NeuroPCP · Primary Care Neurology & Epilepsy

The Epilepsy PathwayFrom "was that a seizure?" to a working diagnosis, a seizure type, and a first medication — organized the way the ILAE framework asks you to think.

Framework: ILAE 2017 classification Setting: Office / primary-care neurology Reading time: ~25 min
Educational reference only. This is a teaching document that summarizes published guidelines. It is not medical advice and does not replace clinical judgment, a full history and exam, specialist consultation, or current drug labeling. Doses, interactions, and contraindications must be verified against a primary source before any prescribing decision.
STEP 0

Is this event actually a seizure?

Most misdiagnosed "epilepsy" is something else entirely. Before you ever classify a seizure, decide whether the paroxysmal event was epileptic at all. The two big pretenders are syncope and psychogenic non-epileptic events.

The single most useful tool here is a careful witnessed history — a phone video of the event is worth more than any test you can order in clinic. Anchor the interview on what happened before, during, and after.

Differentiating the three common paroxysmal events
FeatureEpileptic seizureConvulsive syncopePsychogenic (PNES)
Trigger / prodromeSometimes aura (rising epigastric, déjà vu, focal sensory)Upright posture, pain, heat, standing; greyout, tunnel vision, warmth, nauseaEmotional context; often prolonged, fluctuating build-up
OnsetAbruptGradual over secondsVariable, may be gradual
Motor patternRhythmic, synchronous, decrementing; tonic then clonicBrief (<15 s) arrhythmic multifocal jerks after collapseAsynchronous, side-to-side head, pelvic thrust, waxing/waning, stop-start
EyesOpen, may deviateOpen, rolled upOften forcibly closed, resist opening
Duration1–3 min typicalSecondsOften >2 min, can be very long
Tongue biteLateral tongue (specific)Rare; tip if anyTip of tongue, lips
RecoveryPost-ictal confusion, gradual over minutesRapid, oriented within secondsVariable; may have rapid reorientation or prolonged unresponsiveness
IncontinenceCan occurCan occurCan occur — not discriminating
Watch the classic traps

Convulsive syncope is the most common false-positive. Brief jerks and even incontinence happen with any cause of cerebral hypoperfusion — they do not make the event a seizure. Ask what came first: collapse then jerks (syncope) or jerks then fall (seizure). PNES and epilepsy co-exist in a meaningful minority of patients; the presence of one does not exclude the other, and definitive diagnosis usually needs video-EEG.

STEP 1

Does this patient have epilepsy?

A single seizure is not epilepsy. The ILAE gives a practical, operational definition — and, importantly, an updated one that lets you diagnose epilepsy after a first unprovoked seizure when recurrence risk is high.

The ILAE 2014 operational definition

Epilepsy is a disease of the brain defined by any one of the following three conditions:

  1. At least two unprovoked (or reflex) seizures occurring more than 24 hours apart.
  2. One unprovoked seizure and a probability of further seizures similar to the general recurrence risk after two unprovoked seizures (at least ~60%) over the next 10 years.
  3. Diagnosis of an epilepsy syndrome (e.g., juvenile myoclonic epilepsy on the strength of history + EEG, even after one clinical event).

Provoked vs. unprovoked — this changes everything

An acute symptomatic (provoked) seizure occurs in close temporal relationship to an acute insult and does not by itself indicate epilepsy. Treat the cause, not the "epilepsy." Common provokers:

The "high-risk first seizure" call

Definition #2 is where primary-care neurology earns its keep. After a first unprovoked seizure, features that push recurrence risk toward or above 60% — and therefore justify a diagnosis and treatment — include an epileptiform EEG, a symptomatic lesion on MRI, a remote symptomatic cause (prior stroke, trauma, infection), and a nocturnal seizure. A first seizure with a normal EEG, normal imaging, and no clear cause carries roughly a 30–40% two-year recurrence risk — often watched rather than treated after shared decision-making.

The minimum first-seizure workup

Baseline evaluation after a first apparent unprovoked seizure
TestWhat it answersNote
History + witness / videoWas it a seizure? Focal features at onset?Highest yield of anything here
EEGEpileptiform activity → recurrence risk, classificationA normal routine EEG does not exclude epilepsy; sleep-deprived / repeat raises yield
MRI brain (epilepsy protocol)Structural lesion → focal epilepsy, surgical targetPreferred over CT except in the acute/emergent setting
LabsProvoked cause? (glucose, Na, Ca, Mg, renal, tox)Rules provocation in or out
ECGSyncope mimic, long-QT, arrhythmiaCheap, catches a dangerous mimic
STEP 2

Classify the seizure type

The ILAE 2017 framework classifies at three levels: seizure typeepilepsy typesyndrome. Everything starts with one question that colors the rest of the workup: where did the seizure begin?

The three onset categories

ONSET AXIS

Focal onset

Begins in networks limited to one hemisphere. Sub-classify by awareness (aware vs. impaired) and by first prominent feature (motor vs. non-motor). May evolve to a focal-to-bilateral tonic-clonic seizure.

ONSET AXIS

Generalized onset

Engages bilateral networks from the start. Awareness is not used to sub-classify (it's usually impaired). Split into motor (tonic-clonic, myoclonic, tonic, atonic) and non-motor / absence.

ONSET AXIS

Unknown onset

Used when onset isn't witnessed or is unclear. Still describe observable features (e.g., "unknown-onset tonic-clonic"). Reclassify later as data arrive.

Interactive classifier

Work through the same questions you'd ask a witness. This walks the ILAE branch points and lands on a seizure-type label. It is a teaching aid, not a diagnosis.

STEP 3

Classify the epilepsy type & look for a syndrome

Once you know the seizure type(s), the epilepsy type usually follows. The 2017 framework adds a category most primary-care neurologists under-use: combined generalized and focal epilepsy.

The four ILAE epilepsy types
Epilepsy typeTypical evidencePrescribing implication
FocalFocal seizures; focal epileptiform EEG; structural MRI lesionBroad list works; the "narrow-spectrum" sodium-channel agents are safe here
GeneralizedGeneralized seizure types; generalized spike-wave EEG; usually normal MRIChoose a broad-spectrum drug. Narrow-spectrum agents can worsen absence/myoclonus
Combined focal & generalizedBoth seizure types (e.g., Dravet, Lennox-Gastaut)Broad-spectrum only
UnknownInsufficient information / normal EEG with unclear semiologyDefault to broad-spectrum to avoid aggravation
The single most useful safety heuristic

When you are unsure whether an epilepsy is focal or generalized, prescribe as if it were generalized and pick a broad-spectrum agent. Narrow-spectrum sodium-channel blockers (carbamazepine, oxcarbazepine, phenytoin, and to a degree gabapentin/pregabalin) can aggravate absence and myoclonic seizures. The cost of guessing "generalized" wrong is small; the cost of guessing "focal" wrong can be worsened seizures.

Common syndromes worth recognizing on sight

A syndrome bundles seizure type(s), age of onset, EEG signature, and prognosis into a package that often dictates drug choice directly.

ONSET 12–18 y

Juvenile myoclonic epilepsy

Early-morning myoclonic jerks (dropped coffee cup), generalized tonic-clonic seizures, sometimes absences. Photosensitive. Sleep deprivation & alcohol provoke.

First lineValproate (avoid in people who can become pregnant), levetiracetam, lamotrigine. Usually lifelong.
ONSET 4–10 y

Childhood absence epilepsy

Brief (5–10 s) staring spells, dozens/day, provoked by hyperventilation; 3 Hz generalized spike-wave. Normal development.

First lineEthosuximide (absence only) or valproate; lamotrigine second. Often remits by adolescence.
ONSET 3–13 y

Self-limited epilepsy with centrotemporal spikes

Nocturnal focal seizures — hemifacial twitching, drooling, speech arrest — from sleep. Centrotemporal spikes on EEG. Benign, self-limited.

Often no drugTreat only if frequent/daytime; low-dose narrow-spectrum agents fine. Remits by mid-teens.
ADULT, ANY AGE

Mesial temporal lobe epilepsy

Focal impaired-awareness seizures with rising epigastric aura, déjà vu, automatisms. Hippocampal sclerosis on MRI. Frequently drug-resistant.

First lineAny focal-appropriate agent; refer early for surgical evaluation if drug-resistant — high cure rate.
STEP 4

Reading the EEG signatures

The EEG doesn't diagnose epilepsy on its own, but a handful of interictal patterns are strongly tied to specific classifications. This viewer animates the waveform morphology behind the words in your report.

◉ REC · Simulated single-channel EEG
Report language → what it points to
  • Generalized 3 Hz spike-and-wave: typical absence; classic in childhood absence epilepsy.
  • Generalized polyspike-wave, often photosensitive: juvenile myoclonic epilepsy and other generalized epilepsies.
  • Focal sharp waves / spikes: focal epilepsy; localization hints at the seizure network (e.g., temporal, centrotemporal).
  • Hypsarrhythmia: chaotic high-voltage disorganization — the signature of infantile epileptic spasms; a can't-miss, refer-today pattern.
  • Normal posterior-dominant α rhythm: reassuring but never rules epilepsy out — sensitivity of a single routine EEG is only ~30–50%.
STEP 5

Choosing a first medication

Drug choice follows classification, then gets tuned to the person — age, sex and pregnancy potential, comorbidities, other medications. Pick a rational agent, start low, titrate slowly, aim for monotherapy at the lowest effective dose.

Two non-negotiable safety gates before any prescription
  • Pregnancy potential: valproate is teratogenic and impairs neurodevelopment — avoid in anyone who could become pregnant unless no alternative exists and under specialist oversight. Lamotrigine and levetiracetam are the usual preferred broad-spectrum choices in this group.
  • HLA-B*15:02: in patients of Southeast/East Asian ancestry, screen before carbamazepine (and consider before oxcarbazepine/phenytoin) for risk of Stevens-Johnson syndrome / toxic epidermal necrolysis.
Epilepsy / seizure classification
Key patient factor

Broad- vs. narrow-spectrum: the mental model

Broad-spectrum — safe across the board

Cover both focal and generalized seizures. When onset is uncertain, these are the safe default: lamotrigine, levetiracetam, valproate, topiramate, zonisamide, lacosamide (adjunct), perampanel, brivaracetam.

Narrow-spectrum — focal only

Effective for focal seizures but can worsen absence and myoclonic seizures: carbamazepine, oxcarbazepine, phenytoin, gabapentin, pregabalin, eslicarbazepine, vigabatrin. Don't reach for these when the epilepsy might be generalized.

Universal titration principles
  • Monotherapy first. ~50% are controlled on the first well-chosen drug; another ~15% on the second.
  • Start low, go slow — especially lamotrigine, where fast titration drives serious rash. Slow titration is a safety measure, not a delay.
  • Titrate to effect or to tolerability, not to a target level. Levels guide adherence and toxicity, not the goal.
  • If the first two appropriate drugs fail at adequate doses → drug-resistant epilepsy. That's a referral trigger, not a third-drug trigger.
STEP 6

Status epilepticus — the time-critical branch

A convulsive seizure lasting ≥5 minutes, or repeated seizures without recovery of awareness between them, is status epilepticus. This is a neurological emergency where the algorithm is a clock, not a decision tree.

0–5 min · Stabilization

ABCs, clock, glucose

  • Airway, oxygen, IV access, continuous monitoring. Note the time.
  • Check fingerstick glucose; treat hypoglycemia (with thiamine first if malnourished/alcohol).
  • Send labs: electrolytes, Ca/Mg, renal, LFTs, AED levels, toxicology, consider ABG.
5–20 min · First-line: benzodiazepine

Give an adequately-dosed benzodiazepine — under-dosing is the classic error

  • IM midazolam (no IV), IV lorazepam, or IV diazepam at weight-appropriate doses.
  • May repeat once. Do not stall on this step — a second full dose beats waiting.
20–40 min · Second-line: IV AED

Load a second-line agent if seizures continue

  • Choices with comparable efficacy: IV levetiracetam, IV fosphenytoin, or IV valproate.
  • Choose by comorbidities and interactions; any one of the three is a reasonable first pick.
40+ min · Refractory status

ICU, continuous EEG, anesthetic infusion

  • Refractory status → continuous infusion (midazolam, propofol, or pentobarbital) with intubation.
  • Continuous EEG monitoring, because ongoing seizures can become non-convulsive.
STEP 7

When to escalate or refer

Primary-care neurology handles the common, well-classified epilepsies. These situations should route to epileptology, video-EEG monitoring, or a comprehensive epilepsy center.

Referral triggers
TriggerWhy
Drug-resistant epilepsyFailure of two tolerated, appropriately chosen and dosed regimens. Surgery, neurostimulation, and dietary therapy become options with high value in the right patient.
Diagnostic uncertaintyEvents that could be PNES, or seizures that won't classify — video-EEG resolves both.
Structural lesionMRI shows a resectable focus (e.g., hippocampal sclerosis, focal cortical dysplasia).
Pregnancy / planningRegimen optimization and folate before conception; specialist co-management.
Infantile spasms / hypsarrhythmiaEmergency — time-sensitive treatment protects development.
SUDEP risk counselingPoorly-controlled convulsive seizures carry real mortality; the conversation belongs in the plan.

A reproducible classifier, in code

The onset-first logic above expressed as a small GNU Octave function — a compact way to encode the branch points so the reasoning is explicit and testable rather than implicit.

% classify_seizure.m — teaching model of the ILAE 2017 onset-first branch
% Inputs are strings; returns a seizure-type label. Educational only.
function label = classify_seizure(onset, awareness, feature)
  onset     = lower(onset);      % 'focal' | 'generalized' | 'unknown'
  awareness = lower(awareness);  % 'aware' | 'impaired' | 'na'
  feature   = lower(feature);    % 'motor' | 'nonmotor' | 'tonicclonic' | 'absence' | 'myoclonic'

  switch onset
    case 'focal'
      aw = 'aware';
      if strcmp(awareness,'impaired'), aw = 'impaired awareness'; end
      label = sprintf('Focal %s, %s onset', aw, feature);
      if strcmp(feature,'tonicclonic')
        label = 'Focal to bilateral tonic-clonic';
      end
    case 'generalized'
      switch feature
        case 'absence',     label = 'Generalized non-motor (absence)';
        case 'myoclonic',   label = 'Generalized motor: myoclonic';
        case 'tonicclonic', label = 'Generalized motor: tonic-clonic';
        otherwise,          label = 'Generalized motor (specify)';
      end
    otherwise
      label = sprintf('Unknown-onset %s', feature);
  end
endfunction

% >> classify_seizure('focal','impaired','motor')
% ans = Focal impaired awareness, motor onset
% >> classify_seizure('generalized','na','absence')
% ans = Generalized non-motor (absence)