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Epilepsy & Seizures

Medicine · Neurology · lean revision notes

Epilepsy & Seizures

A perennial NEET PG favourite that cuts across Medicine, Pharmacology and Paediatrics. Mastery of the ILAE classification, age-specific EEG signatures, drug-of-choice by seizure type, and the status epilepticus protocol will reliably fetch you 2–4 questions every exam cycle.

Definitions & key concepts

A seizure is a transient occurrence of signs/symptoms due to abnormal, excessive or synchronous neuronal discharge in the brain. Epilepsy is a disease of the brain defined by ANY of the following (ILAE 2014 operational definition):

  1. At least two unprovoked (or reflex) seizures occurring >24 hours apart, OR
  2. One unprovoked seizure with a probability of recurrence ≥60% over the next 10 years (e.g. a remote symptomatic seizure with an old stroke + epileptiform EEG), OR
  3. Diagnosis of an epilepsy syndrome.

High-yield: A single provoked seizure (hyponatraemia, hypoglycaemia, alcohol withdrawal, fever, eclampsia, drug toxicity) is NOT epilepsy. Correct the trigger — do not commit to long-term antiepileptic drugs (AEDs).

Epileptogenesis involves an imbalance favouring excitation (glutamate, NMDA/AMPA) over inhibition (GABA-A, GABA-B). Common substrates: hippocampal sclerosis (mesial temporal lobe epilepsy — the commonest cause of refractory focal epilepsy in adults), cortical dysplasia, tumours, gliosis post-trauma/stroke/infection (neurocysticercosis is the leading cause of acquired epilepsy in India).

Classification (ILAE 2017)

The 2017 framework classifies by onset:

Onset type Subtypes Awareness Old terminology
Focal Focal aware / Focal impaired awareness; motor vs non-motor onset; focal-to-bilateral tonic-clonic Preserved or impaired Simple partial / Complex partial / Secondarily generalised
Generalised Tonic-clonic, absence (typical/atypical), myoclonic, tonic, atonic, clonic Always impaired Grand mal / Petit mal etc.
Unknown onset Tonic-clonic, epileptic spasms, behaviour arrest

High-yield: The terms "simple partial", "complex partial" and "secondarily generalised" are obsolete. Use focal aware, focal impaired awareness, and focal-to-bilateral tonic-clonic respectively.

Aura = focal aware seizure. It localises the focus: epigastric rising sensation / déjà vu / fear → mesial temporal lobe; visual flashes → occipital; tingling marching up a limb (Jacksonian march) → frontal/motor cortex; forced head/eye deviation → frontal eye field.

Focal vs generalised — distinguishing features

Feature Focal Generalised
Onset One hemisphere/region Bilateral networks from start
Aura Often present Absent
Post-ictal confusion / Todd's palsy Common Variable (absent in absence)
Consciousness May be preserved Lost (except myoclonic where brief)
EEG Focal spikes Generalised spike-wave
Imaging yield High (look for structural lesion) Usually normal

Important epilepsy syndromes (paediatric high-yield)

Syndrome Age Seizure type EEG DOC / note
West syndrome 3–8 months Epileptic (infantile) spasms — flexor "salaam" Hypsarrhythmia ACTH / vigabatrin (vigabatrin first if tuberous sclerosis)
Lennox-Gastaut 1–8 yrs Mixed: tonic, atonic, atypical absence Slow (<2.5 Hz) spike-wave Valproate, lamotrigine, rufinamide; refractory
Childhood absence epilepsy 4–10 yrs Typical absence 3 Hz generalised spike-wave (provoked by hyperventilation) Ethosuximide (or valproate)
Juvenile myoclonic epilepsy (Janz) Teens Early-morning myoclonic jerks ± GTCS 4–6 Hz polyspike-wave Valproate (lifelong); avoid carbamazepine
Benign rolandic (BECTS) 3–13 yrs Nocturnal focal, face/oropharynx Centrotemporal spikes Often no treatment; excellent prognosis
Dravet syndrome <1 yr Prolonged febrile, hemiclonic SCN1A mutation; avoid sodium-channel blockers

High-yield (EEG triad to memorise): 3 Hz spike-wave = absence; hypsarrhythmia = West; slow spike-wave <2.5 Hz = Lennox-Gastaut.

High-yield: In West syndrome, look for tuberous sclerosis (ash-leaf macules, cardiac rhabdomyoma) — vigabatrin is the drug of choice in that setting.

Etiology

Mnemonic "VITAMINS" for seizure causes: Vascular (stroke), Infection (meningitis, NCC, encephalitis), Trauma/tumour, Autoimmune (anti-NMDA-R encephalitis, anti-LGI1), Metabolic (↓Na⁺, ↓glucose, ↓Ca²⁺, ↓Mg²⁺, uraemia), Idiopathic/genetic, Neurodegenerative/congenital malformations, Substance (alcohol/benzodiazepine withdrawal, cocaine, tramadol, isoniazid → give pyridoxine).

Age clue: neonate → HIE, hypoglycaemia, hypocalcaemia, sepsis; child → febrile seizure, genetic; young adult → trauma, NCC; elderlystroke (commonest), tumour.

Clinical features

  • Generalised tonic-clonic (GTCS): sudden loss of consciousness → tonic stiffening (often a cry from forced expiration) → clonic jerking → flaccid post-ictal phase with confusion, tongue-biting (lateral), urinary incontinence, and post-ictal headache/myalgia. Lasts 1–3 min.
  • Absence: abrupt 5–10 s behavioural arrest with staring/eyelid flutter, no aura, no post-ictal confusion, immediate resumption of activity; provoked by hyperventilation.
  • Myoclonic: brief shock-like jerks, consciousness retained.
  • Atonic ("drop attacks"): sudden loss of tone → falls/injury.
  • Focal impaired awareness: automatisms (lip-smacking, fumbling), staring, post-ictal confusion.

Seizure vs syncope vs pseudoseizure (PNES)

Feature Epileptic seizure Syncope Psychogenic (PNES)
Trigger Often none Posture, pain, micturition Emotional/stress
Onset Sudden Pre-syncope (lightheaded) Gradual, situational
Movements Rhythmic, synchronous Few myoclonic jerks Asynchronous, pelvic thrust, side-to-side head, eyes closed
Tongue bite Lateral Rare/tip Rare
Post-ictal Confusion, ↑prolactin (early) Rapid recovery No confusion, normal prolactin
EEG (ictal) Epileptiform Normal Normal during event

High-yield: Serum prolactin rises within 10–20 min after GTCS/focal-impaired-awareness seizures and helps differentiate true seizure from PNES — but a normal level does not exclude a seizure, and it is unreliable for absence/myoclonic types.

Diagnosis & investigation of choice

A first-seizure workup answers two questions: Was it a seizure? and Why?

Stepwise approach: History (eye-witness account is key) rule out provoked causes (glucose, Na⁺, Ca²⁺, Mg²⁺, toxicology, pregnancy test) EEG (best test to characterise seizure type / epileptiform discharges) MRI brain (investigation of choice for structural cause; far superior to CT for hippocampal sclerosis, dysplasia, low-grade tumours) LP if infection suspected.

  • EEG is the investigation of choice to classify the epilepsy and detect epileptiform activity; sensitivity rises with sleep deprivation, hyperventilation, photic stimulation and repeat/prolonged video-EEG. A normal interictal EEG does not exclude epilepsy.
  • MRI brain is the imaging investigation of choice for aetiology (epilepsy protocol). CT is reserved for the acute/emergency setting (bleed, gross lesion) and where MRI is unavailable.
  • Video-EEG monitoring is the gold standard to confirm PNES and for pre-surgical localisation.

High-yield: First single unprovoked seizure with a normal EEG, normal MRI and normal neuro exam → recurrence risk is low; AEDs are generally NOT started after one such seizure. Treat after a second seizure or when recurrence risk is high.

Management / drug of choice

Drug-of-choice by seizure type

Seizure type First-line / DOC Alternatives Avoid (worsen seizures)
Focal (± to bilateral) Lamotrigine / levetiracetam (carbamazepine, oxcarbazepine) Lacosamide, valproate
Generalised tonic-clonic Valproate (lamotrigine, levetiracetam) Topiramate
Absence Ethosuximide (valproate) Lamotrigine Carbamazepine, phenytoin, vigabatrin, gabapentin
Myoclonic / JME Valproate (levetiracetam) Topiramate, clonazepam Carbamazepine, phenytoin, gabapentin
Atonic / Lennox-Gastaut Valproate Lamotrigine, rufinamide
Infantile spasms (West) ACTH / vigabatrin Prednisolone

High-yield: Sodium-channel blockers (carbamazepine, oxcarbazepine, phenytoin) and GABAergic agents (vigabatrin, gabapentin, pregabalin, tiagabine) can WORSEN absence and myoclonic seizures. A classic NEET PG trap: giving carbamazepine in JME aggravates myoclonus.

High-yield: Ethosuximide acts on T-type calcium channels in thalamic neurons → drug of choice for absence only (no effect on GTCS; if both coexist, use valproate).

Principle of monotherapy: Start one drug at low dose, titrate up; ~50% are controlled on the first drug. If it fails at maximal dose, substitute a second monotherapy before resorting to polytherapy. Drug-resistant (refractory) epilepsy = failure of 2 appropriately chosen, tolerated AEDs → refer for surgery (e.g. anterior temporal lobectomy for mesial temporal sclerosis), vagus nerve stimulation, or ketogenic diet.

Mechanisms (commonly asked)

  • Phenytoin, carbamazepine, lamotrigine, lacosamide → block voltage-gated Na⁺ channels.
  • Ethosuximide → blocks T-type Ca²⁺ channels.
  • Valproate → broad: Na⁺ channel + ↑GABA + T-type Ca²⁺ (broadest spectrum AED).
  • Benzodiazepines, barbiturates → enhance GABA-A (BZD ↑frequency of Cl⁻ channel opening; barbiturates ↑duration).
  • Vigabatrin → irreversibly inhibits GABA transaminase.
  • Levetiracetam → binds SV2A synaptic vesicle protein.
  • Topiramate → multiple (Na⁺ block, GABA, AMPA/kainate antagonism, carbonic anhydrase inhibition).
  • Perampanel → AMPA receptor antagonist.

Status epilepticus (SE)

Definition (operational, ILAE 2015): a seizure lasting ≥5 minutes (t1 — time to start treatment) or ≥2 seizures without recovery of consciousness between them. The older "30-minute" cut-off (t2 — risk of permanent injury) is for prognostic damage, not for when to treat. Convulsive SE is a medical emergency with mortality up to 20%.

Stepwise protocol (memorise the timeline):

  1. 0–5 min — Stabilise: ABC, oxygen, IV access, finger-stick glucose; if hypoglycaemic give dextrose (+ thiamine in alcoholics); check Na⁺/Ca²⁺/toxicology.
  2. 5–20 min — First-line (benzodiazepine): IV lorazepam 0.1 mg/kg (max 4 mg/dose, may repeat once). If no IV access → IM midazolam 10 mg or PR/buccal diazepam.
  3. 20–40 min — Second-line (load an AED): IV fosphenytoin (20 mg PE/kg) OR IV valproate (40 mg/kg) OR IV levetiracetam (60 mg/kg). The ESETT trial showed these three are equally effective (~half respond to any one).
  4. 40–60 min — Refractory SE: continuous infusion midazolam / propofol / thiopental under EEG monitoring + intubation/ICU.

Stepwise (one-line flow): Lorazepam fosphenytoin/valproate/levetiracetam anaesthetic infusion (midazolam/propofol) EEG-guided burst-suppression.

High-yield: IV lorazepam is the preferred first-line benzodiazepine (longer anticonvulsant duration than diazepam, which redistributes quickly). When no IV access, IM midazolam is at least as good (RAMPART trial). Don't give phenytoin first — it is second-line.

High-yield: Fosphenytoin is preferred over phenytoin: water-soluble prodrug, less tissue injury, no propylene glycol, can be given IM. Phenytoin must be given slowly (<50 mg/min) with cardiac monitoring; extravasation causes purple glove syndrome.

AED toxicity & teratogenicity (very high-yield)

Drug Hallmark adverse effects
Phenytoin Gum hyperplasia, hirsutism, coarse facies, cerebellar (nystagmus/ataxia), megaloblastic anaemia (↓folate), zero-order kinetics, DRESS, osteomalacia, fetal hydantoin syndrome
Carbamazepine SIADH/hyponatraemia, aplastic anaemia/agranulocytosis, SJS/TEN in HLA-B*1502 (test Asians), diplopia, auto-induction; teratogenic — neural tube defects
Valproate Hepatotoxicity, pancreatitis, hyperammonaemia, weight gain, tremor, alopecia, thrombocytopenia; MOST teratogenic — NTD, ↓IQ, autism
Lamotrigine SJS/TEN (esp. with rapid titration or valproate co-use — titrate slowly)
Levetiracetam Behavioural/mood changes, irritability, depression (give pyridoxine)
Topiramate Weight loss, word-finding difficulty, renal stones, glaucoma, oligohidrosis, metabolic acidosis
Vigabatrin Irreversible visual field constriction (peripheral)
Ethosuximide GI upset, SJS, hiccups
Phenobarbitone Sedation, hyperactivity in children, osteomalacia

High-yield (teratogenicity ladder): Valproate is the most teratogenic AED (neural-tube defects, reduced IQ, autism spectrum). AVOID valproate in women of childbearing potential. Preferred AEDs in pregnancy: lamotrigine and levetiracetam. All women on AEDs should take folic acid 5 mg/day pre-conception.

High-yield: Enzyme-inducing AEDs (phenytoin, carbamazepine, phenobarbitone, topiramate at high dose) reduce efficacy of oral contraceptives — counsel accordingly. Valproate, levetiracetam, lamotrigine are non-inducers.

Complications

  • SUDEP (Sudden Unexpected Death in Epilepsy) — main risk factor is frequent uncontrolled GTCS, especially nocturnal.
  • Status epilepticus — cerebral oedema, rhabdomyolysis, lactic acidosis, hyperthermia, aspiration.
  • Injuries — fractures, burns, drowning (advise against unsupervised swimming/heights).
  • Psychiatric — depression, anxiety, increased suicide risk (esp. with some AEDs).
  • Driving / occupational restrictions until a seizure-free interval is met.
  • Memory & cognitive decline in poorly controlled mesial temporal lobe epilepsy.

Key differentials

  • Syncope (convulsive syncope can mimic seizure — short, no post-ictal confusion).
  • Psychogenic non-epileptic seizures (PNES) — confirm with video-EEG.
  • Transient ischaemic attack (usually "negative" deficits vs "positive" seizure phenomena).
  • Migraine with aura (slower march, visual scintillations, headache).
  • Panic attack, hypoglycaemia, breath-holding spells (children), parasomnias, transient global amnesia, paroxysmal movement disorders.

Recently asked / exam angle

  • EEG image MCQs: identify 3 Hz spike-and-wave (absence) and hypsarrhythmia (West syndrome) — repeatedly asked with clinical vignettes.
  • Drug-of-choice grids: ethosuximide for absence; valproate for JME; avoid carbamazepine in absence/myoclonic — the carbamazepine-aggravation trap.
  • Status epilepticus sequence: "first-line drug" = lorazepam; "no IV access" = IM midazolam; second-line = fosphenytoin / valproate / levetiracetam (ESETT).
  • Teratogenicity: valproate as the most teratogenic AED; preferred agents in pregnancy; folic acid dose.
  • Pharmacology one-liners: ethosuximide = T-type Ca²⁺ channel; levetiracetam = SV2A; vigabatrin = GABA-transaminase inhibitor + visual field defect; lamotrigine + valproate combination → SJS risk.
  • Phenytoin pharmacokinetics: zero-order (saturation) kinetics, gum hypertrophy, fosphenytoin advantages, purple glove syndrome.
  • NCC as the commonest cause of acquired epilepsy / new-onset focal seizures in Indian adults (ring-enhancing lesion on contrast imaging).
  • HLA-B*1502 and carbamazepine-induced SJS/TEN screening in Asians.
  • Refractory epilepsy definition = failure of 2 appropriate AEDs → surgical referral.

Rapid revision

  1. Epilepsy = ≥2 unprovoked seizures >24 h apart, OR 1 seizure with ≥60% recurrence risk, OR a syndrome.
  2. 3 Hz spike-wave = absence; hypsarrhythmia = West; slow (<2.5 Hz) spike-wave = Lennox-Gastaut.
  3. DOC absence = ethosuximide; JME/myoclonic & generalised = valproate; focal = lamotrigine/levetiracetam.
  4. Carbamazepine, phenytoin, vigabatrin, gabapentin WORSEN absence & myoclonic seizures.
  5. Status epilepticus ≥5 min → lorazepam first, then fosphenytoin/valproate/levetiracetam (equal — ESETT), then anaesthetic infusion.
  6. No IV access in SE → IM midazolam (RAMPART).
  7. Valproate = most teratogenic AED (NTD, ↓IQ) — avoid in childbearing women; prefer lamotrigine/levetiracetam + folic acid 5 mg.
  8. MRI brain is the imaging investigation of choice for aetiology; EEG is best to classify; normal EEG does NOT exclude epilepsy.
  9. Ethosuximide → T-type Ca²⁺ channels; levetiracetam → SV2A; vigabatrin → GABA-transaminase (visual field loss).
  10. Mesial temporal sclerosis = commonest cause of refractory focal epilepsy; NCC = commonest acquired cause of epilepsy in India; stroke = commonest in the elderly.
  11. Vigabatrin is DOC for infantile spasms in tuberous sclerosis; otherwise ACTH/vigabatrin for West syndrome.
  12. Refractory epilepsy = failure of 2 appropriate AEDs → consider surgery, VNS, or ketogenic diet.