Last Minute Review
Epilepsy — Last Minute Review
Rapid Review
A last-minute review of high-yield epilepsy facts for the RITE and board exams. Tables, key associations, and must-know one-liners — designed for a quick pass the night before.
Seizure Classification (ILAE 2017 — Board Standard)
Note: ILAE 2017 remains the most familiar board framework, but the ILAE 2025 seizure classification is a published ILAE position paper. Know both: 2017 focal aware/impaired awareness and 2025 consciousness/observable-manifestation terminology.
| Old Term | 2017 Term | 2025 Term |
|---|---|---|
| Simple partial seizure | Focal aware seizure | Focal preserved consciousness seizure (FPC) |
| Complex partial seizure | Focal impaired awareness seizure | Focal impaired consciousness seizure (FIC) |
| Secondarily generalized tonic-clonic | Focal to bilateral tonic-clonic | Focal-to-bilateral tonic-clonic (FBTC) |
| Grand mal | Generalized-onset tonic-clonic | Generalized tonic-clonic (GTC) |
| Petit mal | Absence (non-motor) | Absence seizure (“non-motor” removed) |
| Aura | Focal aware seizure | Focal preserved consciousness seizure |
💎 Board Pearl
- 2025 key changes: “awareness” → consciousness (= awareness + responsiveness); “motor/non-motor” → observable/non-observable; “onset” dropped from class names; 63 → 21 seizure types
- New seizure type: generalized negative myoclonus (brief <500 ms interruption of tone)
- Epileptic spasms = seizure type only in generalized; in focal/unknown = descriptor
- Consciousness is a classifier for focal and unknown seizures only — most generalized seizures impair consciousness; myoclonic seizures are a notable exception (consciousness usually preserved)
Epilepsy Syndromes by Age of Onset
Neonatal (<2 months)
| Syndrome | Seizure Type | EEG Pattern | Key Gene/Etiology | Prognosis |
|---|---|---|---|---|
| Ohtahara (EIEE) | Tonic spasms | Burst suppression (wake + sleep) | STXBP1, KCNQ2, structural | Severe; may evolve to West → LGS |
| KCNQ2 neonatal epilepsy | Tonic, clonic | Burst suppression or multifocal | KCNQ2 | Good if self-limited; poor if DEE variant |
| Benign familial neonatal epilepsy | Clonic, apneic | May be normal interictally | KCNQ2, KCNQ3 | Excellent; remits by 6 months |
| Pyridoxine-dependent epilepsy | Multifocal clonic, myoclonic | Burst suppression or multifocal | ALDH7A1 | Seizure-free on B6; cognitive variable |
| Early myoclonic encephalopathy | Erratic myoclonus | Burst suppression (more in sleep) | Metabolic (NKH, organic acidurias) | Severe |
Infantile (2–12 months)
| Syndrome | Seizure Type | EEG Pattern | Key Gene/Etiology | Prognosis |
|---|---|---|---|---|
| Infantile epileptic spasms (West) | Epileptic spasms (clusters) | Hypsarrhythmia | TSC, structural, genetic (ARX, CDKL5) | Variable; 60–70% have cognitive impairment |
| Dravet syndrome | Prolonged febrile hemiclonic → myoclonic, absence, focal | Normal early; generalized spike-wave later | SCN1A (80%) | Poor; drug-resistant, cognitive decline |
| SCN2A encephalopathy | Tonic, clonic | Multifocal or burst suppression | SCN2A | Variable; early-onset → Na-blockers may help |
| CDKL5 encephalopathy | Epileptic spasms, tonic, hypermotor | Multifocal or hypsarrhythmia | CDKL5 | Severe; Rett-like features |
Childhood (1–12 years)
| Syndrome | Seizure Type | EEG Pattern | Key Feature | Prognosis |
|---|---|---|---|---|
| CAE | Typical absence (10–30 s, pluridaily) | 3 Hz generalized spike-wave | Peak 4–8 yr; hyperventilation provokes absences in >80–90% of untreated CAE | Good; 65–70% remit by adolescence |
| Doose (MAE) | Myoclonic-atonic | 2–3 Hz spike/polyspike-wave | Drop attacks; may have absence, GTCC | Variable; 50–60% seizure-free |
| SeLECTS (BECTS) | Focal hemifacial motor ± somatosensory; sleep predominant | Centrotemporal spikes (horizontal/tangential dipole: negative centrotemporal, positive frontal) | Most common childhood epilepsy; age 3–13 yr | Excellent; virtually all remit by age 16 (>95%) |
| Panayiotopoulos | Autonomic (nausea, vomiting, pallor), eye deviation | Occipital ± multifocal spikes | Prolonged seizures; mimics gastroenteritis or encephalitis | Excellent; remits 1–2 yr after onset |
| LGS | Tonic (sleep), atonic, atypical absence, myoclonic, GTCC | Slow (<2.5 Hz) spike-wave + generalized paroxysmal fast activity (GPFA) in sleep | Onset 1–7 yr; multiple seizure types | Poor; drug-resistant, cognitive decline |
| CSWS / ESES | Variable; may be subtle | Continuous spike-wave in >85% of NREM sleep | Cognitive/behavioral regression | EEG normalizes by puberty; cognitive outcome variable |
| Landau-Kleffner | Focal, absence-like | ESES pattern over temporal/perisylvian | Acquired aphasia (auditory agnosia) | Language recovery variable; seizures remit |
Adolescent / Adult
| Syndrome | Seizure Type | EEG Pattern | Key Feature | Prognosis |
|---|---|---|---|---|
| JME | Myoclonic jerks (morning) + GTCC + absence (30%) | 4–6 Hz polyspike-wave | Onset 12–18 yr; photosensitive; lifelong Rx | Well-controlled but rarely remits (<10%); lifelong ASM |
| JAE | Absence (less frequent than CAE) + GTCC (80%) | 3–4 Hz spike-wave (faster fragments) | Onset 10–17 yr; GTCC common | Good control; lower remission rate than CAE |
| GTCA alone (epilepsy with GTCC only) | GTCC only | Generalized spike-wave / polyspike-wave | Onset 10–25 yr; often on awakening | Good control on ASM |
| TLE (mesial) | Focal impaired awareness — epigastric aura, déjà vu, oral/manual automatisms | Temporal intermittent rhythmic delta (TIRDA), anterior temporal sharp waves | Most common focal epilepsy in adults; hippocampal sclerosis | 30% drug-resistant; surgery 60–80% seizure-free |
| FLE | Brief, nocturnal, hypermotor, bilateral motor; rapid secondary generalization | May be normal interictally; vertex/frontal spikes | Clusters from sleep; bizarre semiology → misdiagnosed as PNES | Variable; surgery less successful than TLE |
💎 Board Pearl
- Ohtahara → West → LGS = classic electroclinical evolution of severe neonatal-onset epilepsy
- CAE: 3 Hz spike-wave provoked by hyperventilation; JME: 4–6 Hz polyspike-wave provoked by sleep deprivation/photic stimulation
- SeLECTS = most common childhood epilepsy; virtually all remit by age 16 (>95%)
- GPFA in sleep = pathognomonic for LGS
EEG Patterns & Epilepsy Associations
| EEG Pattern | Association |
|---|---|
| 3 Hz generalized spike-wave | Childhood absence epilepsy |
| 3–4 Hz spike-wave (slightly faster fragments) | Juvenile absence epilepsy |
| 4–6 Hz generalized polyspike-wave | Juvenile myoclonic epilepsy |
| Hypsarrhythmia | IESS (infantile epileptic spasms syndrome, ILAE 2022) — historically “West syndrome” |
| Burst suppression (neonatal) | Ohtahara / early myoclonic encephalopathy |
| Centrotemporal spikes (horizontal/tangential dipole: negative centrotemporal, positive frontal) | SeLECTS (BECTS) |
| Anterior temporal sharp waves / TIRDA | Mesial temporal lobe epilepsy |
| Slow (<2.5 Hz) spike-wave | Lennox-Gastaut syndrome |
| Generalized paroxysmal fast activity (GPFA) in NREM | LGS (pathognomonic) |
| Continuous spike-wave during NREM (spike-wave index ≥50% (clinical threshold) to ≥85% (Tassinari original)) | CSWS / ESES (now grouped under DEE-SWAS / EE-SWAS in ILAE 2022 framework) |
| Occipital spikes (shifting) | Panayiotopoulos syndrome |
| Photoparoxysmal response (PPR) | JME, progressive myoclonic epilepsies |
| 2–3 Hz polyspike-wave | Doose (myoclonic-atonic epilepsy) |
| Vertex positive sharp waves (neonatal) | Benign neonatal sleep myoclonus (not epilepsy) |
| Stimulus-induced rhythmic, periodic, or ictal discharges (SIRPIDs) | ICU artifact — significance debated |
| Lateralized periodic discharges (LPDs) | Acute structural lesion (stroke, HSV encephalitis); seizure risk 50–60% |
| Generalized periodic discharges (GPDs) | Metabolic/toxic encephalopathy, CJD, post-anoxic |
💎 Board Pearl
- Hypsarrhythmia = chaotic, high-amplitude, asynchronous slow waves + multifocal spikes — disappears during spasm (electrodecremental)
- TIRDA (temporal intermittent rhythmic delta activity) has the same localizing value as temporal sharp waves for TLE
- GPFA is seen in LGS only; slow spike-wave alone is insufficient for diagnosis
ASM Selection by Syndrome
| Syndrome / Seizure Type | First-Line ASM | Alternatives | Avoid |
|---|---|---|---|
| Focal seizures | LEV, LTG, OXC, CBZ | BRV, ZNS, LCM, cenobamate | — |
| Focal to bilateral tonic-clonic | LEV, LTG, OXC, CBZ | LCM, cenobamate, VPA | — |
| Generalized tonic-clonic (IGE) | VPA, LEV, LTG | TPM, PER, CLB | CBZ, OXC, PHT, GBP, TGB (worsen myoclonus/absence) |
| Typical absence | ETX, VPA, LTG | CLB | CBZ, OXC, PHT, GBP, TGB, VGB |
| JME | VPA most effective overall; in women of childbearing potential, LEV is preferred first-line (avoid VPA if clinically feasible — not routine first-line in PWECP); LTG also reasonable but may worsen myoclonus in a subset (~5–10%) | TPM, CLB, PER | CBZ, OXC, PHT, GBP (worsen myoclonus) |
| Infantile spasms | Hormonal therapy (ACTH or high-dose oral prednisolone) first-line for non-TSC; vigabatrin first-line for TSC | ICISS: hormonal + vigabatrin improved early spasm cessation vs hormonal alone; combination considered, especially in high-risk or local-protocol pathways | CBZ, OXC |
| Dravet syndrome | First-line: VPA ± CLB. Add-on FDA-approved: stiripentol, fenfluramine, cannabidiol (Epidiolex) | CBD (Epidiolex), fenfluramine, stiripentol | All Na-channel blockers (CBZ, OXC, LTG, PHT, LCM) — worsen seizures |
| LGS | VPA, LTG, CLB, rufinamide | CBD, felbamate, TPM | CBZ, OXC (may worsen atonic/tonic) |
| CSWS/ESES | High-dose BZDs (clobazam, diazepam), VPA, ETX | Corticosteroids, IVIG, surgery if focal | — |
| TLE (drug-resistant) | Consider surgery early (ERSET: superior to 2 more ASM trials) | Cenobamate, LCM | Delay of surgical referral |
💎 Board Pearl
Drugs That Worsen Specific Syndromes
- Na-channel blockers (CBZ, OXC, LTG, PHT, LCM) → worsen Dravet (SCN1A loss-of-function)
- CBZ, OXC, PHT, GBP, TGB → worsen absence, myoclonus in JME and other IGEs
- LTG may worsen myoclonus in a subset of JME patients (idiosyncratic, not strictly dose-dependent); monitor and switch if myoclonus worsens
- Vigabatrin → irreversible visual field constriction (peripheral); requires visual field monitoring every 3 months
- VPA in women of childbearing age → highest teratogenicity (MCM 6–10%; NTDs 1–2%; IQ ↓ 8–11 points); avoid if possible
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