, 6 had seizure control within 2 weeks of carbamazepine and 5 became seizure-free with phenytoin; the authors propose sodium-channel blockers as first-line (Pisano 2015)
SLC2A1: glucose transporter 1 deficiencyKetogenic diet therapies, the standard treatmentA brain energy failure: ketone bodies supply the fuel that glucose cannot. Confirmed by a low cerebrospinal fluid glucose (hypoglycorrhachia) and an SLC2A1 variant; antiseizure medications alone leave the metabolic defect untreated (Klepper 2020)
ALDH7A1 (α-aminoadipic semialdehyde dehydrogenase): pyridoxine-dependent epilepsyLong-term pyridoxine, with lysine-reduction therapy addedSeizures respond to pyridoxine, but most patients still have developmental delay; a lysine-restricted diet and arginine improved biochemical markers and development in many, not all (Coughlin 2021). The first dose can cause respiratory arrest in a responder, so it is given with respiratory support (Stockler 2011)
POLG (mitochondrial DNA polymerase gamma): mitochondrial diseaseValproate is contraindicated, also in children under 2 clinically suspected of a mitochondrial disorderRisk of acute liver failure and death (boxed warning, valproate label)
TSC1 or TSC2: tuberous sclerosis complexVigabatrin alone first for infantile spasms; everolimus or cannabidiol for the focal seizures that followNICE recommends vigabatrin first for spasms due to tuberous sclerosis complex (NICE 6.3.6); everolimus and cannabidiol are labeled for its seizures (everolimus label; cannabidiol label)

Once a pyridoxine trial has started, it is continued until biochemical or genetic testing has excluded pyridoxine-dependent epilepsy, so that a late or masked response is not missed (Stockler 2011).

Immune causes

Two different questions

  • The ILAE Autoimmunity and Inflammation Task Force separates acute symptomatic seizures secondary to autoimmune encephalitis from autoimmune-associated epilepsy, which implies an enduring predisposition to seizures.
  • The distinction matters for mechanism, treatment, prognosis and social consequences: acute symptomatic seizures from an active encephalitis are not epilepsy.
  • Antibody testing has made these diagnoses more common; the examples in the classification are NMDA receptor and LGI1 encephalitis, both treated with immunotherapy.

Sources: Steriade 2020; Scheffer 2017.

Key points

  • Ask for the cause from the first seizure: six groups, chosen for their treatment consequences, and more than one can apply.
  • An epilepsy-protocol MRI read by an expert found focal lesions with 91% sensitivity, against 39% for a standard scan reported outside an epilepsy center.
  • Offer genetic testing, with counseling, to anyone with unexplained epilepsy; SCN1A, KCNQ2, SLC2A1, ALDH7A1, POLG and TSC1 or TSC2 results change treatment at once.

Quick check A 15-month-old with developmental regression and new refractory focal seizures is clinically suspected of having a mitochondrial disorder; genetic testing has been sent. Which medication is contraindicated?

Quick check A 2-year-old has seizures, abnormal eye–head movements and slowing head growth. The cerebrospinal fluid glucose is low with a normal blood glucose, and an SLC2A1 variant is found. What is the treatment of choice?

Quick check A 32-year-old has drug-resistant focal impaired consciousness seizures that begin with an epigastric aura. A standard MRI at another hospital was reported as normal. What is the next step in the search for a cause?

Sources: as cited. Etiology groups follow the 2017 ILAE classification of the epilepsies (Scheffer 2017).

Functional/dissociative seizures

Clinical reasoning

Functional/dissociative seizures look like epileptic seizures but arise without an epileptic discharge. The diagnosis rests on positive features, recorded on video-electroencephalography (video-EEG) when possible, and it leads to its own treatment. Missing it is not harmless: in the Established Status Epilepticus Treatment Trial (ESETT), 10% of the patients enrolled for benzodiazepine-refractory status epilepticus had functional/dissociative seizures (Kapur 2019).

The name

In 2025 an International League Against Epilepsy (ILAE) task force proposed functional/dissociative seizures for what were called psychogenic nonepileptic seizures, to replace a range of labels that confused professionals and patients. The slash lets the clinician use "functional", "dissociative" or both, depending on the patient and the language (Hingray 2025). Studies cited here used the older term.

How certain is the diagnosis?

Video-EEG is the standard, but it is not available everywhere or for every patient, so the ILAE Nonepileptic Seizures Task Force set out four levels of diagnostic certainty. Each needs a history consistent with the diagnosis; they differ in who saw an event and what the EEG showed (LaFrance 2013).

The four levels of diagnostic certainty of the ILAE Nonepileptic Seizures Task Force (LaFrance 2013).
LevelThe event was seen byThe EEG
PossibleA witness, or the patient's own accountNo epileptiform activity on a routine or sleep-deprived EEG between events
ProbableA clinician, in person or on a video recording, with features typical of functional/dissociative seizuresNo epileptiform activity on a routine or sleep-deprived EEG between events
Clinically establishedA clinician experienced in diagnosing seizure disorders, in person or on videoNo epileptiform activity on a routine or ambulatory EEG during a typical event, in an event whose semiology would make epileptiform activity expected if it were epileptic
DocumentedA clinician experienced in diagnosing seizure disorders, reviewing video-EEGA typical event captured on video-EEG, with no epileptiform activity immediately before, during or after it

The qualifier in the third level matters. Focal seizures with preserved consciousness often show nothing on the scalp EEG: only 18 of 87 (21%) did in one video-EEG series (Devinsky 1988). So a normal EEG during an event counts against epilepsy only when the event is one that would show an epileptic discharge (LaFrance 2013).

Signs at the bedside, weighed

A review of 34 video-EEG studies found good support for a set of signs; it also concluded that the diagnosis should rest on all the evidence, never on one sign alone (Avbersek 2010).

Bedside signs from video-EEG studies (Avbersek 2010; Chung 2006; Geyer 2000), the tongue-bite review (Brigo 2012) and the American Academy of Neurology prolactin assessment (Chen 2005).
SignFavorsEvidence
Eyes closed during the eventFunctional/dissociative seizure50 of 52 patients with functional seizures kept their eyes closed, while 152 of 156 with epileptic seizures opened them (Chung 2006); less reliable when pooled across studies (Brigo 2013)
Long duration and a fluctuating, waxing and waning course; asynchronous movements; side-to-side head or body movementsFunctional/dissociative seizureWell supported (Avbersek 2010)
Crying during the event; recall of the event; no postictal confusionFunctional/dissociative seizureWell supported (Avbersek 2010)
Onset from apparent sleep while the EEG shows wakefulnessFunctional/dissociative seizureWell supported (Avbersek 2010)
Pelvic thrustingNot decisiveSeen in 24% of patients with frontal lobe epilepsy and 17% with functional seizures, and in 2 to 4% with temporal lobe epilepsy (Geyer 2000)
Slow, deep, noisy (stertorous) breathing after the eventGeneralized tonic–clonic seizureDistinguished generalized tonic–clonic seizures from convulsive functional seizures (Avbersek 2010)
A bite on the side of the tongueEpileptic seizurePooled sensitivity 22%, specificity 100%; tongue biting not specified as lateral (sensitivity 38%, specificity 75%) did not help (Brigo 2012)
Serum prolactin at least twice baseline, drawn 10 to 20 minutes after the eventGeneralized tonic–clonic or focal impaired consciousness seizureA useful adjunct (Level B): pooled sensitivity 60% for generalized tonic–clonic and 46% for complex partial (focal impaired consciousness) seizures, specificity about 96%. It also rises after syncope, and it is not established for status epilepticus or repeated seizures (Chen 2005)

Giving the diagnosis, and treatment

Explain it

  • In a multicenter study, 10 neurologists gave the diagnosis to 50 patients using a written leaflet and a structured communication strategy: 94% found the leaflet easy to understand, only 4% felt angry during the consultation, and 86% acknowledged that psychological factors contributed to their seizures.
  • At 3 months, 14% were seizure-free and 63% reported more than a 50% reduction; the study had no control group.

Source: Hall-Patch 2010.

Cognitive behavioral therapy

  • CODES (cognitive behavioral therapy for adults with dissociative seizures), a randomized trial in 368 adults: the therapy added to standardized medical care did not significantly reduce monthly seizure frequency at 12 months (median 4 against 7; incidence rate ratio 0.78, 95% confidence interval 0.56 to 1.09).
  • It did make seizures less bothersome, lengthen seizure-free periods, and improve quality of life, psychosocial functioning, distress and somatic symptoms, with more improvement judged by patients and clinicians.
  • In an earlier pilot trial of 38 patients, cognitive behavioral therapy–informed psychotherapy reduced seizures by 51%, and by 59% with sertraline; sertraline alone did not significantly reduce them.

Sources: Goldstein 2020; LaFrance 2014.

Antiseizure medications

  • In 78 patients in whom coexisting epilepsy had been excluded by standardized criteria, antiseizure medications were withdrawn safely, as outpatients in most, with no serious adverse events; the functional seizures became less frequent overall.
  • 3 patients developed a new type of attack, focal impaired consciousness seizures, after withdrawal: follow-up is part of withdrawal.

Source: Oto 2005.

When epilepsy and functional seizures coexist

Reports of how many patients with functional seizures also have epilepsy have ranged from 10% to more than 50%. In one video-EEG unit, 3 of 32 patients diagnosed in a year (9.4%) had interictal epileptiform discharges supporting coexisting epilepsy (Benbadis 2001). This is why antiseizure medications are withdrawn only once coexisting epilepsy has been excluded, and why follow-up continues after withdrawal (Oto 2005).

Key points

  • Four levels of certainty: documented means a typical event on video-EEG with no epileptiform activity immediately before, during or after it.
  • Weigh signs together: closed eyes, a long fluctuating course, asynchronous movements, crying and recall favor a functional seizure; a bite on the side of the tongue and stertorous breathing favor an epileptic one; pelvic thrusting also occurs in frontal lobe epilepsy.
  • Explain the diagnosis, offer seizure-specific cognitive behavioral therapy, and withdraw antiseizure medications only once coexisting epilepsy is excluded, with follow-up.

Quick check A 28-year-old has had events for 2 years. Her partner describes her slumping with her eyes squeezed shut, turning her head from side to side, with asynchronous limb movements that wax and wane for 15 minutes; afterwards she cries and remembers the event. No clinician has seen one, and a sleep-deprived EEG is normal. What is the level of diagnostic certainty for functional/dissociative seizures?

Quick check A 40-year-old is brought in after a convulsive event that no clinician saw. Which finding most favors an epileptic generalized tonic–clonic seizure over a functional/dissociative seizure?

Quick check A 35-year-old with documented functional/dissociative seizures asks whether cognitive behavioral therapy will stop them. What did the largest randomized trial, CODES, find?

Sources: as cited. The term follows the 2025 ILAE task force proposal (Hingray 2025).

Seizures early in life: the neonate and the child with fever

Clinical

The two commonest settings for a seizure early in life ask opposite things. In a neonate, look harder: most seizures show no clinical sign, so the diagnosis rests on the electroencephalogram (EEG). After a brief generalized seizure with fever in a toddler, look less: the evaluation is of the fever, not of the seizure.

Seizures in the newborn

Seizures in the neonatal period are often provoked by an acute cause and may be electrographic only. The International League Against Epilepsy (ILAE) classification for neonates therefore rests the diagnosis on EEG: seizures with no clinical feature are included, and a clinical event without an EEG correlate is not classified as a seizure. Because neonatal seizures have a focal onset, there is no division into focal and generalized; a seizure is named by its predominant feature, motor (automatisms, clonic, epileptic spasms, myoclonic, tonic), nonmotor (autonomic, behavior arrest) or sequential (Pressler 2021).

Watching is not enough. In term infants recorded with video-EEG from the first hours of life, only 34% of 526 electrographic seizures had any clinical sign on the video, and only 27% of 177 events the nursery staff suspected were seizures on EEG; in all, only 9% of the electrographic seizures were recognized and documented (Murray 2008).

The neonatal classification (Pressler 2021), the ILAE treatment guideline (Pressler 2023) and the NEOLEV2 trial of phenobarbital against levetiracetam (Sharpe 2020).

Record with conventional video-EEG

  • The American Clinical Neurophysiology Society guideline calls conventional video-EEG the gold standard for detecting and quantifying neonatal seizures; single- and dual-channel amplitude-integrated EEG alone is not recommended for this when conventional EEG is available.
  • Use it to tell whether unexplained paroxysmal events are seizures, and to screen neonates at high risk, such as those with acute neonatal encephalopathy.
  • Screen for 24 hours; once seizures are found, continue until the neonate has been 24 hours seizure-free.

Source: Shellhaas 2011.

Look for the acute cause first

  • In 426 consecutive neonates monitored at 7 US centers, the commonest causes were hypoxic–ischemic encephalopathy (38%), arterial or venous ischemic stroke (18%) and intracranial hemorrhage (11%); a neonatal-onset epilepsy accounted for 13%.
  • 16% had only electrographic seizures, and a higher seizure burden was linked to death and to an abnormal examination at discharge.

Source: Glass 2016.

The ILAE treatment guideline makes six recommendations (Pressler 2023):

QuestionRecommendationBasis
First medicationPhenobarbital, whatever the cause, unless a channelopathy is likely (for example from the family history); then phenytoin or carbamazepineEvidence for phenobarbital; expert agreement for the rest
Seizures continuePhenytoin, levetiracetam, midazolam or lidocaine; levetiracetam may be preferred with heart diseaseExpert agreement
Electrographic-only seizuresTreat them too: a lower seizure burden may be associated with a better outcomeExpert agreement
Hypoxic–ischemic encephalopathyTherapeutic hypothermia may reduce seizure burdenEvidence
Suspected vitamin B6-dependent epilepsyA trial of pyridoxine when the features suggest it and seizures continue after second-line medicationExpert agreement
Going homeAfter acute provoked seizures have stopped, with no sign of a neonatal-onset epilepsy, stop the medication before discharge, even if the magnetic resonance imaging (MRI) or EEG is abnormalExpert agreement

Evidence

NEOLEV2: phenobarbital against levetiracetam as the first medication, neonatal seizures of any cause
Judged on EEG by two independent neurophysiologists, 80% (24 of 30) were free of seizures for 24 hours on phenobarbital, against 28% (15 of 53) on levetiracetam. Adverse effects were more frequent with phenobarbital, though not significantly (Sharpe 2020).

Febrile seizures

A febrile seizure is a seizure with fever, a temperature of at least 38 °C by any method, without infection of the central nervous system, in a child aged 6 to 60 months; it affects 2 to 5% of children. It is simple when it is generalized, lasts under 15 minutes and does not recur within 24 hours, and complex when it is prolonged, focal or recurs within 24 hours (American Academy of Pediatrics, 2008 and 2011).

The American Academy of Pediatrics guidelines on the simple febrile seizure (2008; 2011) and febrile status epilepticus in the FEBSTAT study (Lewis 2014; Lewis 2025).
After a simple febrile seizureGuideline
The cause of the feverFind it: that is where the evaluation is directed
Lumbar puncturePerform it with signs or symptoms of meningitis. It is an option at 6 to 12 months when immunization against Haemophilus influenzae type b or Streptococcus pneumoniae is incomplete or unknown, and in a child already given antibiotics
EEGNot in a neurologically healthy child
Blood testsNot routinely to find the cause of the seizure
NeuroimagingNot in the routine evaluation
Continuous or intermittent antiseizure medicationNot recommended after one or more simple febrile seizures
AntipyreticsMay make the child more comfortable, but will not prevent febrile seizures

Sources: American Academy of Pediatrics 2011 (evaluation); American Academy of Pediatrics 2008 (long-term management).

What parents ask next is whether it will happen again, and whether it is epilepsy. About 30% of children have a second febrile seizure when the first comes after 12 months of age, and about 50% when it comes before. After simple febrile seizures, the risk of epilepsy by age 7 is about the same as in the general population, 1%; children who have had several simple febrile seizures, were younger than 12 months at the first and have a family history of epilepsy are at higher risk, with afebrile generalized seizures by age 25 in 2.4% (American Academy of Pediatrics 2008).

Evidence

FEBSTAT (Consequences of Prolonged Febrile Seizures in Childhood): over 200 children with febrile status epilepticus, a febrile seizure of 30 minutes or longer, followed for up to 10 years
On magnetic resonance imaging within days, 22 of 226 (about 1 in 10) had hippocampal T2 hyperintensity, an acute injury; on follow-up imaging it had become hippocampal sclerosis in 10 of the 14 rescanned (Lewis 2014). By 10 years, 71% of children with the acute hyperintensity had epilepsy, against 23% of those with a normal first scan; mesial temporal lobe epilepsy developed in 39% of the first group and in none with a normal scan (Lewis 2025).

Key points

  • Neonatal seizures are diagnosed on EEG: most have no clinical sign, and many suspected events are not seizures.
  • Phenobarbital is the first medication for neonatal seizures unless a channelopathy is likely; stop treatment before discharge once acute provoked seizures are over.
  • After a simple febrile seizure, find the cause of the fever; EEG, blood tests, neuroimaging and preventive medication are not needed.

Quick check A term neonate with hypoxic–ischemic encephalopathy is on video-EEG during therapeutic hypothermia. The EEG shows repeated evolving rhythmic discharges over the left central region; the video shows no movement or other change. What is the next step?

Quick check An 18-month-old, fully immunized, has a 3-minute generalized convulsion with a temperature of 39 °C. An hour later she is alert, playful and neurologically normal, with a red, bulging tympanic membrane. What does she need?

Quick check A neonate's seizures from a left middle cerebral artery stroke stopped on day 2 with phenobarbital. Magnetic resonance imaging shows the infarct; there is no sign of a neonatal-onset epilepsy. The team is planning discharge. What should happen to the phenobarbital?

Sources: as cited. Neonatal seizure terms follow the ILAE neonatal classification (Pressler 2021); febrile seizure terms follow the American Academy of Pediatrics.

Choosing a first antiseizure medication

Clinical reasoning

The first drug is chosen in three steps: classify the seizures and the epilepsy, match the drug's spectrum to them, then fit it to the person who will take it.

The three steps, with the evidence from this lesson: the Standard and New Antiepileptic Drugs (SANAD) II trials (Marson 2021, focal; Marson 2021, generalized) and the carbamazepine label.

1. Classify the seizures and the epilepsy

Focal or generalized seizures, and if possible the epilepsy syndrome, using the classification of the International League Against Epilepsy (ILAE classification). The history and the electroencephalogram (EEG) do most of this work.

2. Match the spectrum

Some drugs treat both focal and generalized seizures; others treat focal seizures only, and ethosuximide treats absence seizures only. For a generalized epilepsy, the wrong spectrum can make things worse. The National Institute for Health and Care Excellence (NICE) lists carbamazepine, oxcarbazepine, phenytoin, gabapentin, pregabalin, tiagabine and vigabatrin as drugs that may exacerbate absence and myoclonic seizures, including in juvenile myoclonic epilepsy (and phenobarbital for absence seizures), advises against them for myoclonic seizures, and notes that lamotrigine can occasionally worsen myoclonic seizures (NICE NG217).

Case series show what happens when a generalized epilepsy is misclassified as focal:

Oxcarbazepine

  • Worsened or started myoclonic jerks and absences in young people with idiopathic generalized epilepsy.

Source: Gelisse 2004.

Carbamazepine

  • In 14 adults with absence or myoclonic status epilepticus precipitated by medication, every one had been given carbamazepine.
  • Withdrawing the aggravating drugs controlled the seizures, although the series included only patients who did well.

Source: Thomas 2006.

Lamotrigine in Dravet syndrome

  • Worsened seizures in 17 of 21 children.

Source: Guerrini 1998.

3. Fit the drug to the person

The possibility of pregnancy

  • Valproate carries the highest risk of major malformations, and of a lower intelligence quotient (IQ) and autism spectrum disorder, in exposed children: avoid it when clinically feasible in anyone who could become pregnant (see the women's health lessons).
  • In the UK, NICE guidance amended in January 2025 also says valproate should not be started for the first time in anyone, of either sex, under 55 unless two specialists independently agree that there is no other effective and tolerated treatment, or that the reproductive risks do not apply.
  • Topiramate is restricted in women and girls who can become pregnant.

Source: NICE NG217.

Mood and behavior

  • In the Standard and New Antiepileptic Drugs (SANAD) II trial, psychiatric adverse effects were reported by 30% of people starting levetiracetam against 13% starting lamotrigine.

Source: Marson 2021, focal.

Weight

  • Weight gain was reported by 10% on valproate against 3% on levetiracetam.

Source: Marson 2021, generalized.

Other medicines

Ancestry

  • Screen for the human leukocyte antigen (HLA) allele HLA-B*15:02 before carbamazepine in people with ancestry in populations where the allele occurs.

Source: carbamazepine label.

What the trials show

Evidence

SANAD II, focal epilepsy: lamotrigine, levetiracetam or zonisamide, 990 people aged 5 and over
Levetiracetam did not meet noninferiority to lamotrigine for 12-month remission; zonisamide did on the main analysis, but lamotrigine was better than both in the per-protocol analysis. Treatment was less likely to fail with lamotrigine than with either alternative, mainly because fewer people stopped it for adverse reactions; adverse reactions were reported by 33% on lamotrigine against 44% and 45%. The authors concluded that lamotrigine should remain a first-line treatment (Marson 2021, focal).
SANAD II, generalized or unclassifiable epilepsy: valproate or levetiracetam, 520 people aged 5 and over
Levetiracetam was not noninferior. Valproate brought 12-month remission sooner (median 445 against 636 days), was superior in the per-protocol analysis and failed less often for lack of seizure control. In a post hoc analysis the advantage was not seen in the absence epilepsies (Marson 2021, generalized).
Childhood absence epilepsy: ethosuximide, valproate or lamotrigine, 453 children
Free of treatment failure at 16 weeks: ethosuximide 53%, valproate 58%, lamotrigine 29%. Attentional dysfunction was more common with valproate (49%) than ethosuximide (33%), so ethosuximide is the preferred first drug (Glauser 2010).

NICE suggests considering lamotrigine or levetiracetam first for focal seizures (a weaker "consider" recommendation); it offers a choice of lamotrigine, levetiracetam or valproate for generalized tonic–clonic seizures, within the valproate rules above; ethosuximide first for absence seizures; and levetiracetam or valproate for myoclonic seizures (NICE NG217).

First-line choices and the drugs that may exacerbate each seizure type, from the National Institute for Health and Care Excellence guideline, chapter 5 (NICE NG217).

What to expect

In a Glasgow cohort of 1,795 people followed for up to 30 years after starting treatment, 50.5% became seizure-free for at least a year on their first regimen. The second regimen added 11.6% and the third 4.4%; all later regimens together added about 2%. At the end, 63.7% were seizure-free, so more than a third were not, and outcomes had not improved over three decades of new drugs (Chen 2018). Why the first drug failed matters: in the original report from the same clinic, 41% of people whose first drug failed because of adverse effects later became seizure-free, against 11% when it failed for lack of efficacy (Kwan 2000).

Key points

  • Classify first: the wrong spectrum can make seizures worse.
  • For focal epilepsy, lamotrigine was the best first choice in SANAD II; for generalized epilepsy valproate was the most effective, but its use is limited by its risks in pregnancy.
  • About half of people become seizure-free on their first medication; each later drug adds less.

Quick check A 17-year-old woman has morning myoclonic jerks and one generalized tonic–clonic seizure; the EEG shows generalized polyspike-and-wave. Which medication is most likely to aggravate her seizures?

Quick check A 45-year-old woman taking apixaban after a pulmonary embolism has had two focal impaired consciousness seizures. Which first antiseizure medication is least suitable for her?

Quick check Two adults with newly diagnosed focal epilepsy stop their first medication: one because of intolerable dizziness, the other because seizures continued at a full, tolerated dose. Which statement fits the Glasgow cohort?

Sources: as cited. NICE NG217 is quoted as updated in August 2026; US labeling does not carry the UK valproate age rule.

How antiseizure medications work

Concept

Where a drug acts explains much of its clinical profile: which seizures it treats, which it can make worse, and which adverse effects to expect.

Seizures start when excitation outruns inhibition in a network. Antiseizure medications shift that balance back in one of four broad ways:

1. Make neurons harder to fire

  • Sodium-channel blockers stabilize the inactivated channel so that rapid, repetitive firing fails.
  • Most act on fast inactivation; lacosamide, and in laboratory studies eslicarbazepine, enhance slow inactivation.

Source: Hebeisen 2015.

2. Act on transmitter release

  • Levetiracetam and brivaracetam bind the synaptic vesicle protein SV2A, which is involved in transmitter release; how this stops seizures is not fully known.
  • Gabapentin and pregabalin bind the α2δ subunit of calcium channels.

3. Strengthen inhibition

  • Benzodiazepines and barbiturates enhance the gamma-aminobutyric acid type A (GABA-A) receptor.
  • Vigabatrin and tiagabine raise gamma-aminobutyric acid (GABA) by blocking its breakdown or its reuptake.

4. Weaken excitation

  • Perampanel blocks α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA) glutamate receptors.
  • Felbamate also blocks N-methyl-D-aspartate (NMDA) glutamate receptors.

A fifth target explains a whole seizure type. Thalamic relay neurons fire in bursts through T-type calcium channels, and that burst firing drives the thalamocortical loop behind generalized spike-and-wave. Ethosuximide reduces the T-type current, which is why its use is limited to absence seizures; its label warns that, used alone in mixed epilepsies, it can increase tonic–clonic seizures.

Schematic. Ethosuximide reduced the T-type current in thalamic neurons, and valproate did not (Coulter 1989); childhood absence epilepsy outcomes from Glauser 2010.

Choose a target or a drug in the model to see where it acts.

Antiseizure medications by mechanism Carbamazepine, oxcarbazepine and phenytoin can aggravate absence and myoclonic seizures; sodium-channel blockers, lamotrigine included, can worsen Dravet syndrome.
  • Sodium channel

    • phenytoin
    • carbamazepine
    • oxcarbazepine
    • lamotrigine
    • rufinamide
    • zonisamide
    • topiramate
  • Slow sodium inactivation

    • lacosamide (distinct from the fast-inactivation drugs)
    • eslicarbazepine (in laboratory studies)
  • Calcium channel

    • ethosuximide (T‑type)
    • gabapentin
    • pregabalin (both bind the α2δ subunit)
  • GABA enhancement

    • benzodiazepines, including clobazam
    • phenobarbital
    • primidone
    • tiagabine (blocks GABA reuptake)
    • vigabatrin (inhibits GABA transaminase)
  • SV2A binding

    • levetiracetam
    • brivaracetam (higher SV2A affinity than levetiracetam)
  • AMPA antagonist

    • perampanel (the only selective, noncompetitive AMPA antagonist)
  • Multiple or other

    • valproate
    • topiramate
    • felbamate (also NMDA)
    • cenobamate (sodium channel and GABA‑A)
    • cannabidiol
  • Disease-specific

    • everolimus (mechanistic target of rapamycin [mTOR] inhibitor; tuberous sclerosis complex)
    • ganaxolone (GABA‑A modulator; cyclin-dependent kinase-like 5 [CDKL5] deficiency disorder)
    • fenfluramine (serotonin; Dravet and Lennox–Gastaut syndromes)

Several drugs act in more than one way and appear under each. Board Review has an interactive map of where each drug acts.

Key points

  • Most drugs work on one of four jobs: neuronal firing, transmitter release, inhibition or excitation.
  • Ethosuximide's T-type calcium target matches absence seizures; it does not protect against tonic–clonic seizures.
  • Carbamazepine, oxcarbazepine and phenytoin can aggravate absence and myoclonic seizures; sodium-channel blockers, lamotrigine included, can worsen Dravet syndrome.

Quick check A 7-year-old has many brief episodes of behavioral arrest a day, and the EEG shows 3-Hz generalized spike-and-wave. Which target explains why ethosuximide works for her seizures?

Quick check A man whose focal seizures are controlled on levetiracetam asks whether any other antiseizure medication acts at the same target. Which drug binds the synaptic vesicle protein SV2A?

Quick check A 6-month-old with infantile epileptic spasms syndrome starts vigabatrin, and the parents are told that vision must be assessed at baseline and at least every 3 months. How does vigabatrin raise brain GABA?

Medication profiles

Reference

Every antiseizure medication used in practice, with its mechanism and the adverse effects that shape the choice. Read it as a set of profiles: spectrum, target and the safety problems to know before prescribing.

Some drugs are broad spectrum (they treat focal and generalized seizures), some treat focal seizures only, and a few are reserved for one syndrome.

Suicidal thoughts and behavior: an FDA class warning

The United States Food and Drug Administration (FDA) labels antiseizure medications as a class for an increased risk of suicidal thoughts or behavior, whatever the indication. In the pooled analysis behind the warning, 199 placebo-controlled trials of 11 drugs, the relative risk was 1.8 (95% confidence interval 1.2 to 2.7): 0.43% of 27,863 patients on a drug against 0.24% of 16,029 on placebo, about one more patient for every 530 treated. Watch for new or worse depression, suicidal thoughts or behavior, or unusual changes in mood or behavior, and tell patients, caregivers and families to report them (lamotrigine FDA label, section 5.6).

Antiseizure medications grouped by the seizures they treat, from their US labels and NICE NG217.
Common adverse effects by mechanism, from the US prescribing information.

Find a medication

Search by generic or brand name, abbreviation, seizure type, mechanism or adverse effect. Several words find drugs that match all of them.

Filter by spectrum or mechanism
Filters
Mechanism
Medication Mechanism / Target Key adverse effects and safety
Acetazolamide Carbonic anhydrase inhibitor Paresthesias, fatigue, dysgeusia, metabolic acidosis, nephrolithiasis
Brivaracetam Binds synaptic vesicle protein 2A (SV2A) with high and selective affinity, about 20 times that of levetiracetam in binding studies; how this stops seizures is not known Drowsiness, dizziness, fatigue, irritability; Stevens–Johnson syndrome and toxic epidermal necrolysis (a 2025 warning): stop at the first rash unless it is clearly not drug related
Carbamazepine Not established by the label; in the literature, blocks sodium channels in their fast-inactivated state Dizziness, drowsiness, diplopia, ataxia, hyponatremia, leukopenia; aplastic anemia and agranulocytosis (boxed); serious rash including Stevens–Johnson syndrome and toxic epidermal necrolysis (human leukocyte antigen HLA-B*15:02 screening recommended before initiation for patients with ancestry in populations where HLA-B*15:02 is common; consider HLA-A*31:01 risk according to ancestry and local guidance)
Cannabidiol (Dravet syndrome, Lennox–Gastaut syndrome or tuberous sclerosis complex) Not established; the label says it does not act through cannabinoid receptors. Proposed in laboratory studies: antagonism at G protein-coupled receptor 55 (GPR55), desensitization of transient receptor potential vanilloid 1 (TRPV1), inhibition of adenosine reuptake Diarrhea, fatigue, decreased appetite, somnolence; raised transaminases, especially with valproate (check liver tests before and during treatment)
Cenobamate Dual: inhibits the persistent sodium current and positively modulates the gamma-aminobutyric acid type A (GABA-A) receptor at a non-benzodiazepine site Dizziness, somnolence, fatigue, headache, nausea; drug reaction with eosinophilia and systemic symptoms when titrated fast; clinically significant liver injury (a 2025 warning; liver tests before starting); shortens the QT interval (contraindicated in familial short QT syndrome)
Clobazam (adjunct for Lennox–Gastaut syndrome) 1,5-benzodiazepine — positive allosteric GABA-A modulator (the other benzodiazepines used for seizures are 1,4-benzodiazepines) Sedation, drowsiness, drooling; the benzodiazepine boxed warning (with opioids; abuse, misuse and addiction; dependence and withdrawal)
Clorazepate Benzodiazepine prodrug → nordiazepam; positive allosteric GABA-A modulator Drowsiness, dizziness, fatigue, ataxia; the benzodiazepine boxed warning
Clonazepam (rarely used long-term) Benzodiazepine — positive allosteric GABA-A modulator Sedation, drowsiness, cognitive impairment, tolerance; the benzodiazepine boxed warning
Diazepam Benzodiazepine — positive allosteric GABA-A modulator Sedation, fatigue, ataxia, muscle weakness; venous thrombosis and phlebitis at the intravenous injection site; somnolence after the rectal gel; the benzodiazepine boxed warning
Eslicarbazepine acetate Prodrug → eslicarbazepine; sodium channel blockade (the label); in laboratory studies it enhances slow inactivation, like lacosamide Dizziness, headache, diplopia, somnolence, hyponatremia
Ethosuximide T-type calcium channel blockade in thalamocortical relay neurons Nausea, vomiting, lethargy, headache, weight loss
Everolimus (tuberous sclerosis complex) Mechanistic target of rapamycin (mTOR) inhibitor — targets the pathway disrupted in tuberous sclerosis Stomatitis, infections, rash, fatigue
Felbamate (rarely used because of its toxicity) Not established; the label describes antagonism at the glycine recognition site of the N-methyl-D-aspartate (NMDA) receptor, with weak effects on GABA-receptor binding Sedation, nausea, vomiting; aplastic anemia and hepatic failure (boxed)
Fenfluramine Not established; agonist at serotonin (5-hydroxytryptamine, 5-HT) 5-HT2 receptors (the label), with sigma-1 receptor modulation in laboratory studies Decreased appetite, somnolence, diarrhea, fatigue; boxed warning requires echocardiographic monitoring for valvular heart disease and pulmonary arterial hypertension
Fosphenytoin (parenteral only) Water-soluble prodrug of phenytoin; voltage-dependent sodium channel blockade. Ordered in phenytoin sodium equivalents (PE) rather than milligrams of fosphenytoin Pruritus, nystagmus, dizziness, somnolence, ataxia; hypotension and cardiac arrhythmias with rapid infusion (cardiac monitoring during and after the infusion)
Gabapentin (adjunctive therapy for focal seizures) Binds the alpha-2-delta subunit of voltage-gated calcium channels. Despite the name, not a GABA agonist Dizziness, somnolence, peripheral edema, ataxia
Ganaxolone (cyclin-dependent kinase-like 5 [CDKL5] deficiency disorder) Neuroactive steroid — positive allosteric modulator of synaptic and extrasynaptic GABA-A receptors Somnolence, pyrexia, salivary hypersecretion
Lacosamide (focal seizures; add-on for primary generalized tonic–clonic seizures) Enhances slow inactivation of sodium channels — a different action from the classic fast-inactivation blockers Dizziness, headache, nausea, diplopia; PR prolongation and atrioventricular block (electrocardiogram when there is cardiac disease or other PR-prolonging drugs)
Lamotrigine Proposed in the label: sodium channel blockade, which in turn reduces the release of glutamate Rash, including Stevens–Johnson syndrome (boxed; more likely with fast titration, valproate or HLA-B*15:02), dizziness, diplopia, headache; class IB antiarrhythmic activity, with a wider QRS complex in structural or functional heart disease; hemophagocytic lymphohistiocytosis
Levetiracetam Binds synaptic vesicle protein 2A (SV2A), thought to be involved in vesicle exocytosis; how this stops seizures is not known Drowsiness, dizziness, irritability, behavioral changes
Lorazepam Benzodiazepine — positive allosteric GABA-A modulator; no active metabolite Sedation, dizziness, weakness, ataxia; with the injection, central nervous system and respiratory depression; the benzodiazepine boxed warning
Methsuximide (absence seizures refractory to other drugs) Succinimide — T-type calcium channel blockade Nausea, vomiting, dizziness, drowsiness
Midazolam Benzodiazepine — positive allosteric GABA-A modulator; short acting Somnolence, headache, nasal discomfort, throat irritation, rhinorrhea; with the injection, respiratory depression and apnea; the benzodiazepine boxed warning
Oxcarbazepine Prodrug → licarbazepine, the monohydroxy derivative; sodium channel blockade Dizziness, sedation, hyponatremia
Perampanel (focal seizures from age 4; primary generalized tonic–clonic seizures from age 12) Selective non-competitive antagonist of the α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA) glutamate receptor Dizziness, somnolence, hostility, irritability, falls; serious psychiatric and behavioral reactions (boxed)
Phenobarbital Barbiturate — prolongs GABA-A chloride channel opening; inhibits glutamate responses at higher concentrations Sedation, cognitive impairment, ataxia, dependence
Phenytoin Use- and voltage-dependent sodium channel blockade (fast inactivation) Nystagmus, ataxia, gingival hyperplasia, hypertrichosis, sedation; intravenous: hypotension and arrhythmias with rapid infusion (boxed)
Primidone (rarely used) Active itself, and metabolized to phenobarbital and phenylethylmalonamide, both active; GABA-A modulation Sedation, ataxia, nausea, dizziness
Pregabalin (adjunctive therapy for focal seizures) Binds the alpha-2-delta subunit of voltage-gated calcium channels Dizziness, somnolence, weight gain, peripheral edema
Rufinamide (labeled for Lennox–Gastaut syndrome only) Prolongs the inactivated state of sodium channels Dizziness, fatigue, nausea, vomiting
Stiripentol (labeled for Dravet syndrome, with clobazam) Positive allosteric GABA-A modulator; also inhibits cytochrome P450 enzymes, raising clobazam and norclobazam levels Drowsiness, ataxia, decreased appetite
Tiagabine (rarely used) Blocks GAT-1, the transporter that takes GABA back up into neurons and glia Dizziness, nervousness, fatigue, tremor
Topiramate Multiple: sodium channels, GABA-A potentiation, AMPA and kainate glutamate-receptor antagonism, carbonic anhydrase inhibition Cognitive slowing and word-finding difficulty, weight loss, paresthesias, nephrolithiasis, metabolic acidosis, oligohidrosis; acute myopia with secondary angle-closure glaucoma; visual field defects
Valproate / Divalproex Sodium Not established; the label cites increased brain GABA. Proposed in the literature: sodium channels and histone deacetylase inhibition; no effect on the thalamic T-type calcium current Nausea, vomiting, dyspepsia, tremor, weight gain, alopecia; hepatotoxicity, pancreatitis and fetal harm (boxed); thrombocytopenia, hyperammonemia; the most teratogenic antiseizure medication (see Pregnancy)
Vigabatrin (restricted use; Risk Evaluation and Mitigation Strategy) Irreversible GABA-transaminase inhibitor, raising brain GABA Permanent visual field loss (boxed warning; vision monitoring under the Risk Evaluation and Mitigation Strategy), sedation, irritability
Zonisamide Sodium channels and T-type calcium channels, with weak carbonic anhydrase inhibition Dizziness, somnolence, decreased appetite, weight loss; metabolic acidosis, nephrolithiasis, oligohidrosis; acute myopia with angle-closure glaucoma; a sulfonamide, contraindicated in sulfonamide hypersensitivity

Adverse effects and boxed warnings from the US prescribing information, linked from each drug's name. Every antiseizure medication also carries a class warning for suicidal thoughts and behavior. Mechanisms from the labels where they state one; otherwise from the literature (Sills 2020; valproate and the thalamic calcium current, Coulter 1989; eslicarbazepine and slow inactivation, Hebeisen 2015; brivaracetam binding, Gillard 2011; cannabidiol, Gray 2020; fenfluramine and sigma-1, Martin 2020). Boxed warnings and monitoring are gathered in the next lesson, and drug levels in Drug levels and interactions.

Quick check Before starting carbamazepine in a man whose family comes from Southeast Asia, which test does the US label recommend?

Quick check A 34-year-old starting lamotrigine for focal epilepsy has read that it carries an FDA warning about suicidal thoughts and behavior. Which statement about the warning is accurate?

Quick check A 70-year-old taking primidone for focal epilepsy has a serum phenobarbital level reported, although phenobarbital was never prescribed. What is the explanation?

Boxed warnings, monitoring and organ effects

Concept

A boxed warning is the strongest warning in US prescribing information, and each one carries an action: a test before the first dose, monitoring during treatment, a limit on titration or infusion rate, or a restricted program. After the boxed warnings come the organ effects that examinations return to, grouped by system.

Nine boxed warnings sorted by the action each asks for. From the US prescribing information linked in the table below.

The boxed warnings

Drug Boxed warning What to do
Carbamazepine Stevens–Johnson syndrome and toxic epidermal necrolysis, strongly associated with the human leukocyte antigen (HLA) allele HLA-B*15:02; aplastic anemia and agranulocytosis In patients with ancestry in populations where the allele occurs, genotype before starting, and do not start in a carrier unless the benefit clearly outweighs the risk; obtain a baseline complete blood count
Lamotrigine Serious rash, including Stevens–Johnson syndrome: 0.3 to 0.8% of children aged 2 to 17 and 0.08 to 0.3% of adults. Risk rises with valproate, with exceeding the starting dose or the escalation, and with HLA-B*15:02 Follow the label's titration exactly, using its slower schedule when valproate is taken; stop at the first sign of rash unless it is clearly not drug related
Valproate Hepatotoxicity, highest in children under 2 and in mitochondrial disease due to variants in POLG (mitochondrial DNA polymerase gamma); fetal risk: neural tube defects, other major malformations and a lower intelligence quotient (IQ); pancreatitis Liver tests before starting and at frequent intervals, especially in the first 6 months; contraindicated in hepatic disease and POLG disease; not in pregnancy, planned pregnancy or a woman who could become pregnant unless other drugs have failed or are unacceptable, and then with effective contraception; evaluate abdominal pain, nausea, vomiting or anorexia promptly for pancreatitis
Felbamate Aplastic anemia; hepatic failure Only for patients who respond inadequately to alternatives, with their written acknowledgment of the risks; not with a history of hepatic dysfunction; serum transaminases at baseline and periodically, stopping if either reaches 2 times the upper limit of normal; a hematologist at once for any hematologic abnormality
Vigabatrin Permanent bilateral concentric visual field constriction (in adult studies, 30% or more of patients, per the label's warnings section) Assess vision at baseline (no later than 4 weeks after starting), at least every 3 months, and 3 to 6 months after stopping; withdraw if there is no substantial benefit within 2 to 4 weeks in infantile spasms or 3 months in refractory focal seizures; prescribed only through a Risk Evaluation and Mitigation Strategy (REMS)
Perampanel Serious psychiatric and behavioral reactions: aggression, hostility, irritability, anger, homicidal ideation and threats, with or without a psychiatric history Monitor closely during titration and at higher doses; reduce the dose if they appear, and stop at once if they are severe or worsening
Fenfluramine Valvular heart disease and pulmonary arterial hypertension Echocardiogram before, during and after treatment; prescribed only through a REMS
Intravenous phenytoin and fosphenytoin Hypotension and arrhythmias when infused rapidly Keep within the labels' maximum infusion rates: in adults, fosphenytoin's, counted in phenytoin sodium equivalents, is three times phenytoin's, and children have a weight-based limit or the adult limit, whichever is slower; monitor the heart during and after the infusion
Clobazam and the other benzodiazepines, such as clonazepam With opioids, profound sedation, respiratory depression, coma and death; abuse, misuse and addiction; dependence and withdrawal reactions Combine with opioids only when alternatives are inadequate, at the lowest doses for the shortest time; assess the risk of abuse before and during treatment; taper gradually to stop or to reduce the dose

Oxcarbazepine, eslicarbazepine, topiramate, zonisamide, lacosamide, levetiracetam, cenobamate, rufinamide, ethosuximide, cannabidiol, stiripentol and ganaxolone carry no boxed warning. The warning on suicidal thoughts and behavior covers every antiseizure medication, but it sits in Warnings and Precautions, not in a box (see Medication profiles).

Organ effects by system

The organ effects below, on one figure. From the US prescribing information linked in each card.

Sodium

  • Carbamazepine: dose-related hyponatremia, in many cases as the syndrome of inappropriate antidiuretic hormone secretion.
  • Oxcarbazepine: sodium below 125 mmol/L in 2.5% of trial patients, usually in the first 3 months, sometimes after more than a year.
  • Eslicarbazepine: below 125 mmol/L in 1.0% and 1.5% at the two doses reported, none on placebo.
  • Consider measuring sodium during maintenance treatment, above all with other drugs that lower it, or with nausea, malaise, headache, lethargy, confusion or more frequent seizures.

Sources: carbamazepine label; oxcarbazepine label; eslicarbazepine label.

Acidosis, nephrolithiasis and hyperthermia: topiramate and zonisamide

  • Both inhibit carbonic anhydrase: the kidney loses bicarbonate, causing a hyperchloremic, non-anion-gap metabolic acidosis. Measure bicarbonate at baseline and periodically.
  • Nephrolithiasis: 1.5% of adults on topiramate in adjunctive trials, 4% of adults on zonisamide.
  • Oligohidrosis and hyperthermia, mostly in children: watch for decreased sweating and a rising temperature in hot weather.
  • Zonisamide is a sulfonamide: contraindicated in sulfonamide hypersensitivity; fatal reactions have occurred.

Sources: topiramate label; zonisamide label.

The eye

  • Topiramate and zonisamide: acute myopia with secondary angle-closure glaucoma, typically within 1 month of starting. Treatment: stop the drug as rapidly as possible.
  • Topiramate can also cause visual field defects without raised intraocular pressure, mostly reversible on stopping.
  • Vigabatrin: permanent visual field loss (the boxed warning above).

Sources: topiramate label (sections 5.1 and 5.2); zonisamide label.

Topiramate and zonisamide inhibit carbonic anhydrase: the kidney loses bicarbonate, nephrolithiasis can follow, sweating falls, and either drug can close the drainage angle of the eye (topiramate and zonisamide labels).

Weight

  • Gain with valproate: 10% against 3% on levetiracetam in the Standard and New Antiepileptic Drugs (SANAD) II trial.
  • Gain with pregabalin: 9% gained 7% or more of baseline weight, against 2% on placebo, with dose and duration.
  • Loss with topiramate: 9% against 3% on placebo in adult adjunctive trials.
  • Loss with fenfluramine in the Dravet syndrome trials: decreased appetite in 37% against 8%, decreased weight in 9% against 1%.

Sources: Marson 2021, generalized; pregabalin label; topiramate label; fenfluramine label.

Phenytoin over the years

  • Gingival hyperplasia, coarsening of the facial features and hypertrichosis; hyperglycemia.
  • Rarely, irreversible cerebellar dysfunction or cerebellar atrophy with high levels.
  • Bradycardia and cardiac arrest, both at recommended doses and levels and with toxicity, mostly in people with heart disease.
  • Intravenous phenytoin and fosphenytoin: purple glove syndrome at the infusion site.

Sources: phenytoin label; phenytoin injection label; fosphenytoin label.

Bone

  • Chronic phenytoin: decreased bone mineral density and osteomalacia.
  • Topiramate monotherapy in children: lower bone mineral density in a 1-year controlled study of 63, linked to the fall in bicarbonate; too small to show whether fracture risk rises.

Sources: phenytoin label; topiramate label.

Mood and behavior

  • Perampanel: the boxed warning above.
  • Levetiracetam: nonpsychotic behavioral symptoms in 13% of adults against 6% on placebo; psychotic symptoms in 1% against 0.2%.
  • In SANAD II, psychiatric adverse effects in 30% starting levetiracetam against 13% starting lamotrigine.
  • Topiramate: word-finding difficulty.

Sources: levetiracetam label; Marson 2021, focal; topiramate label.

Schematic intervals, not to scale (lacosamide, lamotrigine, cenobamate and rufinamide labels).

Cardiac conduction

  • Lacosamide: prolongs the PR interval, dose-dependently; arrhythmias reported after marketing, mostly with proarrhythmic conditions or conduction-slowing drugs. Obtain an electrocardiogram before starting and after titration with conduction disease, severe heart disease, a sodium channelopathy such as Brugada syndrome, or a PR-prolonging drug.
  • Lamotrigine: class IB antiarrhythmic activity; can widen the QRS complex and cause proarrhythmia in clinically important structural or functional heart disease.
  • Cenobamate and rufinamide: shorten the QT interval; contraindicated in familial short QT syndrome.

Sources: lacosamide label; lamotrigine label; cenobamate label; rufinamide label.

Ammonia and temperature

  • Valproate can raise ammonia, with or without encephalopathy, even when liver tests are normal. With unexplained lethargy, vomiting or a change in mental status, measure an ammonia level.
  • Valproate is contraindicated in urea cycle disorders.
  • Topiramate added to valproate raises the risk of hyperammonemia and can cause hypothermia.

Sources: valproate label; topiramate label.

Blood

  • Carbamazepine and felbamate: aplastic anemia (boxed).
  • Valproate: dose-related thrombocytopenia.
  • Ethosuximide: blood dyscrasias, some fatal; periodic blood counts.
  • Lamotrigine: hemophagocytic lymphohistiocytosis.

Sources: valproate label; ethosuximide label; lamotrigine label.

Multiorgan hypersensitivity

  • Drug reaction with eosinophilia and systemic symptoms (DRESS) is a warning in most antiseizure medication labels: carbamazepine, oxcarbazepine, eslicarbazepine, phenytoin and fosphenytoin, lamotrigine, valproate, levetiracetam, lacosamide, topiramate, zonisamide, rufinamide, clobazam, perampanel, gabapentin, ethosuximide and cenobamate among them.
  • Cenobamate: it occurred, once fatally, when the dose was raised weekly or faster, so it is started low and raised every 2 weeks, never faster.
  • Oxcarbazepine: 25 to 30% of patients with hypersensitivity to carbamazepine react to it.

Sources: cenobamate label; oxcarbazepine label.

Liver

  • Valproate and felbamate: the boxed warnings above.
  • Cannabidiol raises transaminases, mostly with valproate: above 3 times the upper limit of normal in 30% of patients taking valproate and clobazam and 21% taking valproate without clobazam, in the Lennox–Gastaut and Dravet syndrome trials.
  • Check alanine and aspartate aminotransferases and total bilirubin before starting and at 1, 3 and 6 months. Stop cannabidiol for transaminases above 3 times the upper limit of normal with bilirubin above 2 times, or above 5 times if they stay high.
  • Cenobamate (warning added 2025): clinically significant liver injury after marketing, including acute liver failure needing transplantation. Check the transaminases and bilirubin before starting, and interrupt or stop it for liver injury without another cause.

Sources: cannabidiol label; cenobamate label (section 5.4).

Key points

  • Each boxed warning has its action: genotype before carbamazepine; follow lamotrigine's titration, slower with valproate; liver tests and pregnancy avoidance with valproate; vision checks with vigabatrin; echocardiograms with fenfluramine; rate limits for intravenous phenytoin and fosphenytoin.
  • Hyponatremia: carbamazepine, oxcarbazepine and eslicarbazepine. Acidosis, nephrolithiasis and oligohidrosis: topiramate and zonisamide. Angle-closure glaucoma: topiramate and zonisamide.
  • Valproate raises ammonia even with normal liver tests, more so with topiramate; cannabidiol raises transaminases, mostly with valproate; cenobamate can injure the liver.
  • Conduction: lacosamide prolongs the PR interval, lamotrigine can widen the QRS complex in heart disease, and cenobamate and rufinamide shorten the QT interval.

Quick check A 28-year-old woman started topiramate 3 weeks ago. She now has sudden blurred vision and pain in both eyes. What is the first step?

Quick check A 76-year-old woman taking hydrochlorothiazide starts oxcarbazepine for focal seizures. Six weeks later she is confused and having more seizures, and her sodium is 121 mmol/L. What is the most likely cause?

Quick check A 19-year-old man whose generalized epilepsy is controlled on valproate adds topiramate for migraine. Ten days later he is lethargic and vomiting, and his liver tests are normal. Which test comes first?

Quick check A 10-year-old with Lennox–Gastaut syndrome taking valproate and clobazam is to start cannabidiol. Which monitoring does the label call for?

Quick check A 35-year-old with drug-resistant focal epilepsy starts cenobamate at a low dose, to be raised only every 2 weeks. Why is the titration so slow?

Sources: as cited; boxed warnings and warnings from the current US prescribing information on DailyMed, checked October 2026.

Medicines for epilepsy syndromes

Concept

Once an epilepsy syndrome is recognized, the syndrome rather than the seizure type chooses the medicine. Several drugs are approved by the US Food and Drug Administration (FDA) for one syndrome only, and in some syndromes a drug chosen by seizure type alone makes the seizures worse.

Syndrome Choose Avoid or watch Source
Infantile epileptic spasms syndrome Hormonal therapy (adrenocorticotropic hormone [ACTH] or prednisolone) or vigabatrin, without delay; the National Institute for Health and Care Excellence (NICE) offers prednisolone with vigabatrin first unless steroid risk is high Delay. On vigabatrin, vision assessment and withdrawal if there is no benefit within 2 to 4 weeks; on ACTH, infection, hypertension and adrenal insufficiency when it is stopped Go 2012; NICE 6.3; ACTH label; vigabatrin label
Tuberous sclerosis complex For spasms, vigabatrin alone, adding prednisolone if it has not worked after 1 week; for later seizures, everolimus from age 2 and cannabidiol from age 1 Everolimus dosing is guided by trough levels; vigabatrin's vision checks NICE 6.3.6; everolimus label; cannabidiol label
Dravet syndrome Valproate first; clobazam, stiripentol (with clobazam) or fenfluramine; cannabidiol Sodium-channel blockers for maintenance: lamotrigine (contraindicated in children by the consensus) and carbamazepine worsen seizures. Phenytoin is not contraindicated for status epilepticus Wirrell 2022; Guerrini 1998; Shi 2016
Lennox–Gastaut syndrome Valproate first, then lamotrigine, then rufinamide; later clobazam, cannabidiol, fenfluramine, felbamate or topiramate NICE: carbamazepine, gabapentin, lacosamide, lamotrigine, oxcarbazepine, phenobarbital, pregabalin, tiagabine and vigabatrin may worsen seizures; the expert algorithm also urges caution with eslicarbazepine and phenytoin. Start rufinamide lower with valproate Auvin 2025; NICE 6.2.8; rufinamide label
Cyclin-dependent kinase-like 5 (CDKL5) deficiency disorder Ganaxolone, from age 2 Somnolence and sedation; a Schedule V controlled substance ganaxolone label
Childhood absence epilepsy Ethosuximide first; if it fails, lamotrigine, levetiracetam or valproate. With other seizure types, start with one of these three Carbamazepine, oxcarbazepine, phenytoin, phenobarbital, gabapentin, pregabalin, tiagabine and vigabatrin may worsen absence seizures; valproate under the UK restrictions Glauser 2010; NICE 5.3
Juvenile myoclonic epilepsy Levetiracetam or valproate; in women and girls who could become pregnant, valproate only if other treatments fail Do not use carbamazepine, oxcarbazepine, phenytoin, gabapentin, pregabalin, tiagabine or vigabatrin; lamotrigine can occasionally worsen myoclonic seizures NICE 5.4; levetiracetam label

Infantile epileptic spasms syndrome

Time to treatment, the combined-therapy trial and the tuberous sclerosis exception (Go 2012; O'Callaghan 2017; NICE NG217 6.3).

Refer and treat without delay

  • NICE: referral to a tertiary pediatric neurologist within 24 hours.
  • American Academy of Neurology and Child Neurology Society guideline: a shorter lag to treatment gives better development.

Sources: NICE 6.3.1; Go 2012.

Hormonal therapy, vigabatrin or both

  • Go 2012: low-dose ACTH probably as effective as high-dose; ACTH more effective than vigabatrin for short-term control, excluding infants with tuberous sclerosis complex; better long-term development after successful hormonal therapy than after vigabatrin in spasms of unknown cause.
  • International Collaborative Infantile Spasms Study: adding vigabatrin to hormonal therapy stopped spasms between days 14 and 42 in 72% against 57% with hormonal therapy alone.
  • NICE: prednisolone with vigabatrin first; vigabatrin alone when the risk of steroid adverse effects is high; test varicella zoster antibodies before prednisolone.

Sources: Go 2012; O'Callaghan 2017; NICE 6.3.4 to 6.3.7.

US labels

  • Repository corticotropin: monotherapy under age 2.
  • Vigabatrin: monotherapy from 1 month to 2 years, with its vision checks.

Sources: ACTH label; vigabatrin label.

Dravet syndrome: no sodium-channel blockers

More than 85% of people with Dravet syndrome carry a pathogenic variant in SCN1A, which leaves them with too little of the Nav1.1 sodium channel (Wirrell 2022). In a mouse model, losing Nav1.1 reduced the sodium current in inhibitory interneurons but not in excitatory pyramidal neurons, which points to failing inhibition as the cause of the seizures (Yu 2006).

Nav1.1 loss silences inhibitory interneurons; sodium-channel blockers deepen it (Yu 2006; Guerrini 1998; Shi 2016; Wirrell 2022).

Sodium-channel blockers worsen it

  • Lamotrigine worsened seizures in 17 of 21 children; the international consensus rates it contraindicated in children with Dravet syndrome.
  • Carbamazepine had no effect or aggravated seizures in every SCN1A-positive patient who received it, in a retrospective series.

Sources: Guerrini 1998; Shi 2016; Wirrell 2022.

What to choose

  • Valproate first; clobazam, stiripentol or fenfluramine as first or second choices.
  • Cannabidiol: physicians placed it among the first three choices; caregivers supported it as first or second.
  • US labels: stiripentol only with clobazam, from 6 months of age and 7 kg, with no data as monotherapy; fenfluramine from age 2, with the echocardiograms of its boxed warning; cannabidiol from age 1.

Sources: Wirrell 2022; stiripentol label; fenfluramine label; cannabidiol label.

Status epilepticus

  • After a benzodiazepine, the consensus prefers intravenous valproate; levetiracetam and phenytoin or fosphenytoin are also appropriate.
  • Phenytoin is not contraindicated in that setting, although it is avoided for maintenance.

Source: Wirrell 2022.

Lennox–Gastaut syndrome

First choices for Lennox–Gastaut syndrome, tuberous sclerosis complex, CDKL5 deficiency disorder, childhood absence epilepsy and juvenile myoclonic epilepsy (Auvin 2025; NICE NG217 chapters 5 and 6; US labels).

The order of treatment

  • Expert algorithm: valproate first, then add lamotrigine, then rufinamide; after those, in alphabetical order, cannabidiol, clobazam, felbamate, fenfluramine and topiramate.
  • No more than two antiseizure medications together whenever possible.
  • NICE advises careful titration and frequent review of each added medicine.

Sources: Auvin 2025; NICE 6.2.

Drugs that may worsen it

  • NICE: carbamazepine, gabapentin, lacosamide, lamotrigine, oxcarbazepine, phenobarbital, pregabalin, tiagabine and vigabatrin. Lamotrigine is both a second-line choice and on this list.
  • Expert algorithm: caution with carbamazepine, oxcarbazepine, eslicarbazepine, tiagabine and phenytoin.
  • Clonazepam lost its effect in up to 30% of responders, often within 3 months, and can bring on tonic–clonic seizures when several seizure types coexist.

Sources: NICE 6.2.8; Auvin 2025; clonazepam label.

US labels and an interaction

  • Labeled for the syndrome: clobazam from age 2, rufinamide from age 1, cannabidiol from age 1, fenfluramine, lamotrigine and topiramate from age 2, felbamate, and clonazepam.
  • Valproate raises rufinamide levels by up to 70%, so with valproate rufinamide starts below the usual starting dose, in children and in adults.

Source: rufinamide label and the labels of each drug.

Tuberous sclerosis complex and CDKL5 deficiency disorder

Tuberous sclerosis complex

  • Spasms: vigabatrin alone first, adding prednisolone if it has not worked after 1 week (NICE). The finding that ACTH works better than vigabatrin did not include these infants.
  • The focal seizures that follow: the mechanistic target of rapamycin (mTOR) inhibitor everolimus, labeled as an add-on from age 2, with dosing guided by trough levels; cannabidiol from age 1.

Sources: NICE 6.3.6; Go 2012; everolimus label; cannabidiol label.

Cyclin-dependent kinase-like 5 (CDKL5) deficiency disorder

  • One labeled drug: ganaxolone, from age 2, a neuroactive steroid that positively modulates gamma-aminobutyric acid type A (GABA-A) receptors.
  • A Schedule V controlled substance; somnolence and sedation.

Source: ganaxolone label.

Childhood absence and juvenile myoclonic epilepsy

Both are generalized epilepsies, so the drugs that worsen absence and myoclonic seizures are the ones to avoid (see Choosing a first antiseizure medication). Since January 2025, NICE follows the UK medicines regulator: valproate is not started in anyone under 55, of either sex, unless two specialists independently agree, and in women and girls who could become pregnant only if other treatments fail, with the Pregnancy Prevention Programme (NICE NG217, box 2).

Childhood absence epilepsy

  • Ethosuximide first: it matched valproate for efficacy with fewer attentional effects, and both did better than lamotrigine.
  • If it fails, NICE offers a choice of lamotrigine, levetiracetam or valproate; with other seizure types, one of these three comes first.
  • Ethosuximide treats absence seizures only: used alone in mixed seizure types it may increase tonic–clonic seizures.

Sources: Glauser 2010; NICE 5.3.1 to 5.3.4; ethosuximide label.

Juvenile myoclonic epilepsy

  • NICE offers a choice of levetiracetam or valproate, with valproate under the restrictions above.
  • Do not use carbamazepine, oxcarbazepine, phenytoin, gabapentin, pregabalin, tiagabine or vigabatrin; lamotrigine can occasionally worsen myoclonic seizures.
  • US label: levetiracetam as an add-on for myoclonic seizures in juvenile myoclonic epilepsy from age 12.

Sources: NICE 5.4.2 to 5.4.5; levetiracetam label.

Key points

  • Infantile spasms: treat within days with hormonal therapy, vigabatrin or both; in tuberous sclerosis complex, vigabatrin alone first.
  • Dravet syndrome: valproate, clobazam, stiripentol, fenfluramine or cannabidiol, and no sodium-channel blockers for maintenance.
  • Lennox–Gastaut syndrome: valproate, then lamotrigine and rufinamide, with many labeled add-ons; start rufinamide lower with valproate.
  • Absence: ethosuximide first, then lamotrigine, levetiracetam or valproate. Juvenile myoclonic epilepsy: levetiracetam or valproate, with valproate's restrictions for women and girls who could become pregnant. Avoid carbamazepine, oxcarbazepine and phenytoin in both.

Quick check A 3-year-old with Dravet syndrome due to an SCN1A variant still has frequent prolonged convulsive seizures on valproate and clobazam. Which addition fits best?

Quick check A 5-month-old with tuberous sclerosis complex has clusters of spasms on waking, and the electroencephalogram (EEG) shows hypsarrhythmia. Which first treatment does NICE recommend?

Quick check A 16-year-old girl has myoclonic jerks after waking and has had one generalized tonic–clonic seizure; juvenile myoclonic epilepsy is diagnosed. Which first medication does NICE recommend for her?

Quick check A 9-year-old with Lennox–Gastaut syndrome taking valproate is to start rufinamide. What does the rufinamide label advise?

Sources: as cited; indications and ages from the current US labels on DailyMed. The 2012 infantile spasms guideline has since been retired by the American Academy of Neurology and is cited for its evidence review; NICE NG217 recommendations are numbered as on the NICE website.

Drug levels and interactions

Concept

A drug level answers a specific question; it is not a target to chase. And because several antiseizure medications induce or inhibit liver enzymes, they change the levels of each other and of many other drugs.

When a level helps

The International League Against Epilepsy (ILAE) position paper on drug monitoring lists when a level is most likely to help (Patsalos 2008):

  • Once seizures are controlled, to record the patient's own effective level, so that later changes can be interpreted.
  • To diagnose suspected toxicity.
  • To check adherence, especially after a breakthrough seizure.
  • When pharmacokinetics vary: children, older adults, other illness, a change of formulation.
  • When a large change is expected: pregnancy, or an interacting drug added or stopped.
  • For drugs whose kinetics depend on the dose, above all phenytoin.
A level answers a question; the reference range is a population band, not a target (Patsalos 2008; Patsalos 2018).

The laboratory reference range is not a therapeutic range. It marks a population band below which a response is less likely and above which toxicity is more likely, and some patients are controlled below it while others need and tolerate levels above it. Treat the patient, not the level (Patsalos 2018).

Total and free levels

Laboratories usually report the total concentration, bound and unbound together, but only the unbound drug acts. Phenytoin is about 92% bound to plasma proteins, mostly albumin (Patsalos 2018). With low albumin, or with kidney or liver disease, the unbound fraction rises, so a total level inside the range can hide toxicity; the label advises monitoring unbound phenytoin in these patients (phenytoin label). In kidney failure the cause is weaker binding: in serum from patients with chronic kidney failure treated conservatively, measured in the laboratory, the free fraction of phenytoin was about twice normal at a creatinine near 800 µmol/L (about 9 mg/dL) and up to 4 to 5 times normal in uremia (Tiula 1987). Valproate both displaces phenytoin from albumin and slows its metabolism, and valproate's own binding saturates, so its free fraction rises at higher concentrations and with low albumin (valproate label).

Phenytoin's metabolism also saturates, at a level that differs from patient to patient and is sometimes within the usual range, so a modest increase in dose can cause a very large rise in the level, and toxicity (phenytoin label; Patsalos 2018).

Schematic, not to scale. Phenytoin is about 90% bound to plasma proteins, mostly albumin (Patsalos 2018); in serum from patients with kidney failure the free fraction was about twice normal (Tiula 1987); the label advises measuring unbound phenytoin in these patients and describes its saturable metabolism (phenytoin label).

Enzyme inducers and inhibitors

Inducers speed the clearance of other drugs; valproate slows it (carbamazepine, phenytoin and valproate labels).

Strong inducers

  • Carbamazepine, phenytoin and phenobarbital are potent inducers of liver enzymes, including cytochrome P450 3A4 (CYP3A4); primidone is metabolized to phenobarbital.
  • They lower the levels of many other drugs: lamotrigine, valproate and other antiseizure medications, hormonal contraceptives, warfarin, cyclosporine and protease inhibitors among them.
  • Carbamazepine induces its own metabolism: its half-life of 25 to 65 hours at first falls to 12 to 17 hours, and autoinduction is complete after 3 to 5 weeks, so the level at an unchanged dose falls over the first weeks.

Sources: carbamazepine label; phenytoin label.

Weaker inducers, and drugs that do both

  • Hormonal contraception may be less effective with oxcarbazepine, eslicarbazepine, cenobamate, rufinamide and clobazam, and with perampanel and topiramate at higher doses (see Contraception).
  • Cenobamate raises phenytoin and phenobarbital and lowers lamotrigine and carbamazepine.
  • Felbamate raises phenytoin and valproate and lowers carbamazepine.

Sources: the labels of oxcarbazepine, eslicarbazepine, cenobamate, rufinamide, clobazam, perampanel, topiramate and felbamate.

Valproate inhibits

  • A weak inhibitor of some cytochrome P450 enzymes, of epoxide hydrolase and of glucuronidation; it also displaces drugs from albumin.
  • So it raises lamotrigine, phenobarbital and the active carbamazepine epoxide, and the free fraction of phenytoin.

Source: valproate label.

Interactions worth knowing by name

Valproate and lamotrigine

  • Valproate slightly more than doubles lamotrigine concentrations.
  • It heads the risk factors for serious rash in lamotrigine's boxed warning: adults taking both were hospitalized for rash in 1% against 0.16% without valproate. Use the label's slower titration.

Source: lamotrigine label.

Carbapenems and valproate

  • In a retrospective series of 52 patients, valproate levels began to fall within 24 hours of starting a carbapenem; the mean falls were 72% with ertapenem, 67% with meropenem and 42% with imipenem, below the usual range in 90%.
  • Raising the valproate dose did not restore the levels during ertapenem or meropenem.
  • Choose another antibiotic, or cover with another antiseizure medication, and monitor valproate levels often.

Sources: Wu 2016; meropenem label (section 5.5); valproate label (section 5.15).

Inducers and direct oral anticoagulants

  • The apixaban and rivaroxaban labels say to avoid drugs that are both P-glycoprotein and strong CYP3A4 inducers, naming carbamazepine and phenytoin.
  • The anticoagulant level falls, and the risk of stroke and thrombosis rises.

Sources: apixaban label; rivaroxaban label.

Inducers and warfarin

  • Carbamazepine and phenobarbital lower warfarin's effect; with phenytoin the international normalized ratio (INR) has been reported both to rise and to fall.
  • Check the INR closely whenever an antiseizure medication is started, stopped or changed.
  • Inducers and hormonal contraception: see Contraception.

Sources: warfarin label; phenytoin label.

From the current US labels (carbamazepine label; phenytoin label; valproate label; apixaban label; rivaroxaban label; warfarin label) and, for carbapenems, Wu 2016.

Kidney and liver

Drugs cleared mainly by the kidney accumulate as creatinine clearance falls, and several are removed by hemodialysis. Drugs metabolized by the liver need care in hepatic impairment. The labels give the doses; the patterns are what to know.

Kidney failure: lower doses

  • Levetiracetam is excreted 66% unchanged in the urine: dosed by creatinine clearance.
  • Gabapentin (not appreciably metabolized) and pregabalin (eliminated mainly by the kidney) are dosed by creatinine clearance.
  • Vigabatrin, topiramate, oxcarbazepine, felbamate and lacosamide need lower doses, or a lower maximum, in renal impairment, by the label's steps.
  • Zonisamide should not be used when the estimated glomerular filtration rate is below 50 mL/min.

Sources: the labels of levetiracetam, gabapentin, pregabalin, vigabatrin, topiramate, oxcarbazepine, felbamate, lacosamide and zonisamide.

After hemodialysis

  • Hemodialysis removes levetiracetam, gabapentin, pregabalin, lacosamide and topiramate: their labels advise a supplemental dose after a session (for topiramate, one may be required).
  • A 4-hour session halves lacosamide exposure.
  • Brivaracetam and cenobamate are not recommended in end-stage renal disease on dialysis.

Sources: the labels of levetiracetam, gabapentin, pregabalin, lacosamide, topiramate, brivaracetam and cenobamate.

Hepatic impairment

  • Valproate is contraindicated in hepatic disease or significant hepatic dysfunction; felbamate is not prescribed with any history of hepatic dysfunction.
  • Brivaracetam needs a dose adjustment in all stages of hepatic impairment.
  • Lacosamide and cenobamate have lower maximum doses in mild to moderate impairment and are not recommended in severe impairment; cenobamate can also injure the liver (see Boxed warnings, monitoring and organ effects).
  • Levetiracetam needs no adjustment. For phenytoin, measure the unbound level, as above.

Sources: the labels of valproate, felbamate, brivaracetam, lacosamide, cenobamate and levetiracetam.

Key points

  • Treat the patient, not the reference range; a level is most useful compared with the same patient's level when seizures were controlled.
  • With low albumin or kidney failure, measure free phenytoin.
  • Strong inducers lower many drugs, hormonal contraceptives included; valproate raises lamotrigine; carbapenems sharply lower valproate levels.
  • In kidney failure, lower the dose of renally cleared drugs; after hemodialysis, give the supplemental dose the label advises.

Quick check A man well controlled on valproate is admitted with pneumonia, starts meropenem, and has a seizure two days later. His valproate level is far below his usual. What is the most likely explanation?

Quick check A man with focal epilepsy and a normal albumin has a total phenytoin level in the lower part of the reference range. After two breakthrough seizures his daily dose is raised by a third. Two weeks later he has nystagmus and ataxia, and the level is well above the range. What explains the disproportionate rise?

Quick check A 62-year-old whose focal seizures are controlled on levetiracetam starts hemodialysis three times a week. What does the levetiracetam label advise?

Sources: as cited; labels are the current US prescribing information on DailyMed.

When a seizure becomes status epilepticus

Emergency

Status epilepticus is defined by two clocks. The first says when to start treating; the second, when there is a risk of lasting injury. Both depend on the type of seizure.

The “five minute rule” is not a rule about seizures in general — it is t1 for convulsive status only. The International League Against Epilepsy (ILAE) 2015 operational definition sets two separate clocks, and they differ by seizure type:

  • t1 — the point beyond which the seizure is unlikely to stop on its own. This is when emergency treatment starts.
  • t2 — the point beyond which there is a risk of long-term consequences: neuronal injury and death, and altered neuronal networks. This is what you are racing.
Type of status epilepticus t1 — treat now t2 — risk of lasting injury
Tonic–clonic (convulsive) 5 minutes 30 minutes
Focal with impaired consciousness 10 minutes > 60 minutes
Absence status 10–15 minutes Unknown
The operational definition of the International League Against Epilepsy, drawn to scale (Trinka 2015).

Use the clocks correctly: these operational estimates vary by seizure type and do not establish a safe waiting period. Clinical deterioration, recurrent seizures without recovery, or uncertain onset requires prompt assessment and treatment. The time associated with injury is less certain in nonconvulsive status.

Trinka E, Cock H, Hesdorffer D, et al. A definition and classification of status epilepticus — Report of the ILAE Task Force on Classification of Status Epilepticus. Epilepsia 2015;56(10):1515–23.

Types of status epilepticus

The same report classifies status along four axes: its semiology, its etiology, its electroencephalographic (EEG) correlates and the patient's age. The first axis divides status into two groups that are treated differently:

With prominent motor symptoms

  • Convulsive status (bilateral tonic–clonic, of generalized or focal onset): the emergency with the shortest clocks.
  • Myoclonic, focal motor, tonic and hyperkinetic status.

Without prominent motor symptoms: nonconvulsive status

  • With coma, as in the intensive care unit.
  • Without coma, such as absence status and focal status with impaired consciousness.
  • Recognized on the EEG.
The first axis of the classification of the International League Against Epilepsy (Trinka 2015).

Key points

  • Five minutes is t1 for convulsive status only; focal status with impaired consciousness has a later t1.
  • t2 is the deadline you are racing: 30 minutes for convulsive status.
  • Nonconvulsive status is diagnosed on the EEG, with or without coma.

Quick check In the ILAE definition, what does t2 mark for convulsive status epilepticus?

Treating convulsive status epilepticus

Emergency

Treatment is a sequence run against the clock: stabilize, give a benzodiazepine early and in full, add a second-line medication if convulsions continue, and escalate to anesthesia if that fails. The common errors are delay and underdosing.

Treatment clock for convulsive status epilepticus Drawn to scale over the first hour. Minutes 0 to 5: stabilize. At 5 minutes, t1 for convulsive status, give a benzodiazepine; the American Epilepsy Society places this initial therapy between 5 and 20 minutes. From 20 to 40 minutes, if convulsions continue, a second-line antiseizure medication. From 40 to 60 minutes, an anesthetic infusion with continuous EEG. Thirty minutes is t2, after which there is a risk of lasting injury; it falls during second-line treatment. Stabilize Benzodiazepine Second-line medication Anesthetic + continuous EEG 0 5 min 20 min 30 min 40 min 60 min t₁: treat now t₂: risk of lasting injury To scale: minutes from the start of the seizure.

t₁ = 5 minutes: treat now. t₂ = 30 minutes: from here, a risk of lasting injury. Refractory status begins when a benzodiazepine and one second-line medication fail.

Phases from the American Epilepsy Society guideline: stabilization to 5 minutes, initial therapy from 5 to 20, second therapy from 20 to 40 and third therapy from 40 to 60 (Glauser 2016). The times are targets, not waiting periods: move on as soon as a step fails, and refractory status is defined by treatment failure, not by the clock. If consciousness does not return toward baseline after the movements stop, get an urgent electroencephalogram (EEG) for nonconvulsive seizures.
From the first minute

Stabilize

  • Airway, breathing and circulation; note the time the seizure began.
  • Intravenous access, an electrocardiogram (ECG) and a finger-stick glucose.
  • Treat hypoglycemia at once. Give thiamine before or with glucose when deficiency is possible, but never delay glucose.
At 5 minutes

Benzodiazepine

  • The first-line treatment. Give it promptly and in a full dose; underdosing is common and makes it less likely to work.
  • No intravenous access: intramuscular midazolam, or an intranasal or buccal benzodiazepine.
If convulsions continue

Second-line medication

  • Levetiracetam, fosphenytoin or valproate, given intravenously; none was superior in the Established Status Epilepticus Treatment Trial (ESETT).
  • Choose by the patient: valproate is avoided in pregnancy, liver disease and mitochondrial disease; fosphenytoin needs cardiac and blood pressure monitoring.
  • Phenobarbital is the alternative when none of these is available.
If both fail

Refractory status

  • Convulsions continue after an adequate benzodiazepine and one second-line medication.
  • Call neurocritical care: airway, an anesthetic infusion and continuous EEG.
  • Escalate when the second-line drug fails, whatever the clock says, and look for the cause in parallel.

The minute marks differ a little between guidelines; the American Epilepsy Society guideline gives phases of stabilization from 0 to 5 minutes, initial therapy from 5 to 20, second therapy from 20 to 40 and third therapy from 40 to 60 minutes (Glauser 2016). The order is the same in all of them, and the times are targets, not waiting periods.

Why the benzodiazepine must be early and full

In the American Epilepsy Society guideline, a benzodiazepine is the initial therapy of choice (Level A). In adults, the second therapy is less effective than the first and the third substantially less (Level A); in children, the second also appears less effective, on weaker evidence (Level C) (Glauser 2016). Two prehospital trials show why it should come early:

The Prehospital Treatment of Status Epilepticus trial (Alldredge 2001), the Rapid Anticonvulsant Medication Prior to Arrival Trial (Silbergleit 2012) and first doses in the Established Status Epilepticus Treatment Trial (Sathe 2021).

Evidence

Prehospital Treatment of Status Epilepticus trial (PHTSE): lorazepam, diazepam or placebo given by paramedics, 205 adults
Status had stopped by arrival at the emergency department in 59% with lorazepam and 43% with diazepam, against 21% with placebo. Respiratory or circulatory complications were about twice as common with placebo (23% against 10–11%), though the difference was not significant (Alldredge 2001).
Rapid Anticonvulsant Medication Prior to Arrival Trial (RAMPART): intramuscular midazolam or intravenous lorazepam, 893 children and adults
Seizures had stopped on arrival without rescue therapy in 73% with intramuscular midazolam against 63% with intravenous lorazepam. Among patients whose seizures stopped before arrival, the injection went in a median of about 3½ minutes sooner, which made up for its slower onset, and intubation rates were the same (Silbergleit 2012).

In practice, benzodiazepines are often given late and in too small a dose:

Established Status Epilepticus Treatment Trial

  • More than three-quarters of first doses of midazolam and of lorazepam were below the guideline dose.
  • Median time from onset to the first dose: 27 minutes.

Source: Sathe 2021.

Registry of 1,049 adults

  • In Germany, Austria and Switzerland, about three-quarters of first doses were below recommendations.
  • A benzodiazepine as the first drug, and a higher cumulative early dose of antiseizure medication, went with faster control.

Source: Kellinghaus 2019.

Children with refractory convulsive status

  • A first benzodiazepine 10 minutes or more after onset went with more deaths, more continuous infusions and longer convulsions.
  • An observational study; the estimate for death is imprecise.

Source: Gaínza-Lein 2018.

Choosing the second-line medication

Evidence

Established Status Epilepticus Treatment Trial (ESETT): levetiracetam, fosphenytoin or valproate after a benzodiazepine failed, 384 patients aged 2 and older
Seizures stopped, with responsiveness improving, at 60 minutes in 47% with levetiracetam, 45% with fosphenytoin and 46% with valproate. The trial stopped for futility, and hypotension, arrhythmia, intubation and death did not differ significantly (Kapur 2019). The three drugs did not differ in children, adults or older adults (Chamberlain 2020).
Levetiracetam or phenytoin in children: the EcLiPSE trial (United Kingdom, 286 children) and the ConSEPT trial (Australia and New Zealand, 233 children)
Two open-label trials found levetiracetam no better than phenytoin: convulsive status stopped in 70% against 64% in one (Lyttle 2019) and 50% against 60% in the other (Dalziel 2019).
PHTSE (Alldredge 2001), RAMPART (Silbergleit 2012) and ESETT (Kapur 2019).

None of the three was superior; they had similar efficacy, so the choice is made by the patient's other conditions and by local practice. The labels set the limits:

Fosphenytoin

  • Contraindicated in sinus bradycardia, sinoatrial block, second- and third-degree atrioventricular block and Adams–Stokes syndrome.
  • Monitor the electrocardiogram, blood pressure and respiratory function continuously during the infusion.

Source: fosphenytoin label.

Intravenous valproate

  • Contraindicated in hepatic disease or significant hepatic dysfunction.
  • In mitochondrial disorders caused by mutations in mitochondrial DNA polymerase gamma (POLG), and in children under 2 suspected of a POLG-related disorder.
  • In known urea cycle disorders.

Source: valproate sodium injection label.

Not every case was epileptic

  • In ESETT, 10% of the patients enrolled for benzodiazepine-refractory status turned out to have functional/dissociative seizures (formerly called psychogenic nonepileptic seizures).

Source: Kapur 2019.

Key points

  • Treat at 5 minutes with a benzodiazepine at full dose; without intravenous access, intramuscular midazolam works at least as well.
  • Levetiracetam, fosphenytoin and valproate had similar efficacy as second-line drugs; choose by the patient.
  • Failure of a benzodiazepine and one second-line drug defines refractory status, whatever the clock says.

Quick check A man has been convulsing for 7 minutes on arrival. The nurse cannot place an intravenous line. What is the best next step?

Quick check A 74-year-old man with known second-degree atrioventricular block and no pacemaker is still convulsing after a full dose of intravenous lorazepam. Which second-line plan is best?

Sources: treatment phases and evidence levels, Glauser 2016; clocks, Trinka 2015 (previous lesson); trials as cited above. The clock follows the American Epilepsy Society phases; for doses, see the Emergency Neurological Life Support status epilepticus protocol (version 6.0, 2024) or your institution's protocol.

After the convulsions stop, and refractory status

Emergency

Stopping the movements is not the same as stopping the seizure. And when first- and second-line treatment both fail, the problem moves to the intensive care unit, where the EEG becomes the way to follow treatment.

When consciousness does not return

In a prospective study of 164 patients monitored with continuous electroencephalography (EEG) for at least 24 hours after their convulsive status had been controlled, 48% still had electrographic seizures and more than 14% were in nonconvulsive status epilepticus; these patients were comatose, with no visible sign of convulsions (DeLorenzo 1998). The American Clinical Neurophysiology Society (ACNS) consensus therefore recommends continuous EEG when mental status stays abnormal after convulsive status, and suggests considering it when alertness has not clearly improved within 10 minutes, or consciousness is still impaired 30 minutes after the clinical seizures stop (Herman 2015).

Continuous EEG after convulsive status (DeLorenzo 1998). Stages: refractory (Brophy 2012; Herman 2015) and super-refractory (Shorvon 2011).

So when a patient treated for convulsive status is not returning toward baseline, arrange an urgent EEG rather than attributing the coma to the medications. Subtle signs, such as nystagmoid eye jerks or subtle clonic movements of the face or a limb, can be the only clinical trace of ongoing seizures.

Refractory and super-refractory status

Status that continues after an initial benzodiazepine and a second antiseizure medication is refractory: it is defined by failed treatment, not by the clock (Brophy 2012; Herman 2015). Status that continues or returns 24 hours or more after anesthesia began, including when the anesthetic is being reduced, is super-refractory (Shorvon 2011).

Refractory (Brophy 2012) and super-refractory status (Shorvon 2011); new-onset refractory status epilepticus (Hirsch 2018).

The principles of care in the intensive care unit:

Anesthetic infusion with continuous EEG

  • Midazolam, propofol or a barbiturate, titrated to stop electrographic seizures or to a burst-suppression target set by the local neurocritical care protocol.
  • No anesthetic has been shown better than another in adults: the only randomized trial in adults, of propofol against barbiturates, recruited 24 of the 150 patients it needed in 3 years and could not answer the question.

Source: Rossetti 2011.

Keep a maintenance antiseizure medication

  • Keep it going, and add a second one, so that seizures do not return when the infusion is weaned.

Find the cause in parallel

  • Imaging, cerebrospinal fluid, toxicology and metabolic causes.
  • Autoimmune and paraneoplastic antibodies.

Other options

  • Considered by a neurocritical care team: ketamine, the ketogenic diet, and immunotherapy when an autoimmune or inflammatory cause is suspected.
  • Most rest on case series rather than trials.

When there is no obvious cause

New-onset refractory status epilepticus (NORSE)

  • A clinical presentation rather than a diagnosis: refractory status in a person without active epilepsy or another relevant neurological disorder.
  • No clear acute or active structural, toxic or metabolic cause.
  • When the full workup finds no cause: cryptogenic NORSE.

Sources: Hirsch 2018; Wickstrom 2022.

Febrile infection-related epilepsy syndrome (FIRES)

  • A subcategory of NORSE, at any age.
  • A febrile infection began between 2 weeks and 24 hours before the refractory status.

Source: Hirsch 2018.

The practical point is to search early for autoimmune and infectious causes, because some of them can be treated. An international consensus advises first-line immunotherapy within 72 hours of onset and, if seizures continue, second-line immunotherapy and the ketogenic diet early (Wickstrom 2022).

Key points

  • After convulsive status is controlled, a patient whose consciousness does not return needs an urgent EEG.
  • Refractory status follows failure of a benzodiazepine and a second-line drug; super-refractory status continues or returns 24 hours or more after anesthesia began.
  • New-onset refractory status without a clear cause (NORSE) calls for an early search for autoimmune and infectious causes.

Quick check Lorazepam and then levetiracetam stopped a woman's convulsions 30 minutes ago. She is still unresponsive, with occasional subtle clonic movements at the corner of her mouth. What should happen next?

Sources: as cited. The Neurocritical Care Society guideline (Brophy 2012) also sets a goal of definitive control within 60 minutes of onset.

Seizures on continuous EEG and the ictal–interictal continuum

Clinical reasoning

In the intensive care unit most seizures have no visible sign. Continuous electroencephalography (EEG) finds them, and it also finds patterns that are neither clearly seizures nor clearly harmless. Deciding what to do with those is where EEG reading turns into management.

Who needs continuous EEG, and for how long

In 570 critically ill patients monitored with continuous EEG, 19% had seizures, and in 92% of those every seizure was nonconvulsive. The first seizure came within 24 hours in 88%, but it came later in 20% of comatose patients against 5% of those not in coma (Claassen 2004). A routine 30 to 60 minute EEG finds only about half (45 to 58%) of the patients who go on to have seizures (Herman 2015).

Who needs continuous EEG

The American Clinical Neurophysiology Society (ACNS) recommends it for critically ill patients with:

  • Persistently abnormal mental status after convulsive status or other clinical seizures.
  • Acute supratentorial brain injury with altered mental status.
  • Fluctuating or unexplained altered mental status.
  • Periodic discharges on a routine EEG.
  • Pharmacological paralysis with a risk of seizures.
  • Suspected paroxysmal events.

Source: Herman 2015.

For how long

  • At least 24 hours of recording.
  • 48 hours or more may be needed in comatose or sedated patients and those with periodic discharges.

Source: Herman 2015.

The 2HELPS2B score: when a shorter recording is enough

  • 2 points for brief potentially ictal rhythmic discharges.
  • 1 point each for lateralized periodic discharges, lateralized rhythmic delta or bilateral independent periodic discharges; a prior seizure; sporadic epileptiform discharges; any periodic or rhythmic pattern faster than 2 Hz; and plus features.
  • On the first hour of EEG: 0 meant a seizure risk under 5% (a 1-hour screen was enough); 1 meant about 12% (monitor 12 hours); 2 or more meant over 25% (monitor at least 24 hours).
  • The validation excluded patients after cardiac arrest and planned admissions to the epilepsy monitoring unit.

Sources: Struck 2017; Struck 2020.

Timing of the first seizure (Claassen 2004); risk by score (Struck 2017) and monitoring time (Struck 2020).

Seizure, status or continuum: the definitions

The ACNS 2021 terminology separates what is certainly a seizure from what might be (Hirsch 2021):

Electrographic seizure

  • Epileptiform discharges averaging more than 2.5 Hz for 10 seconds or more,
  • or any pattern with definite evolution lasting 10 seconds or more.

Electrographic status epilepticus

  • An electrographic seizure lasting 10 continuous minutes or more,
  • or adding up to 20% or more of any 60-minute period.

Electroclinical seizure

  • Any pattern with a definite clinical correlate time-locked to it,
  • or one after which both the EEG and the patient improve with a parenteral antiseizure medication.

Ictal–interictal continuum

  • Periodic discharges or spike-and-wave averaging more than 1 and up to 2.5 Hz; or 0.5 to 1 Hz with a plus modifier or fluctuation; or lateralized rhythmic delta above 1 Hz with a plus modifier or fluctuation.
  • A description of the EEG, not a diagnosis; the ACNS calls it a concept still under development.
  • Lasting 10 continuous minutes, or 20% of any hour, and improving on the EEG after medication without clinical improvement: possible electroclinical status.

Not every pattern carries the same risk. In 4,772 critically ill adults, lateralized periodic discharges were most strongly associated with seizures at every frequency, especially with plus features; generalized periodic discharges and lateralized rhythmic delta became riskier as they got faster; and generalized rhythmic delta carried no more risk than no pattern at all (Rodriguez Ruiz 2017).

Where periodic and rhythmic patterns fall: interictal, on the ictal–interictal continuum, or an electrographic seizure (ACNS 2021). Point at or tab to a band for its rule.

Diagnosing nonconvulsive status: the Salzburg criteria

For patients without a known epileptic encephalopathy, the Salzburg criteria turn the definitions into a diagnosis (Beniczky 2013; Leitinger 2015):

Enough by themselves

  • Epileptiform discharges faster than 2.5 Hz.

Need one more feature

Slower discharges, or rhythmic delta or theta faster than 0.5 Hz, need one of:

  • subtle clinical ictal signs;
  • typical evolution in space and time;
  • improvement of both the EEG and the patient after an intravenous antiseizure medication.

Only possible

  • Improvement on the EEG alone, or fluctuation without evolution: nonconvulsive status is only possible.

In the validation study's 120 patients with suspected nonconvulsive status, the criteria had a sensitivity of 97.7% and a specificity of 89.6%; in 100 controls with abnormal EEGs the specificity was 97.0% (Leitinger 2016).

Does treating the pattern help?

Evidence

Continuous EEG Randomized Trial in Adults (CERTA): continuous or two routine EEGs, 364 adults with impaired consciousness and no recent seizure
Continuous EEG found seizures more often (15.7% against 4.4%) and led to more changes in antiseizure medication, but mortality at 6 months was the same: 48.9% against 48.4% (Rossetti 2020).
Treatment of Electroencephalographic Status Epilepticus After Cardiopulmonary Resuscitation (TELSTAR): treating rhythmic and periodic patterns after cardiac arrest, 172 comatose patients
Stepwise antiseizure treatment suppressed the patterns completely in 56%, but a poor outcome at 3 months was as common as with standard care: 90% against 92% (Ruijter 2022).
The Continuous EEG Randomized Trial in Adults (Rossetti 2020) and the Treatment of Electroencephalographic Status Epilepticus After Cardiopulmonary Resuscitation trial (Ruijter 2022).

These trials do not show that seizures in critically ill patients are harmless. They show that finding and suppressing a pattern does not by itself change the outcome, which depends at least as much on the cause and the patient.

A reasonable approach to a continuum pattern

No trial tells you exactly what to do, so the approach is a judgment that weighs the pattern against the patient:

  1. Describe it precisely. Name, frequency, prevalence, plus modifiers and any evolution or fluctuation. Higher frequency, plus features and lateralized patterns carry more seizure risk.
  2. Look for a clinical correlate: a focal deficit, fluctuating responsiveness or subtle clonic movements time-locked to the pattern.
  3. Consider a diagnostic medication trial while watching the EEG and the examination. Improvement in both supports treating the pattern as nonconvulsive status.
  4. Treat the cause and limit harm. A non-sedating antiseizure medication is a common choice; escalating to anesthesia for a continuum pattern alone is weighed carefully against its risks.

Key points

  • Most seizures in critically ill patients are nonconvulsive; comatose patients may need 48 hours of recording to catch the first one.
  • An electrographic seizure lasts 10 seconds or more; electrographic status is 10 continuous minutes or 20% of any hour.
  • Suppressing rhythmic and periodic patterns after cardiac arrest did not improve outcome in TELSTAR; treat the patient, not only the pattern.

Quick check A comatose patient's EEG shows lateralized periodic discharges at 1.5 Hz without evolution, for most of the record. How does the American Clinical Neurophysiology Society 2021 terminology classify this?

Sources: as cited. Pattern names follow the ACNS 2021 terminology taught in EEG Fundamentals.

Drug-resistant epilepsy: definition and referral

Clinical pathway

About a third of people with epilepsy keep having seizures despite medication. Recognizing that early, and referring, is the most useful thing a general neurologist can do for them.

The International League Against Epilepsy (ILAE) defines drug-resistant epilepsy as failure of adequate trials of two antiseizure medication schedules to achieve sustained seizure freedom. The schedules must have been tolerated, appropriately chosen and appropriately used, alone or in combination (Kwan 2010).

  • Appropriate: a drug with proven efficacy for the patient's seizure type, preferably in randomized trials.
  • Adequate: enough of it, for long enough.
  • Seizure freedom: no seizures for three times the longest interval between seizures before the change, or 12 months, whichever is longer.
From drug resistance to treatment options Refer as soon as two appropriate medications have failed; a third medication rarely brings freedom from seizures.
  1. 1Two medication trials failTolerated, appropriately chosen and adequately dosed, alone or combined
  2. 2Refer to an epilepsy centerConfirm the diagnosis, epilepsy type and adherence
  3. 3Presurgical evaluation and stereo EEGVideo-EEG in the epilepsy monitoring unit · epilepsy-protocol magnetic resonance imaging (MRI) · neuropsychology · positron emission tomography, single-photon emission computed tomography or magnetoencephalography as needed · stereo EEG when noninvasive data do not agree
  4. 4Choose a treatmentResection or laser ablation for a localized onset · vagus nerve, responsive or deep brain stimulation · corpus callosotomy for drop seizures · dietary therapy · trials

Why two medications are enough to call it

Because each drug that fails makes the next one less likely to work. In 1,795 people followed from their first treatment, the first regimen made 50.5% of the whole cohort seizure-free, the second a further 11.6% and the third a further 4.4%; every later regimen together added about 2%. Failure of the first drug raised the odds that each later regimen would fail too (Chen 2018).

The Glasgow cohort (Chen 2018).

Rule out pseudoresistance

Some apparent drug resistance is something else. Before calling it, check four things, which the epilepsy center will also review:

The diagnosis

  • Functional seizures and syncope do not respond to antiseizure medication.
  • Video-EEG of the habitual events settles it.

The drug for the epilepsy

  • A sodium-channel blocker for a generalized epilepsy can fail, or make absence and myoclonic seizures worse.

The dose and adherence

  • A trial counts only if the drug was taken at an adequate dose for long enough.

Triggers

  • Sleep deprivation and alcohol.

Why refer early

The evidence for surgery is strongest when it comes early, yet patients still reach it late. At one epilepsy surgery center, the mean time from diagnosis to referral for temporal lobe epilepsy was about 17 years in 1995–1998 and about 19 years in 2005–2008, no shorter after the 2001 randomized trial and the 2003 practice parameter below (Haneef 2010).

Referral delay (Haneef 2010), the two surgical trials (Wiebe 2001; Engel 2012) and the 2022 consensus (Jehi 2022), all cited below.

Evidence

Wiebe 2001: surgery or a further year of medication, 80 patients with temporal lobe epilepsy
At 1 year, 58% of the surgical group were free of seizures impairing awareness, against 8% of the medical group (Wiebe 2001).
Early Randomized Surgical Epilepsy Trial (ERSET): early surgery, 38 patients with mesial temporal lobe epilepsy disabling for no more than 2 years after two drugs failed
In the second year, 11 of 15 (73%) after surgery were free of disabling seizures, against none of 23 who continued medication. The trial stopped early because recruitment was too slow, not because of its results (Engel 2012).

The American Academy of Neurology practice parameter (Level A) says that patients with disabling focal seizures with impaired consciousness who have failed appropriate first-line drugs should be considered for referral to an epilepsy surgery center (Engel 2003). The 2022 ILAE expert consensus goes further: referral for a surgical evaluation should be offered to every patient with drug-resistant epilepsy up to 70 years of age as soon as drug resistance is established, whatever the seizure type, epilepsy type, duration or comorbidities (Jehi 2022). Referral is for an evaluation; it does not commit anyone to surgery.

Uncontrolled seizures also carry a risk of death. Sudden unexpected death in epilepsy (SUDEP) affects about 1 in 1,000 adults and 1 in 4,500 children with epilepsy each year. Generalized tonic–clonic seizures are its major risk factor, and the risk rises with their frequency. The American Academy of Neurology and American Epilepsy Society guideline asks clinicians to give patients, or the parents of young children, these numbers; to keep working to reduce seizures in anyone who still has tonic–clonic seizures; and to explain that freedom from seizures, particularly tonic–clonic seizures, is strongly associated with a lower risk (Level B; Harden 2017).

The yearly risk as the guideline asks clinicians to put it (Harden 2017).

Key points

  • Drug-resistant epilepsy is failure of two tolerated, appropriate and adequately used medication schedules.
  • After two failures, further drugs rarely bring lasting seizure freedom; refer to an epilepsy center.
  • Referral is for evaluation; it does not commit anyone to surgery.

Quick check A 28-year-old woman with focal seizures with impaired consciousness has had monthly seizures despite adequate trials of lamotrigine and then levetiracetam, both taken reliably. What is the best next step?

Sources: as cited; the pathway figure follows Kwan 2010.

Presurgical evaluation and stereo EEG

Clinical pathway

Before anyone operates, the epilepsy team has to agree on where the seizures begin and what that cortex does. No single test answers either question.

What the evaluation looks for

The aim is to find the epileptogenic zone, the cortex whose removal or disconnection stops the seizures, and to map the function around it. No test shows it directly. Each test defines a different zone, the team infers the epileptogenic zone from where they overlap, and it is only proven when the patient is seizure-free after surgery (Rosenow & Lüders 2001).

Schematic zones on a lateral view of one hemisphere, after the zone concept of Rosenow & Lüders 2001.
From the noninvasive workup to a treatment decision. The more the tests agree, the better the chance of seizure freedom.
ZoneWhat it isMain test
Seizure-onset zoneWhere recorded seizures beginIctal scalp or intracranial EEG
Irritative zoneWhere interictal spikes arise; often larger than the onset zoneEEG, magnetoencephalography
Symptomatogenic zoneCortex that produces the first symptoms; it can lie away from the onsetVideo of the seizures (semiology)
Epileptogenic lesionThe structural abnormalityEpilepsy-protocol magnetic resonance imaging (MRI)
Functional deficit zoneCortex that works abnormally between seizuresExamination, neuropsychology, fluorodeoxyglucose positron emission tomography
Eloquent cortexCortex that must be spared, such as language and motor areasFunctional MRI, Wada test, electrical stimulation mapping

Stereo EEG (SEEG)

When the noninvasive results disagree, show no lesion, suggest a deep or bilateral onset, or point to cortex near language or motor areas, the team states a hypothesis about where seizures start and how they spread, and tests it with depth electrodes. Each lead passes through a small drill hole along a trajectory planned on MRI and vascular imaging to avoid vessels, and is placed with a stereotactic frame or robot. About a dozen leads is typical: 902 electrodes in 76 implantations in one series (van der Loo 2017).

What it records

  • A typical electrode (DIXI Microdeep) is 0.8 mm across, with 5 to 18 contacts, 2 mm long and 1.5 mm apart, numbered from the deepest; other makes differ slightly.
  • One lead samples a deep target and the cortex it passes through; both hemispheres can be sampled in one implant.

In the monitoring unit

  • Antiseizure medications are reduced to record habitual seizures.
  • Electrical stimulation through pairs of contacts maps function and can reproduce the patient’s aura or seizure.

Compared with subdural grids and strips

  • Stereo EEG needs no craniotomy and reaches sulcal depths, the insula, the cingulate and the mesial temporal structures.
  • Grids cover a continuous area of surface cortex, which helps functional mapping of the convexity.

Safety

  • Meta-analysis, pooled and each estimated separately: any complication 1.3%, hemorrhage 1.0%, infection 0.8%.
  • One center: major complications after 2.6% of implantations.

Sources: Mullin 2016; van der Loo 2017.

What comes next

  • Resection or laser ablation.
  • Radiofrequency thermocoagulation through the stereo EEG contacts.
  • Responsive neurostimulation leads at onset zones that cannot be removed.

Lead names

  • Side, internal target and external entry region.
  • At Mount Sinai, LHplT is the left lead from the hippocampus to the lateral temporal cortex; see the stereo EEG lead naming reference.
A coronal slice through the hippocampal bodies, seen from the front with the patient’s left on the right. Original schematic, not a patient implant; the insular and cingulate leads are drawn in this one plane for teaching.

Key points

  • The epileptogenic zone is inferred from where the tests agree, and proven only by seizure freedom after surgery.
  • Stereo EEG tests a stated hypothesis with depth electrodes when noninvasive results disagree or point to deep or eloquent cortex.
  • Stimulation through the contacts maps function and can reproduce the habitual aura.

Quick check Scalp video-EEG suggests left temporal onset, but the MRI is normal and positron emission tomography points to the insula. What is the usual next step?

Surgery, devices and diet

Evidence

For drug-resistant epilepsy the choice is between removing or disconnecting the source, modulating the network, and dietary or syndrome-specific treatment. Only removing, ablating or disconnecting a localized source regularly brings freedom from seizures.

The three families of treatment, from the trials and labels cited in the cards below.

Remove or disconnect the source

Surgery and ablation

  • Resection: lesionectomy, lobar or multilobar resection, or hemispherotomy or hemispherectomy when seizures arise from one whole hemisphere.
  • Laser interstitial thermal therapy guided by magnetic resonance imaging (MRI), for a well-localized onset.
  • Corpus callosotomy may reduce drop seizures (tonic or atonic seizures with falls).
  • In the randomized trial of temporal lobe surgery (patients aged 16 and older), 58% were free of seizures with impaired consciousness at 1 year, against 8% who continued medication.

Source: Wiebe 2001.

Modulate the network

Vagus nerve stimulation

  • Approved by the US Food and Drug Administration in 1997; since 2017 labeled for focal seizures from age 4; also used off label for generalized epilepsies such as Lennox–Gastaut syndrome.
  • Pivotal trial: mean seizure frequency fell 28% with high against 15% with low stimulation at 3 months.
  • Registry of 5,554 patients: responders (at least 50% fewer seizures) rose from 49% at 0–4 months to 63% at 24–48 months; about 8% became seizure-free.

Sources: Handforth 1998; Englot 2016.

Responsive neurostimulation

  • Approved in 2013 for adults with one or two seizure foci.
  • Pivotal trial: mean seizure frequency fell 37.9% against 17.3% with sham in the 12-week blinded period.
  • Open label: median reduction 75% at 9 years; 18% had at least one year free of seizures.

Sources: Morrell 2011; Nair 2020.

Deep brain stimulation

  • Approved in 2018 for adults whose focal seizures have failed three or more antiseizure medications.
  • Bilateral stimulation of the anterior nucleus of the thalamus: a median 40.4% reduction against 14.5% with sham at the end of the blinded phase.
  • Open label: median reductions of 41% at 1 year, 69% at 5 years and 75% at 7 years.

Sources: Fisher 2010; Salanova 2015; Salanova 2021.

All three are palliative: the benefit grows over years and seizure freedom is uncommon. Diathermy is contraindicated with each device, and MRI is possible only under the manufacturer's conditions for the implanted system.

Diet and syndrome-specific treatment

Dietary therapy

  • Ketogenic diet, or a less restrictive version such as the modified Atkins diet or low glycemic index treatment, in children and adults.
  • The treatment of choice for glucose transporter type 1 (GLUT1) deficiency and pyruvate dehydrogenase deficiency.
  • In a randomized trial in 145 children with daily seizures despite two or more drugs, 38% on the diet against 6% of controls had more than 50% fewer seizures at 3 months.

Sources: Kossoff 2018; Neal 2008.

Syndrome-specific and other treatment

  • Cannabidiol: Lennox–Gastaut syndrome, Dravet syndrome, tuberous sclerosis complex.
  • Fenfluramine: Dravet and Lennox–Gastaut syndromes. Stiripentol: Dravet syndrome.
  • Everolimus: tuberous sclerosis complex. Ganaxolone: cyclin-dependent kinase-like 5 (CDKL5) deficiency disorder.
  • Immunotherapy when an autoimmune cause is suspected, and clinical trials.

Neuromodulation devices: where they sit

A front view, so the patient's left is on the right of the drawing. Choose a device for its parts, its target, the trial results and when it stimulates; point at or tab to a part for what it is.

Pivotal trials: seizure reduction, active versus control

Pivotal trials of vagus nerve stimulation (Handforth 1998), responsive neurostimulation (Morrell 2011) and deep brain stimulation (Fisher 2010), and open-label follow-up (Nair 2020; Salanova 2021). The control arm was low-level stimulation in the vagus nerve trial and sham stimulation in the other two; the trial results are mean reductions for vagus nerve and responsive stimulation and the median for deep brain stimulation.

What the devices look like

Original drawings for teaching, not photographs or patient films. The hardware is drawn to scale within each picture from the manufacturers' published dimensions, except that the leads are shortened and the vagus nerve coils and the deep brain stimulation lead tip are also shown enlarged. The films show typical placements. The numbers on each picture match the list below it.

Key points

  • Resection or ablation is the treatment most likely to stop seizures when the onset is localized.
  • Vagus nerve, responsive and deep brain stimulation reduce seizures; their benefit grows over years and seizure freedom is uncommon.
  • The ketogenic diet is the treatment of choice for GLUT1 deficiency.

Quick check An adult has two independent mesial temporal seizure onsets on stereo EEG, one in each hippocampus. Which treatment fits best?

Sources: definition, International League Against Epilepsy (ILAE) task force (Kwan 2010); vagus nerve stimulation, American Academy of Neurology guideline update (Morris 2013); device indications from the US Food and Drug Administration approvals and the manufacturers' labeling; trial results as linked in each card.

Stopping antiseizure medication

Clinical

Many people who become seizure-free will ask whether they can stop. The decision trades a higher chance of recurrence against freedom from the medication, and it belongs to the patient once the risks are clear.

Key considerations for withdrawal

  • Use shared decision-making after an individualized review of epilepsy syndrome, seizure-free interval, prior course, treatment adverse effects, and consequences of recurrence
  • In adults seizure-free for at least 2 years, discuss that recurrence is possibly more likely after withdrawal; in the largest trial the excess was concentrated in the first 2 years, and no single waiting period suits everyone
  • In children seizure-free for 18–24 months, incorporate the syndrome's natural history and obtain an EEG when withdrawal is being considered
  • Plan the taper with the patient; in children, tapering by a quarter every 10 to 14 days was probably no different from tapering over 2 months, and the advisory recommends going no faster than that
  • Discuss driving, employment, injury, pregnancy, and rescue-plan implications; follow current jurisdiction-specific driving rules during and after tapering

The prediction tool below estimates an individual patient's risk of recurrence after withdrawal, to use in the discussion.

What the evidence shows

Evidence

Medical Research Council withdrawal study: 1,013 people seizure-free for at least 2 years
By 2 years, 41% had a recurrence after slow withdrawal against 22% who continued treatment; the gap narrowed afterwards. A longer seizure-free period lowered the risk; taking more than one drug and a history of tonic–clonic seizures raised it (Medical Research Council 1991). Of the 409 who relapsed, 95% had another year free of seizures within 3 years, and the study found no evidence that withdrawal changes the long-term prognosis (Chadwick 1996).
Akershus: double-blind withdrawal, 160 adults seizure-free for more than 2 years on one drug
At 12 months, 15% had relapsed after withdrawal against 7% who continued; the difference did not reach significance (Lossius 2008).
Individual-patient meta-analysis: 1,769 people who stopped treatment
46% had a recurrence, a few as late as 13 years after withdrawal, but only 9% were having seizures in their last year of follow-up. The model behind the prediction tool above uses the duration of epilepsy before remission, the seizure-free interval, age at onset, febrile seizures, the number of seizures before remission, whether the syndrome is self-limited, developmental delay and an epileptiform EEG before withdrawal (Lamberink 2017).
Medical Research Council 1991, with outcomes after relapse from Chadwick 1996; Akershus (Lossius 2008); individual-patient meta-analysis (Lamberink 2017).

The American Academy of Neurology advisory asks clinicians to cover these points (Gloss 2021):

Adults seizure-free for 2 years

  • Recurrence is possibly more likely after withdrawal.
  • There is a small chance medication will not regain control.
  • Recurrent seizures carry a risk of status epilepticus and death, although withdrawal possibly does not raise the risk of status epilepticus in adults.

Children seizure-free for 18 to 24 months

  • Withdrawal can be considered, guided by the natural history of the syndrome.
  • Obtain an EEG: interictal epileptiform activity possibly raises the risk of recurrence.
  • Taper no faster than a quarter every 10 to 14 days.
The trade-off the advisory asks clinicians to lay out (Gloss 2021).

Key points

  • Withdrawal after a seizure-free period roughly doubles the short-term chance of recurrence compared with continuing.
  • Individual risk depends on the epilepsy, its history and the EEG; a prediction model helps the discussion.
  • Driving, work and the consequences of a seizure often decide the choice.

Quick check A 34-year-old woman has been seizure-free for 2 years on one medication and asks to stop. What is the best way to approach it?

Sources: as cited. The online tool is the published model of Lamberink 2017.

Seizure clusters and rescue medicines

Clinical

Some people have seizures in bursts, closer together than their usual pattern. A written plan and a rescue benzodiazepine that needs no intravenous access let the family or caregivers act at once, which can help avoid progression to status epilepticus and visits to the emergency department.

No single definition

Studies have defined a seizure cluster as 3 or more seizures in 24 hours, 2 or more in 24 hours, or 2 or more in 6 hours (Jafarpour 2018). The UK National Institute for Health and Care Excellence (NICE) manages repeated or cluster seizures, typically 3 or more self-terminating seizures in 24 hours, as a medical emergency (NICE NG217). The US labels of the rescue medicines describe their target as intermittent, stereotypic episodes of frequent seizure activity, distinct from the patient's usual seizure pattern: the comparison is with the patient's own seizures.

Clusters are more likely in severe, poorly controlled epilepsy, and most studies come from tertiary epilepsy centers. They increase health care use and lower the quality of life of patients and caregivers, yet rescue medicines are underused (Jafarpour 2018).

Definitions and plans (Jafarpour 2018; NICE NG217) and the US labels of the four rescue medicines, by route and labeled age, without doses.

The written plan

What the plan says

  • Which rescue medicine, and by which route.
  • When to give it: what a cluster looks like for this patient, against the usual pattern.
  • Who gives it: teach the patient and caregivers to recognize a cluster and use the medicine.
  • NICE asks for an individualised emergency management plan to be agreed after repeated or cluster seizures and followed when they happen, and for expert guidance if they recur.

Sources: Jafarpour 2018; NICE NG217.

When to call for emergency help

  • A convulsive seizure lasting 5 minutes or more is status epilepticus and needs emergency treatment (Trinka 2015; see Treating convulsive status epilepticus).
  • The cluster looks different from earlier ones, or its frequency, severity or length, or the person's color or breathing, is alarming.
  • The written plan says what to do if the seizures do not stop after the rescue medicine; follow it.
  • Shallow or slowed breathing, breathing that stops, or excessive sleepiness, above all with opioids, alcohol or other central nervous system depressants.

Source: the diazepam nasal spray Medication Guide (DailyMed), except where cited.

Rescue medicines on the US label

All four are benzodiazepines for the acute treatment of seizure clusters in people with epilepsy. They are named here by route and labeled age only; doses come from the label and the prescriber.

Medicine and routeLabeled agesHow often, at most
Diazepam rectal gel (label)2 years and olderOne episode every 5 days; five episodes a month
Diazepam nasal spray (label)2 years and olderOne episode every 5 days; five episodes a month
Diazepam buccal film (label)2 to 5 yearsOne episode every 5 days; five episodes a month
Midazolam nasal spray (label)12 years and olderOne episode every 3 days; five episodes a month

Each label says the medicine is not recommended for chronic, daily use as an anticonvulsant, and each carries the boxed warning on concomitant use with opioids; abuse, misuse and addiction; and dependence and withdrawal reactions. Patients and caregivers mostly preferred non-rectal routes (Jafarpour 2018). In the UK, NICE names buccal midazolam or rectal diazepam as the first-line benzodiazepine in the community for a convulsive seizure of 5 minutes or more (NICE NG217).

Needing rescue medicine often is a signal about the epilepsy itself. The labels cap use at five episodes a month, clusters are more likely when epilepsy is poorly controlled, and NICE advises seeking expert guidance when clusters recur: ask whether the epilepsy is drug resistant (see Drug-resistant epilepsy).

Key points

  • A cluster has no single definition; the labels define it against the patient's own usual pattern, and NICE treats 3 or more seizures in 24 hours as an emergency.
  • Give every patient at risk a written plan naming the rescue medicine, when to use it and who gives it, and teach the caregivers.
  • Rescue medicines are for intermittent use, at most five episodes a month; needing them often calls for expert review of the epilepsy.

Quick check A 30-year-old with focal epilepsy on two medications uses his midazolam nasal spray for clusters about every week. What is the best next step?

Quick check A mother gives her son his rescue medicine for a cluster, as his plan says. Which finding means she should call for emergency help?

Sources: as cited. Labeled ages, frequency limits and warnings are from the US prescribing information on DailyMed; no doses are given.

Living with epilepsy: sudden death, supervision and safety

Clinical

Uncontrolled tonic–clonic seizures carry a risk of sudden death and of injury. Recordings of sudden deaths in monitoring units, and studies of the people who died, point to what lowers the risk: control of tonic–clonic seizures, and someone nearby at night.

How often, and how to tell patients

Sudden unexpected death in epilepsy (SUDEP) is the leading cause of death in chronic refractory epilepsy (Ryvlin 2013). The American Academy of Neurology and American Epilepsy Society guideline puts the risk at 0.22 per 1,000 patient-years in children and 1.2 per 1,000 in adults, and asks clinicians to tell parents that SUDEP typically affects 1 in 4,500 children with epilepsy a year, and adults that it affects 1 in 1,000 adults with epilepsy a year. Generalized tonic–clonic seizures are the major risk factor, and the risk rises with their frequency; freedom from seizures, particularly tonic–clonic seizures, is strongly associated with a lower risk (Level B; Harden 2017). The numbers were introduced in Drug-resistant epilepsy; this lesson is about the mechanism and the night.

What the recordings show

MORTEMUS, a study of cardiorespiratory arrests in epilepsy monitoring units, surveyed the units of Europe, Israel, Australia and New Zealand, 147 of 160 of which responded, and invited units elsewhere to report cases. 29 cardiorespiratory arrests were reported, including 16 SUDEP, 14 of them at night. In the 10 deaths with cardiorespiratory recordings, a consistent sequence followed a generalized tonic–clonic seizure (Ryvlin 2013):

The sequence in the monitored deaths of MORTEMUS (Ryvlin 2013).
  1. Rapid breathing. After the seizure, 18 to 50 breaths a minute.
  2. Breathing and heart falter. Within 3 minutes of the seizure's end, transient or terminal failure of breathing and heart function: an early postictal, centrally mediated shutdown.
  3. Terminal apnea, then cardiac arrest. Where the failure was transient, it recurred: terminal apnea within 11 minutes of the end of the seizure, followed by cardiac arrest.

In adult monitoring units, SUDEP occurred at 5.1 per 1,000 patient-years, about 1.2 per 10,000 video-electroencephalography (video-EEG) monitorings, probably aggravated by suboptimal supervision and possibly by withdrawal of antiseizure medication. The authors called for better supervision in monitoring units, particularly at night (Ryvlin 2013).

Who is at risk

Two case-control studies show that the danger is a tonic–clonic seizure with no one there.

Odds of SUDEP in a Swedish nationwide case-control study (Sveinsson 2020) and protection by supervision at night in a UK one (Langan 2005).
FactorOdds of SUDEPStudy
Generalized tonic–clonic seizures in the past year27 timesSweden, 255 deaths
Only seizures without tonic–clonic onesNo excess risk (odds ratio 1.15)Sweden
Tonic–clonic seizures at night15 timesSweden
Living alone5 timesSweden
Tonic–clonic seizures and no one sharing the bedroom67 timesSweden
Tonic–clonic seizures in the past 3 months13.8 timesUK, 154 deaths
Supervision at nightOdds ratio 0.4UK
Someone in the same bedroom, or a listening deviceOdds ratio 0.1UK

The Swedish authors estimated that 69% of these deaths in people with tonic–clonic seizures who lived alone could be prevented if they were not unattended at night or were free of tonic–clonic seizures; a previous diagnosis of substance abuse or alcohol dependence also carried excess risk (Sveinsson 2020).

The International League Against Epilepsy (ILAE) and the International Federation of Clinical Neurophysiology recommend clinically validated wearable devices for detecting generalized and focal to bilateral tonic–clonic seizures, especially in people who are unsupervised, where an alarm can bring rapid help. The recommendation is weak: detection is accurate, but it is uncertain whether the alarms improve outcomes, and the devices are not recommended for other seizure types (Beniczky 2021).

Everyday safety

Water

  • In a meta-analysis of 51 cohorts, the risk of drowning in people with epilepsy was 15 to 19 times that of the general population.
  • It was highest in people in institutional care, with a learning disability or after temporal lobe surgery.
  • The authors ask that people with epilepsy and their carers be told.

Source: Bell 2008.

Exercise and sport

  • Exercise and sport may improve seizure control, with wider health and psychosocial benefits.
  • The ILAE task force sorts sports by the risk of injury or death if a seizure occurs: no significant extra risk; moderate risk to the person but none to bystanders; major risk.
  • Advice weighs the sport, the chance of a seizure, its type, severity, triggers and usual timing, and how much risk the person accepts.

Source: Capovilla 2016.

Driving

  • US driving rules for people with seizures are set by each state and vary widely: some set a single seizure-free period in law, others weigh individual clinical factors, and the physician's role and legal protection differ.
  • Check the current rules of the patient's own state before advising.

Source: survey of the 50 states and the District of Columbia (Krauss 2001).

Key points

  • SUDEP typically affects 1 in 1,000 adults and 1 in 4,500 children with epilepsy a year; tell patients and families, with the numbers.
  • In monitored deaths, breathing and heart function failed within minutes of a tonic–clonic seizure, mostly at night.
  • Tonic–clonic seizures with no one nearby carry the highest risk; seizure control lowers it most, and supervision at night protects.

Quick check A 26-year-old man with focal epilepsy has about four focal to bilateral tonic–clonic seizures a year, mostly from sleep. He lives alone. What lowers his risk of SUDEP most?

Quick check In the monitored deaths of MORTEMUS, what was the usual sequence?

Quick check The parents of a 7-year-old with epilepsy ask how often children with epilepsy die suddenly. What does the guideline ask you to tell them?

Sources: as cited. SUDEP incidence and counseling follow the American Academy of Neurology and American Epilepsy Society guideline (Harden 2017).

Hormones and the menstrual cycle

Clinical

Sex hormones and antiseizure medications act on each other in both directions, and the questions change across a lifetime: the menstrual cycle, contraception, planning and carrying a pregnancy, breastfeeding, gender-affirming hormones and menopause. Most people with epilepsy who plan a pregnancy have a healthy one. Do not stop an antiseizure medication abruptly because of pregnancy.

Most of the evidence comes from cisgender women, so this topic says “women”. The same principles apply to anyone who menstruates, can become pregnant or takes sex hormones, including transgender and nonbinary people.

Across the lifespan

What to think about at each stage. Each theme is covered below.

How hormones, seizures and antiseizure medications interact

Estrogen tends to excite neurons and progesterone to calm them, through its metabolite allopregnanolone at the gamma-aminobutyric acid type A (GABA-A) receptor. Seizures cluster when estrogen is high relative to progesterone. Separately, many antiseizure medications change hormone levels, and hormones change the levels of antiseizure medications.

Point at or tab to an arrow for its explanation.

Catamenial epilepsy

Catamenial epilepsy is a cyclical worsening of seizures tied to the menstrual cycle. Herzog's working definition is a twofold or greater rise in the average daily seizure frequency in one phase of the cycle (Herzog 1997). In 87 women with focal epilepsy who charted three cycles, 39% met it in at least two of the three (Herzog 2004).

Schematic hormone curves on a 28-day cycle. Day 1 is the first day of bleeding; days before the next bleed are counted back, so ovulation is day −14.
PatternDaysSeizures rise againstWhy
C1 perimenstrual−3 to +3Follicular and luteal daysProgesterone and allopregnanolone fall before menses
C2 periovulatory10 to −13Follicular and luteal daysThe preovulatory estrogen surge, not yet opposed by progesterone
C3 luteal, in inadequate luteal-phase cycles10 to +3Follicular days (4 to 9)Estrogen rises at midcycle but progesterone stays low

Making the diagnosis. Ask for a diary of seizures and bleeding days over two or three cycles. A midluteal (day 22) progesterone below 5 ng/mL marks an inadequate luteal phase. The calculator applies Herzog's comparisons to one charted cycle.

Enter seizure counts from one cycle. The day counts adjust to the cycle length; the ovulatory phase absorbs the difference.

Treatment

Optimize the everyday antiseizure medication first. Apart from one large negative trial of progesterone, the options below rest on small or uncontrolled studies. A Cochrane review found only four randomized trials that met its criteria (treatment for at least 12 weeks), all of progesterone or norethisterone (Maguire 2021).

OptionEvidence
Clobazam around menses, for about 10 daysDouble-blind crossover in 18 women: clobazam preferred by 14 (Feely 1982). Needs predictable cycles; clobazam may make hormonal contraception less effective.
Acetazolamide, intermittent or dailyRetrospective survey of 20 women: 40% reported at least 50% fewer seizures (Lim 2001).
A temporary rise in the usual antiseizure medication dose over the vulnerable daysCommon practice; not tested in trials.
Progesterone lozenges in the second half of the cycleNational Institutes of Health phase III trial, 294 women: no benefit overall. In a prespecified secondary analysis, women whose seizures at least tripled perimenstrually (about a fifth of the trial) responded more often: 37.8% vs 11.1% on placebo (Herzog 2012).
Suppressing the cycle: depot medroxyprogesterone, continuous hormonal contraception, gonadotropin-releasing hormone analoguesUncontrolled series only; for example, of 14 women with uncontrolled seizures given medroxyprogesterone, the 11 who developed amenorrhea averaged 30% fewer seizures a month, and 7 of them reported fewer seizures (Mattson 1984). Consider interactions and bone health.

Key points

  • Estrogen is proconvulsant; progesterone, through allopregnanolone, is anticonvulsant.
  • Catamenial epilepsy is a twofold or greater rise in seizures in one phase of the cycle; chart two or three cycles to show it.
  • Apart from one large negative trial of progesterone, every hormonal treatment for it rests on small or uncontrolled studies.

Quick check A woman's seizures cluster in the two days before menses and the first days of menses. What hormonal change best explains the C1 (perimenstrual) pattern?

Contraception

Clinical

An unplanned pregnancy carries more risk with epilepsy: the Centers for Disease Control and Prevention (CDC) lists epilepsy among the conditions in which pregnancy raises the risk of adverse health events, and a switch away from valproate or topiramate takes months to plan. Choose a method that the antiseizure medication does not weaken.

US Medical Eligibility Criteria for Contraceptive Use, 2024 (Centers for Disease Control and Prevention summary chart).

Enzyme inducers

  • Named by the CDC: phenytoin, carbamazepine, barbiturates, primidone, topiramate and oxcarbazepine.
  • By their US labels: eslicarbazepine, cenobamate, rufinamide and clobazam can make hormonal contraception less effective, as can perampanel and topiramate at higher doses.
  • Felbamate lowered the progestin of a combined pill, without hormonal evidence of ovulation.
  • They make pills, patches, rings and implants less reliable; intrauterine devices and depot medroxyprogesterone are unaffected.

Sources: US Medical Eligibility Criteria 2024; the antiseizure medications' US labels (felbamate label).

Lamotrigine works in both directions

  • Combined pills more than halved lamotrigine levels: 13 against 28 µmol/L.
  • In the hormone-free week levels rose by a median 27%.
  • Progestin-only methods did not change lamotrigine.
  • The other way: lamotrigine lowered levonorgestrel by 19%, without evidence of ovulation.

Sources: Sabers 2003; Contin 2006; Reimers 2005; Sidhu 2006.

Emergency contraception on an enzyme inducer

  • On an inducer, or within 4 weeks of stopping one: offer a copper intrauterine device.
  • If that is not possible, levonorgestrel at the guideline's adjusted dose can be considered, with unknown effectiveness.
  • Ulipristal is not recommended.

Source: the emergency contraception guideline of the Faculty of Sexual and Reproductive Healthcare (FSRH, now the College of Sexual and Reproductive Healthcare) (FSRH 2020, amended 2023).

Valproate and topiramate

  • Valproate requires highly effective contraception under the European and UK pregnancy prevention programmes.
  • The programmes now also cover topiramate: European Union from 2023, UK from June 2024.
Levels in two groups of women (Sabers 2003) and, schematically, the rise in the hormone-free week (Contin 2006); Reimers 2005 and Sidhu 2006 for the progestin side.

Key points

  • Enzyme-inducing medications make hormonal pills, patches, rings and implants less reliable; intrauterine devices and depot medroxyprogesterone are unaffected.
  • Combined estrogen methods lower lamotrigine levels, and levels rise again in the hormone-free week.
  • On an enzyme inducer, the emergency method of choice is a copper intrauterine device.

Quick check A 24-year-old taking carbamazepine wants reliable contraception. Which method is not weakened by her medication?

Quick check A 22-year-old whose focal seizures are controlled on lamotrigine starts a combined oral contraceptive with a 7-day hormone-free week. What happens to her lamotrigine level?

Pregnancy: planning and the nine months

Clinical

Most women with epilepsy who plan a pregnancy have a healthy one. The decisive work happens before conception, in choosing the medication and recording a baseline level, and continues with levels through the nine months.

Planning a pregnancy

The work before conception, from the 2024 American Academy of Neurology, American Epilepsy Society and Society for Maternal-Fetal Medicine guideline (Pack 2024) and the studies in the cards below.

Choose the antiseizure medication before conception

  • The 2024 guideline asks for medications and doses that optimize both seizure control and fetal outcome, at the earliest opportunity before conception.
  • Consider lamotrigine, levetiracetam or oxcarbazepine; avoid valproate when clinically feasible.
  • Never stop a drug abruptly.

Aim for seizure control first

  • Seizure freedom for at least 9 months before pregnancy was probably associated with an 84–92% chance of staying seizure-free during it.
  • From the 2009 American Academy of Neurology and American Epilepsy Society (AAN/AES) review, retired in 2025 for updating.

Source: AAN/AES 2009.

Folic acid, before conception and through pregnancy

  • The 2024 guideline sets a minimum equal to the usual prenatal supplement: Level B to lower neural tube defects, and Level A to possibly improve neurodevelopment.
  • The Royal College of Obstetricians and Gynaecologists (RCOG) advises a higher dose for all women with epilepsy through the first trimester. The best dose is uncertain.
  • Neurodevelopmental Effects of Antiepileptic Drugs study (NEAD): periconceptional folate, mean intelligence quotient (IQ) 108 against 101.
  • Maternal Outcomes and Neurodevelopmental Effects of Antiepileptic Drugs study (MONEAD): no neurodevelopmental signal of harm at higher doses.
  • A Nordic registry study linked high-dose folic acid with more childhood cancer in children of mothers with epilepsy (adjusted hazard ratio 2.7).

Sources: Pack 2024; NEAD, Meador 2013; Meador 2025; Vegrim 2022.

Fertility is usually normal

  • In the Women with Epilepsy: Pregnancy Outcomes and Deliveries study (WEPOD), women with epilepsy trying to conceive became pregnant within 12 months as often as controls: 60.7% against 60.2%.
  • 81.5% of pregnancies in each group ended in a live birth.

Source: WEPOD, Pennell 2018.

Record a baseline level, and enroll in a registry

During pregnancy

Convulsive seizures are the main danger to mother and baby, so the guideline's first priority is to minimize them (Level A). Death during the delivery hospitalization was 80 per 100,000 deliveries in women with epilepsy against 6 per 100,000 in women without, and preeclampsia, preterm labor and stillbirth were modestly more common (MacDonald 2015). In the UK, sudden unexpected death in epilepsy (SUDEP) caused 11 of 14 epilepsy-related maternal deaths in 2006–08 (Edey 2014).

Deaths during the delivery hospitalization in a US inpatient sample (MacDonald 2015) and UK epilepsy-related maternal deaths (Edey 2014).

Malformations and development

Guidance Medications Notes
Avoid when clinically feasible Valproate Dose-dependent risk of major malformations (neural tube, cardiac); lowers the intelligence quotient (IQ) and raises the risk of autism spectrum disorder. The guideline also says to avoid valproate and topiramate to reduce babies born small for gestational age.
Avoid for specific risks Phenobarbital, Topiramate Phenobarbital: cardiac malformations. Phenobarbital and topiramate: oral clefts. Topiramate: babies born small for gestational age. The guideline says to avoid them when clinically feasible alternatives exist.
Risk rises with dose Carbamazepine, Phenytoin Risk varies by medication and dose; higher-dose carbamazepine carried more than twice the odds of levetiracetam (Tomson 2018). The guideline's rationale calls the lowest appropriate dose reasonable practice.
Consider first Lamotrigine, Levetiracetam, Oxcarbazepine The 2024 guideline says to consider these to minimize major congenital malformations (Level A). No medication is universally safest for every epilepsy.
Major malformations with each monotherapy at conception in EURAP, the International Registry of Antiepileptic Drugs and Pregnancy, with 95% intervals from the counts (Battino 2024). The dose bands in the table below are the exposure bands of the registry's 2018 report (Tomson 2018).
MedicationLower exposure bandMiddle exposure bandHigher exposure band
Valproate≤650 mg/day: 6.3%651–1450 mg/day: 11.3%>1450 mg/day: 25.2%
Phenobarbital≤80 mg/day: 2.7%81–130 mg/day: 6.2%>130 mg/day: 11.7%
Carbamazepine≤700 mg/day: 4.5%—>700 mg/day: 7.2%
Lamotrigine≤325 mg/day: 2.5%—>325 mg/day: 4.3%

Malformation rate by daily dose at conception, EURAP 2018. The bands are registry exposure bands, grouping the doses women were already taking; they are not prescribing guidance. Dose dependence was significant for all four in 2018; the registry's 2024 analysis, in the chart above, reported a significant dose effect for valproate, phenobarbital and carbamazepine.

Intelligence quotient

  • At age 6, mean IQ after valproate exposure was 97, against 105 for carbamazepine and 108 for lamotrigine and phenytoin.
  • It fell with the valproate dose.

Source: NEAD, Meador 2013.

Autism spectrum disorder

  • US cohort: autism spectrum disorder by age 8 in 10.5% after valproate, 6.2% after topiramate, 4.1% after lamotrigine and 4.2% unexposed; after adjustment, only valproate raised the risk (hazard ratio 2.67).
  • Nordic registries: higher adjusted risks of autism spectrum disorder (hazard ratio 2.8) and intellectual disability (3.5) after topiramate as well, so the evidence for topiramate conflicts.

Sources: Hernández-Díaz 2024; Bjørk 2022.

Lamotrigine and levetiracetam

  • In MONEAD, where most mothers with epilepsy took one or both, their children's language and verbal scores matched those of children of healthy women at ages 2, 3 and 6.
  • Higher exposure was linked to lower scores on some measures, varying by medication (secondary analyses at ages 2 and 3; a primary analysis, by third-trimester blood levels, at age 6).
  • The guideline recommends developmental screening for every exposed child.

Sources: Meador 2021 (age 2); Meador 2023 (age 3); Meador 2025 (age 6).

Levels and seizures

Clearance rises through pregnancy and falls back within weeks of delivery. In MONEAD, 23% of pregnant women had more seizures with impaired consciousness, about the same as nonpregnant women with epilepsy (25%), but the pregnant women's doses were changed more than twice as often (74% vs 31%; Pennell 2020). Monitoring and adjusting doses is part of why.

Top: A schematic from published magnitudes; individual courses vary. With lamotrigine, seizures worsened when the level fell below 65% of the individual's target (Pennell 2008). Bottom: Pennell 2022.

Check levels

  • Against the prepregnancy baseline, through pregnancy, guided by each drug's pharmacokinetics; many centers check lamotrigine, levetiracetam and oxcarbazepine monthly.
  • Raise the dose when the level falls or seizures return (guideline, Level B).

Once she is pregnant

  • Be cautious about removing or replacing an antiseizure medication that controls tonic–clonic seizures (Level B).
  • The choice to avoid valproate or topiramate belongs before conception.

In labor

  • Continue antiseizure medications.
  • Treat a convulsive seizure promptly with the institution's pregnancy-aware protocol, and consider eclampsia.
Historical registry data (2006–2012)

Do not use this older table as a current counseling calculator. It reproduces selected results from 2006–2012 publications with different enrollment, comparator, dose, and outcome methods. Current counseling should use the 2024 American Academy of Neurology, American Epilepsy Society and Society for Maternal-Fetal Medicine guideline and the latest pregnancy-registry publications. The historical data remain useful for illustrating dose dependence and why estimates differ among registries.

Medication North American Antiepileptic Drug Pregnancy Registry (Hernández-Díaz 2012) UK and Ireland Epilepsy and Pregnancy Register (Morrow 2006) EURAP (Tomson 2011)
Lamotrigine 2.0% 3.2% 2.0% (<300mg)
4.5% (≥300mg)
Levetiracetam 2.4% 0% (small sample) -
Carbamazepine 3.0% 2.2% 3.4% (<400mg)
8.7% (≥1000mg)
Phenytoin 2.9% 3.7% -
Valproate 9.3% 6.2% 5.6% (<700mg)
24.2% (≥1500mg)
Topiramate 4.2% 7.1% -
Phenobarbital 5.5% - 5.4% (<150mg)
13.7% (≥150mg)
Unexposed / Control 1.1% 3.5% -

Key points

  • Choose the medication before conception: lamotrigine, levetiracetam and oxcarbazepine carry the lowest malformation risks; avoid valproate when clinically feasible.
  • Record a baseline level while seizures are controlled; clearance rises through pregnancy, so check levels against it.
  • Convulsive seizures are the main danger to mother and baby. Never stop a medication abruptly because of pregnancy.

Quick check A woman whose seizures are controlled on lamotrigine is 16 weeks pregnant and has had her first breakthrough seizure. What is the most likely cause?

Quick check A 27-year-old with generalized epilepsy, free of tonic–clonic seizures for 3 years on valproate, is first seen at 20 weeks of an unplanned pregnancy. What is the best plan for her medication?

After delivery, and later in life

Clinical

The questions continue after the birth: doses raised in pregnancy, breastfeeding and sleep, then bone health, gender-affirming hormones and menopause.

Delivery and after

A schematic level after delivery with the dose left at its pregnancy level; Royal College of Obstetricians and Gynaecologists guideline 68 (RCOG 2016).

Lower any dose raised in pregnancy

  • Plan the reduction before delivery and review it within 10 days of birth, as the Royal College of Obstetricians and Gynaecologists (RCOG) advises.
  • As clearance returns, watch for dizziness, diplopia and ataxia.

Source: RCOG 2016.

Sleep and safety

  • Protect sleep: night feeds can be shared, for example with expressed milk.
  • Handle the baby safely: change and feed on the floor or a low surface, use a very shallow baby bath, and do not bathe the baby alone.

Screen for depression

  • Peripartum depression: 26.7% of women with epilepsy against 18.9% without.
  • Anxiety: 22.4% against 14.8%.

Source: Bjørk 2015.

Vitamin K

  • Newborns receive intramuscular vitamin K routinely; RCOG specifically advises it after exposure to enzyme inducers.
  • In the 2009 American Academy of Neurology and American Epilepsy Society (AAN/AES) review, evidence was insufficient to support giving the mother vitamin K before delivery.

Sources: RCOG 2016; AAN/AES 2009.

Breastfeeding

Breastfeeding is encouraged. In the Neurodevelopmental Effects of Antiepileptic Drugs study (NEAD), children who were breastfed had an intelligence quotient (IQ) 4 points higher at age 6 than those who were not (Meador 2014). In the Maternal Outcomes and Neurodevelopmental Effects of Antiepileptic Drugs study (MONEAD) about half of infant blood levels were too low to measure; the median infant level was 28.9% of the mother's for lamotrigine and 5.3% for levetiracetam (Birnbaum 2020). Levetiracetam passes freely into milk, but infants eliminate it quickly (Johannessen 2005). With lamotrigine, watch the infant for rash, apnea, drowsiness, poor sucking and poor weight gain (lamotrigine label). Watch for sedation and poor feeding with phenobarbital, primidone and benzodiazepines.

Bone and reproductive health

Bone, sex hormones and perimenopause, from the studies in the cards below and in Perimenopause and menopause.

Valproate and polycystic ovary features

  • In a 12-month randomized comparison, new features of polycystic ovary syndrome (hyperandrogenism or ovulatory dysfunction) appeared in 54% on valproate and 38% on lamotrigine; the full syndrome in 9% against 2%.
  • Hyperandrogenism was more frequent with valproate mainly when it was started before age 26 (44% against 23%).
  • Features improved after switching to lamotrigine.

Sources: Morrell 2008; Isojärvi 1998.

Anovulatory cycles

  • Over three cycles: 10.9% of cycles in controls, 14.3% in focal epilepsy and 27.1% in idiopathic generalized epilepsy.
  • Women taking valproate, or within 3 years of taking it, were most affected.

Source: Morrell 2002.

Enzyme inducers and sex hormones

  • Carbamazepine, phenytoin and phenobarbital raise sex hormone–binding globulin and lower bioactive testosterone and estradiol.
  • Result: menstrual disorders in women and reduced sexual function in men.

Source: Isojärvi 2005.

Bone

  • Enzyme inducers and valproate are associated with lower bone density and more fractures (odds ratio 1.38 for enzyme inducers).
  • The National Institute for Health and Care Excellence (NICE) advises considering vitamin D and calcium for people at risk.
  • The timing of bone density scans is expert opinion, for example after 5 years of treatment or at menopause.

Source: Vestergaard 2004.

Gender-affirming hormone therapy

Evidence is thin. The only cohort with seizure data before and after starting hormones had 34 people: 21 on masculinizing and 13 on feminizing therapy. More had seizures only before starting hormones than only after (18 vs 6), and feminizing therapy made no significant difference (Blackman 2025). In a US inpatient database of 4,765 transgender women, of whom 254 used feminizing hormones, the hormones were not associated with seizures during hospitalization, with a wide confidence interval (Reddy 2026). The rest is extrapolated from contraception and menopause data, so plan changes with the endocrinologist and follow the individual closely.

Interactions that matter when someone on an antiseizure medication starts or changes gender-affirming hormones (Alick-Lindstrom 2025; World Professional Association for Transgender Health, Standards of Care 8).

Before and after a change

  • Keep a seizure diary.
  • Check lamotrigine and other relevant antiseizure medication levels.
  • On an enzyme inducer, check hormone levels and consider a non-inducing antiseizure medication.

Estrogen choice

  • The World Professional Association for Transgender Health advises against ethinyl estradiol.
  • It favors transdermal estradiol for people over 45 or with a past venous thromboembolism.
  • Hormone levels are usually checked every 3 months in the first year.

Bone, fertility and mood

  • Antiseizure medications and gonadotropin-releasing hormone agonists can both lower bone density.
  • Discuss fertility preservation before starting.
  • Screen for depression and suicidality: in a clinic cohort of 65 transgender and gender-diverse people with epilepsy, 68% had documented suicidal ideation and 29% had functional/dissociative seizures.

Source: Byrnes 2025.

Perimenopause and menopause

Seizures often worsen in perimenopause, when estrogen swings high relative to progesterone, especially in women with a catamenial pattern. After menopause the picture is mixed, and a past catamenial pattern predicted improvement (Harden 1999). Menopause came earlier in women with more seizures: at a mean 46.7 years with frequent seizures against 49.9 years with infrequent ones (Harden 2003).

Top: Harden 1999. Bottom: Harden 2006.

Hormone therapy

  • Conjugated estrogens with medroxyprogesterone worsened seizures in a dose-related way; the two women on lamotrigine had levels 25–30% lower.
  • No study has tested the regimens used today in women with epilepsy. Transdermal estradiol with micronized progesterone may have a better safety profile, based on general-population observational data; choose the regimen with the women's health specialist.
  • Check lamotrigine levels when hormone therapy starts or stops.

Sources: Harden 2006; Smith 2026.

Bone

  • In the Women's Health Initiative, postmenopausal women taking antiseizure medications had more fractures (hazard ratio 1.44; 1.36 for enzyme inducers).
  • Review vitamin D, calcium, falls and bone density.

Source: Carbone 2010.

Primary guidance: American Academy of Neurology, American Epilepsy Society and Society for Maternal-Fetal Medicine practice guideline on teratogenesis, perinatal and neurodevelopmental outcomes after in-utero antiseizure medication exposure (Pack 2024); US Medical Eligibility Criteria for Contraceptive Use of the Centers for Disease Control and Prevention (CDC 2024); RCOG Green-top Guideline 68. This topic supports counseling and does not replace individualized neurology, obstetric, endocrine and maternal-fetal-medicine care.

Key points

  • Plan before delivery how to lower any dose raised in pregnancy, and review it within days of the birth.
  • Breastfeeding is encouraged; infant blood levels are far below the mother's, about 5% with levetiracetam and about 29% with lamotrigine, so watch a lamotrigine-exposed infant.
  • Enzyme inducers and valproate affect bone and sex hormones; seizures often worsen in perimenopause.

Quick check A mother taking levetiracetam asks whether she can breastfeed. What does the evidence support?

Further reading

Reference

The guidelines and consensus statements this course draws on most, and one review for the history and pharmacology of the drugs.

Background reading

For how each antiseizure medication was found and how it acts, from bromide to cenobamate, read the review by Löscher and Klein 2021. It is a history and pharmacology review, not a prescribing guide: for the choice of a first drug, follow Choosing a first antiseizure medication and the guidelines below.

Guidelines and consensus statements

  • Definition of epilepsy: International League Against Epilepsy (ILAE) official report, a practical clinical definition (Fisher 2014).
  • First seizure in adults: American Academy of Neurology and American Epilepsy Society evidence-based guideline on management of an unprovoked first seizure (Krumholz 2015).
  • Choosing medications: National Institute for Health and Care Excellence (NICE) guideline NG217, epilepsies in children, young people and adults (NICE 2022, amended since, including in January 2025).
  • Status epilepticus: definition and classification (Trinka 2015); American Epilepsy Society treatment guideline for convulsive status (Glauser 2016); the Emergency Neurological Life Support (version 6.0) status epilepticus protocol, which also gives doses.
  • Critical care EEG: American Clinical Neurophysiology Society (ACNS) standardized critical care EEG terminology (Hirsch 2021) and consensus on continuous EEG in critically ill patients (Herman 2015).
  • Drug-resistant epilepsy: ILAE consensus definition (Kwan 2010).
  • Pregnancy: American Academy of Neurology, American Epilepsy Society and Society for Maternal-Fetal Medicine guideline on teratogenesis and outcomes after in-utero exposure (Pack 2024).

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