372.4 📋 章末速蚘 Summary

372.4.1 🔑 䞀句話瞜結

Seizure = transient abnormal excessive/synchronous neuronal activity; epilepsy ILAE 2014 = ≥ 2 unprovoked seizures OR 1 + high recurrence risk OR syndrome diagnosis; ILAE 2017 classification — (1) onset focal vs generalized vs unknown, (2) awareness aware vs impaired (focal only), (3) motor (clonic/tonic/atonic/myoclonic/automatisms) vs non-motor (sensory/emotional/autonomic/behavior arrest) features; common types — GTC (LOC + tonic 10-30 sec → clonic 30-90 sec → post-ictal + lateral tongue bite specific), focal aware (aura preserved awareness), focal impaired awareness (automatisms lip smacking/picking + post-ictal confusion, temporal/frontal), absence (childhood, brief, no post-ictal, 3 Hz spike-wave EEG), myoclonic (JME adolescents morning + photosensitive), atonic (drop attacks LGS), tonic/clonic; etiology by age — children (idiopathic/genetic, birth injury, febrile, congenital); young adults (idiopathic, trauma, drug/alcohol withdrawal, tumor); older adults (stroke most common new-onset > 60 + tumor + trauma + dementia + metabolic); provoked (acute symptomatic — NOT epilepsy) — hypoglycemia, hyponatremia, hypocalcemia, alcohol withdrawal, drug toxicity/withdrawal, fever, stroke, head injury, CNS infection; unprovoked recurrence ~ 35-40% after 1st, > 70% after 2nd; workup — witnessed account critical + glucose + electrolytes + toxicology + pregnancy + ECG (rule out long QT/cardiogenic) + EEG within 24 hr (sleep + sleep-deprived ↑ yield) + MRI brain preferred + LP if meningitis + genetic testing select; distinguish from mimics — syncope (convulsive syncope brief jerks), PNES (psychogenic non-epileptic seizures — eyes closed, asynchronous, pelvic thrusting, side-to-side head, memory during, normal EEG, video-EEG gold standard), TIA, migraine, movement disorders; ASMs (antiseizure medications) by type — focal: levetiracetam (first-line minimal interactions) + lamotrigine (slow titration SJS) + lacosamide (IV/PR interval) + brivaracetam + cenobamate (2019 highly effective refractory + DRESS risk slow titration) + carbamazepine + perampanel + clobazam + eslicarbazepine; generalized: valproate (most effective + teratogenic avoid pregnancy) + lamotrigine + levetiracetam + topiramate + zonisamide; absence: ethosuximide first-line (T-type Ca) + valproate; myoclonic JME: valproate + levetiracetam + topiramate — AVOID carbamazepine/oxcarbazepine/phenytoin/gabapentin worsen; adverse effects critical — lamotrigine SJS/TEN (slow titration!), **carbamazepine SJS HLA-B*1502 screening Asians + SIADH + AV block, valproate hepatotoxic/pancreatitis/teratogenic NTD, phenytoin gum hyperplasia/hirsutism/ataxia/SJS, topiramate kidney stones/glaucoma/oligohidrosis/cognitive, levetiracetam behavioral (irritability, depression), lacosamide PR prolongation, vigabatrin visual field constriction, perampanel psychiatric BBW, cenobamate DRESS; pregnancy — lamotrigine + levetiracetam safest, avoid valproate (10% malformations + cognitive deficits), avoid topiramate (orofacial clefts) + folate 0.4-5 mg/d + continue ASMs; refractory (30% of epilepsy, 2 ASM failure) — combination + epilepsy surgery (mesial temporal sclerosis 70%+ seizure free) + VNS + RNS (Responsive Neurostimulation closed-loop) + DBS anterior thalamus + ketogenic diet + cannabidiol (Epidiolex FDA Dravet/LGS/TSC) + cenobamate + fenfluramine (Fintepla Dravet/LGS) + everolimus (TSC) + vigabatrin (TSC infantile spasms); SUDEP ~ 1.2/1000 patient-years (refractory 10x higher) — counsel + GTC seizures + nocturnal + prone; autoimmune epilepsy — anti-NMDA-R/anti-LGI1 (faciobrachial dystonic seizures pathognomonic!**) /anti-CASPR2/anti-GAD65/anti-Ma2 → immunotherapy + ASMs。

372.4.2 💊 治療粟芁

  • Focal-onset ASMslevetiracetam (Keppra) 500-3000 mg/d in 2 divided doses (renal adjust) first-line minimal interactions; lamotrigine (Lamictal) slow titration to 200-400 mg/d (SJS risk!); lacosamide (Vimpat) 200-400 mg/d (IV available, PR caution); brivaracetam 50-200 mg/d; cenobamate (Xcopri) titrate to 200-400 mg/d (DRESS, very slow titration!); older carbamazepine 600-1200 mg/d + oxcarbazepine 600-2400 mg/d + phenytoin
  • Generalized ASMsvalproate 500-3000 mg/d (most effective + teratogenic — avoid pregnancy + monitor LFTs + amylase + platelets) + lamotrigine + levetiracetam + topiramate (kidney stones, glaucoma, cognitive, oligohidrosis); zonisamide
  • Absenceethosuximide 250-1500 mg/d first-line (T-type Ca); valproate; lamotrigine
  • Myoclonic JMEvalproate first-line + levetiracetam + topiramate — AVOID carbamazepine, oxcarbazepine, phenytoin, gabapentin (worsen myoclonic)
  • Lennox-Gastautvalproate + lamotrigine + clobazam + rufinamide + cannabidiol (Epidiolex) + fenfluramine (Fintepla) + VNS + corpus callosotomy for drop attacks
  • Dravet syndrome (SCN1A)valproate + clobazam + stiripentol + cannabidiol + fenfluramine — AVOID sodium channel blockers (worsen)
  • Tuberous sclerosis epilepsyvigabatrin first-line for TSC infantile spasms + everolimus mTOR inhibitor for refractory SEGA + epilepsy
  • Pregnancylamotrigine + levetiracetam safest — avoid valproate (NTD 10%, cognitive) + topiramate (orofacial clefts); folate 0.4-5 mg/d preconception + throughout
  • Refractory (2 ASM failure)combination ASMs + epilepsy surgery (mesial temporal lobectomy for MTS — 70%+ seizure free) + VNS (vagus nerve stimulator) + RNS (Responsive Neurostimulation, NeuroPace) + DBS anterior thalamic nucleus + LITT (Laser Interstitial Thermal Therapy) + ketogenic diet (children) + cannabidiol + cenobamate
  • Autoimmune epilepsyIVIG 2 g/kg or methylprednisolone pulse + rituximab + cyclophosphamide + ASMs; anti-LGI1 → immunotherapy quickly to prevent encephalitis
  • **HLA-B*1502 screening before CBZ in Asians**Han Chinese, Thai, Malaysian, Indian — high risk SJS/TEN

372.4.3 🎯 盧醫垫的考前提醒

  1. Epilepsy ILAE 2014 definition: ≥ 2 unprovoked seizures > 24 hr apart, OR 1 unprovoked + ≥ 60% recurrence risk over 10 years, OR epilepsy syndrome; provoked seizures (acute symptomatic from hypoglycemia/hyponatremia/alcohol withdrawal/drug toxicity/fever/stroke) are NOT epilepsy
  2. ILAE 2017 classification framework: onset (focal vs generalized) + awareness (aware vs impaired, focal only) + motor vs non-motor features
  3. ASM choice by seizure type: focal — levetiracetam/lamotrigine/lacosamide/cenobamate first-line; generalized — valproate (most) + lamotrigine + levetiracetam + topiramate; absence — ethosuximide first-line + valproate; myoclonic JME — valproate + levetiracetam (avoid CBZ/OXC/PHT/GBP!)
  4. Adverse effects to memorize: **lamotrigine SJS (slow titration!) + carbamazepine SJS (HLA-B*1502 Asian screening) + SIADH + AV block + valproate hepatotoxic/pancreatitis/teratogenic (NTD 10%) + phenytoin gum hyperplasia + topiramate kidney stones + glaucoma + cognitive + levetiracetam behavioral + cenobamate DRESS (slow titration)**
  5. Pregnancy ASM choice: lamotrigine + levetiracetam safest; AVOID valproate (10% major malformations + cognitive deficits in offspring) + topiramate (orofacial clefts + low birth weight); folate 0.4-5 mg/d preconception + throughout; continue ASMs (untreated seizures dangerous)
  6. Distinguish epileptic seizure vs syncope vs PNES: epileptic (aura + tonic-clonic + lateral tongue bite specific + post-ictal confusion minutes); syncope (prodrome + brief LOC + quick recovery + brief myoclonic OK); PNES (eyes closed + asynchronous + pelvic thrusting + side-to-side head + memory during + normal EEG during event — video-EEG gold standard)
  7. Stroke is most common cause of new-onset seizure in patients > 60; in elderly — also consider metabolic, drug toxicity, dementia, tumor
  8. Refractory epilepsy (30% of patients, 2 ASM failure): epilepsy surgery (mesial temporal sclerosis 70%+ seizure free is best outcome) + VNS + RNS (Responsive Neurostimulation) + DBS anterior thalamus + LITT + ketogenic diet + cannabidiol (Epidiolex) + cenobamate (Xcopri 2019) + fenfluramine (Fintepla)
  9. Autoimmune epilepsy (consider in subacute-onset adult new seizures): anti-NMDA-R (psychiatric + seizures + movement) + anti-LGI1 (faciobrachial dystonic seizures PATHOGNOMONIC + amnesia) + anti-CASPR2 + anti-GAD65 → immunotherapy IVIG/methylprednisolone/rituximab + ASMs — early treatment critical
  10. SUDEP (Sudden Unexplained Death in Epilepsy) ~ 1.2/1000 patient-years (refractory 10x higher): counsel patients especially with GTC seizures + refractory + nocturnal + prone position; seizure freedom is protective — maximize ASM optimization or consider surgery