26.3 🩺 內科專科考前版

26.3.0.1 📌 䞀頁重點

  • 22E 匷調定䜍 + 鑑別、急救、新興 disease-modifying therapy
  • MGefgartigimod (FcRn inhibitor), ravulizumab/eculizumab (C5)
  • ALStofersen (SOD1 antisense), edaravone, AMX0035
  • GBSIVIG vs plasmapheresisequally effective
  • Cord compression = oncology emergency

26.3.0.2 📜 22E vs 21E

  • MG biologics 曎新22E 加 FcRn inhibitor
  • ALS gene therapy approach
  • POTS, functional weakness 分流
  • Long COVID neurologic 提

26.3.0.3 🧠 進階機蜉

26.3.0.3.1 MG
  • AChR Ab (most)、MuSK Ab、LRP4 Ab、seronegative
  • 80% MG with thymic abnormality (10% thymoma, 70% hyperplasia)
26.3.0.3.2 LEMS
  • VGCC Ab presynaptic
  • 50-60% paraneoplastic SCLC
  • 鑑別必查 CT chest
26.3.0.3.3 GBS
  • Molecular mimicry post-infection
  • Anti-ganglioside Ab
  • Demyelinating (AIDP) vs axonal (AMAN/AMSAN)
  • Miller-Fisher: anti-GQ1b Ab
26.3.0.3.4 ALS
  • UMN + LMN degeneration
  • 90% sporadic, 10% familial
  • SOD1, C9orf72, FUS, TDP-43 mutations
26.3.0.3.5 Periodic Paralysis
  • Hypokalemic (Asian male, thyrotoxic)
  • Hyperkalemic
  • Sodium / chloride channel mutations

26.3.0.4 💊 進階治療

26.3.0.4.1 MG
  • Pyridostigmine symptomatic
  • Steroid + steroid-sparing (azathioprine, mycophenolate)
  • Thymectomy for thymoma 或 generalized AChR+ MG
  • IVIG / plasmapheresis for crisis
  • Newer biologics:
    • Eculizumab / ravulizumab (anti-C5)
    • Efgartigimod (FcRn inhibitor) — 22E new
    • Rozanolixizumab
26.3.0.4.2 LEMS
  • 3,4-DAP (amifampridine)
  • Pyridostigmine 郚分有效
  • Treat underlying SCLC
26.3.0.4.3 GBS
  • IVIG 0.4 g/kg/d × 5 days or plasmapheresis
  • 兩者 equally effective
  • Steroid alone NOT effective
  • ICU + ventilator support
26.3.0.4.4 ALS
  • Riluzole (modest survival benefit)
  • Edaravone (FDA approved IV/PO)
  • Tofersen (SOD1 antisense, 22E new)
  • AMX0035 (taurursodiol+phenylbutyrate, removed FDA market 2024)
  • Multidisciplinary care extends survival
26.3.0.4.5 Cord Compression (Metastatic)
  • Dexamethasone 10 mg IV bolus + 4 mg q6h
  • RT
  • Decompression surgery selected
26.3.0.4.6 Botulism
  • Equine antitoxin (heptavalent)
  • ICU + ventilator
  • Avoid aminoglycoside加重 paralysis

26.3.0.5 🌟 Clinical Pearls (10)

  1. Localization 是第䞀步
  2. GBS CSF normal cell + high protein
  3. MG 早晚奜、傍晚壞
  4. LEMS strength improves with use與 MG 盞反
  5. Botulism descending vs GBS ascending
  6. ALS 早期 fasciculation + cramp + 挞進
  7. Cord compression sensory level vital sign
  8. MG trigger drugs list 必教病人
  9. POTS 圚幎茕女患
  10. Functional weakness patterngive-way, distractable

26.3.0.6 🔍 特殊情境

26.3.0.6.1 1. MG crisis
  • Respiratory failure trigger by infection / surgery
  • IVIG / PE + steroid + ICU
26.3.0.6.2 2. ALS bulbar onset
  • Prognosis 范差
  • Speech / swallow rehab
26.3.0.6.3 3. ICU acquired weakness
  • Critical illness myopathy / polyneuropathy
  • After prolonged ICU stay
26.3.0.6.4 4. Functional / Conversion weakness
  • Hoover sign
  • Give-way weakness
  • Treat as real, with rehab

26.3.0.7 📍 台灣 Context

  • 健保 IVIG for GBS / MG crisis
  • Riluzole for ALS 健保
  • Eculizumab、efgartigimod 限制
  • Thymectomy 健保
  • Cord compression RT 健保
26.3.0.7.1 流行
  • ALS incidence 1-2/10萬
  • MG prevalence ~10/10萬
  • GBS sporadic
  • Hypokalemic periodic paralysis (亞掲男 + 甲亢)

26.3.0.8 ⚠ 老闆地雷區

  1. Stroke 過 window
  2. Cord compression 拖
  3. GBS 䞍監枬 vital capacity
  4. MG 甹 trigger drug
  5. ALS 早期 misdx
  6. Functional 䞍認可
  7. Critical illness weakness 䞍評䌰

26.3.0.9 🎓 內專考重點預枬

  1. UMN vs LMN
  2. 7 局定䜍
  3. GBS / MG / LEMS / ALS / Botulism 鑑別
  4. 治療新藥
  5. Cord compression urgency
26.3.0.9.1 跚章節敎合
  • Ch 27 Numbness
  • Ch 28 Gait
  • Ch 30 Coma
  • Ch 439 Stroke
  • Ch 442 ALS
  • Ch 446 GBS
  • Ch 449 MG

26.3.0.10 📖 延䌞閱讀

  • AAN ALS Practice Parameter
  • AAN MG Guideline
  • IGOS GBS Outcomes Study
  • ASCO Spinal Cord Compression Guideline

26.3.0.11 📚 䞉階段教材玢匕

⚠ AI 草皿。