Myasthenic Crisis
Encounter flow
11/12 authoredCanonical 12-phase frame with authored status for this dossier.
Frame
Acute neuromuscular respiratory failure in known/suspected MG — exclude cholinergic crisis + GBS + botulism (AAN 2024)
crisis classified vs cholinergic vs mimic
Patient inputs (12)
Age + thymoma screening trigger; older onset (>50) more often AChR-positive (AAN 2024)
Known MG with subtype (AChR/MuSK/seronegative) gates IVIG vs PLEX choice (PLEX preferred MuSK) (AAN 2024)
Crisis triggers — infection, surgery, fluoroquinolones, aminoglycosides, magnesium, beta-blockers (AAN 2024)
Identify offending agents — fluoroquinolones, aminoglycosides, magnesium, beta-blockers, ICIs (AAN 2024)
Thymoma screen + aspiration / pneumonia trigger (AAN 2024)
Bulbar weakness predicts aspiration + airway compromise (AAN 2024)
20-30-40 rule — FVC <20 mL/kg or NIF magnitude <30 → intubation threshold (AAN 2024)
Tachypnoea → impending failure; paradoxical breathing is late (AAN 2024)
Late marker; can be preserved until rapid decompensation (AAN 2024)
IgA deficiency screen before IVIG (anaphylaxis risk) (AAN 2024)
AChR-Ab positive 80–85% of generalised MG; informs efgartigimod / rozanolixizumab eligibility (AAN 2024)
MuSK-Ab → favour PLEX (IVIG less effective); rituximab early (AAN 2024)
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Severity triggers (16)
- informationallife_threateningfull_crisisFull myasthenic crisis — FVC <15 mL/kg OR NIF magnitude <20 cmH2O OR severe bulbar weakness with aspiration OR hypercapnia → mechanical ventilation indicated (MGFA 2020 Update PMID 33144515)Trigger could not be auto-evaluated — needs clinician judgement.
- informationalsevereimpending_crisisImpending myasthenic crisis — FVC 15–20 mL/kg AND/OR NIF magnitude 20–30 cmH2O AND/OR bulbar progression with single-breath count <15; act BEFORE mechanical ventilation required (MGFA 2020 Update Int Consensus PMID 33144515)Trigger could not be auto-evaluated — needs clinician judgement.
- informationalseverecholinergic_crisisCholinergic crisis from pyridostigmine overdose — SLUDGE (Salivation, Lacrimation, Urination, Defecation, GI cramping, Emesis) + fasciculations + miosis + bradycardia mimicking myasthenic weakness (MGFA 2020)Trigger could not be auto-evaluated — needs clinician judgement.
- informationalsevereaspiration_riskSevere bulbar weakness with cough / swallow failure / drooling — leading cause of MG mortality (MGFA 2020)Trigger could not be auto-evaluated — needs clinician judgement.
- informationalseveremusk_phenotypeMuSK-Ab positive MG (~5–8% of generalised MG) — predominantly bulbar / respiratory / facial / neck flexor weakness; less response to AChE-I; rituximab first-line per international consensusTrigger could not be auto-evaluated — needs clinician judgement.
- informationalsevereachr_phenotypeAnti-AChR-Ab positive MG (~80% of generalised MG) — thymus pathology common (hyperplasia or thymoma); responsive to AChE-I, IVIG, PLEX, steroids, eculizumab, efgartigimod, rozanolixizumab, zilucoplanTrigger could not be auto-evaluated — needs clinician judgement.
- informationalsevereseronegative_phenotypeSeronegative MG (~5–10%) — standard AChR + MuSK Ab tests negative; clustered-AChR cell-based assay positive in subset; clinical features match generalised MGTrigger could not be auto-evaluated — needs clinician judgement.
- informationalsevererefractory_failed_two_therapiesRefractory MG — failed ≥2 prior therapies (typically prednisone + a steroid-sparing IS or PLEX/IVIG); persistent generalised disease despite maximal conventional therapyTrigger could not be auto-evaluated — needs clinician judgement.
- informationalseverepregnancy_phenotypeMG in pregnancy — disease course unpredictable (1/3 improve, 1/3 stable, 1/3 worsen); risk of transient neonatal MG from passive antibody transfer (~10–20% of infants)Trigger could not be auto-evaluated — needs clinician judgement.
- informationalsevereprecipitant_medication_triggerCrisis precipitant — recent exposure to an MG-aggravating medication: fluoroquinolones (ciprofloxacin, levofloxacin, moxifloxacin), aminoglycosides (gentamicin, tobramycin, amikacin), macrolides (azithromycin, erythromycin, telithromycin), magnesium IV (especially obstetric), β-blockers (esp. propranolol, timolol), immune-checkpoint inhibitors (nivolumab, pembrolizumab), procainamide, quinidine, D-penicillamine, succinylcholineTrigger could not be auto-evaluated — needs clinician judgement.
- informationalseverepregnancy_peripartum_magnesium_special_popMG in pregnancy / peripartum — disease course unpredictable (~1/3 worsen, peak first trimester + first postpartum month); IV magnesium sulfate for pre-eclampsia/eclampsia is CONTRAINDICATED (potent NMJ block → precipitates crisis); transient neonatal MG in ~10–20% of infants from transplacental maternal-antibody transfer (onset within 72 h, resolves 2–4 wk)Trigger could not be auto-evaluated — needs clinician judgement.
- informationalseverecrisis_differential_routing_and_cholinergic_pivotAcute neuromuscular respiratory failure differential — the load-bearing pivot is cholinergic-vs-myasthenic crisis (both cause weakness + respiratory failure in a known-MG patient on pyridostigmine); the discriminating cluster is muscarinic excess (SLUDGE + miosis + fasciculations + bradycardia → cholinergic) vs its absence (→ myasthenic). When the post-test probability of a non-MG mimic dominates, route by engine_id with carryover state (accumulated findings, current FVC/NIF, time-since-onset, therapy stack)Trigger could not be auto-evaluated — needs clinician judgement.
- informationalmoderateiga_deficiencyTotal IgA <7 mg/dL pre-IVIG — anaphylaxis risk with standard IVIG productsTrigger could not be auto-evaluated — needs clinician judgement.
- informationalmoderatethymoma_detectedAnterior mediastinal mass on CT/MRI chest — 10–15% of generalised MG; majority AChR-Ab positiveTrigger could not be auto-evaluated — needs clinician judgement.
- informationalmoderatelrp4_phenotypeAnti-LRP4-Ab positive MG (rare, ~1–3%) — typically milder generalised disease; often double-negative for AChR/MuSK on standard assays; cell-based clustered-AChR assay may upgradeTrigger could not be auto-evaluated — needs clinician judgement.
- informationalmoderateoffending_medication_exposureFluoroquinolone / aminoglycoside / macrolide / β-blocker / magnesium / ICI started (legacy id retained for backward compat — superseded by precipitant_medication_trigger above which has the complete avoid list)Trigger could not be auto-evaluated — needs clinician judgement.
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Recommended regimen
Myasthenic crisis immunomodulation + airway + chronic IS (AAN 2024)- ivigfirst linepooled_human_IgG2 g/kg total over 5 days (typically 0.4 g/kg/d × 5 d) • IV • daily × 5 d (max: 2 g/kg total)triggers: myasthenic_crisis, AChR_positive, IgA_replete2020 MGFA — IVIG and PLEX equivalent for AChR; check IgA before infusion (anaphylaxis risk in IgA deficient)rxcui 1426680
- plasmapheresisfirst lineapheresis5 sessions over 7–10 days (every other day) • IV/large-bore • 5 sessions q2 days (max: 5 sessions per course)triggers: MuSK_positive, rapid_response_needed, IgA_deficiencyPLEX favoured in MuSK + when faster onset needed (response within 24–72 h vs 5–10 d for IVIG) (AAN 2024)
outpatient playbook — drug actions (2)
- 1. maintenance IS (AAN 2024)Azathioprine 2–3 mg/kg/d OR MMF 1 g BID (AAN 2024) • PO • dailytrigger: Stable (AAN 2024)Maintenance (AAN 2024)
- 2. CGRP-class biologic if refractory (AAN 2024)Per agent (efgartigimod, rozanolixizumab, eculizumab, ravulizumab, zilucoplan, rituximab) (AAN 2024) • IV/SC • per agenttrigger: Refractory gMG (AAN 2024)Newer agents (AAN 2024)
Auto-drafted A&P note
outpatientSubjective
- Possible entry pathways: Bulbar weakness — dysphagia / dysarthria / dysphonia (AAN 2024); Respiratory failure / paradoxical breathing (AAN 2024); Rapidly worsening weakness in known MG (AAN 2024).
Objective
- No vitals, labs, or imaging entered for this encounter.
Assessment
**Myasthenic Crisis** (neuro.mg-crisis.core.v1). Phenotype framing: AChR vs MuSK vs seronegative; LEMS; congenital myasthenic syndromes; ICI-induced MG (AAN 2024) Scope: Acute neuromuscular respiratory failure in known/suspected MG — exclude cholinergic crisis + GBS + botulism (AAN 2024) No severity triggers fired against current inputs.
Plan
Regimen axis: **Myasthenic crisis immunomodulation + airway + chronic IS (AAN 2024)** — step "Step 1 — Immunomodulation (IVIG OR PLEX)". 1. ivig 2 g/kg total over 5 days (typically 0.4 g/kg/d × 5 d) IV daily × 5 d (pooled_human_IgG, first line) — 2020 MGFA — IVIG and PLEX equivalent for AChR; check IgA before infusion (anaphylaxis risk in IgA deficient) 2. plasmapheresis 5 sessions over 7–10 days (every other day) IV/large-bore 5 sessions q2 days (apheresis, first line) — PLEX favoured in MuSK + when faster onset needed (response within 24–72 h vs 5–10 d for IVIG) (AAN 2024) Setting playbook (outpatient) — Maintain remission, prevent crisis, manage chronic IS, monitor for relapse (AAN 2024) 3. maintenance IS (AAN 2024) Azathioprine 2–3 mg/kg/d OR MMF 1 g BID (AAN 2024) PO daily — Stable (AAN 2024) (Maintenance (AAN 2024)) 4. CGRP-class biologic if refractory (AAN 2024) Per agent (efgartigimod, rozanolixizumab, eculizumab, ravulizumab, zilucoplan, rituximab) (AAN 2024) IV/SC per agent — Refractory gMG (AAN 2024) (Newer agents (AAN 2024)) Non-pharmacologic actions: - Patient education on crisis warning signs (AAN 2024) - Medication-avoidance card (AAN 2024) - MyMG / MGFA emergency wallet card (AAN 2024) AVOID / contraindication checks: - Avoid_succinylcholine_in_MG_unpredictable_response (AAN 2024) - Avoid_aminoglycosides_fluoroquinolones_macrolides_in_MG (AAN 2024) - Avoid_magnesium_high_dose_in_MG (AAN 2024) - Avoid_non_selective_beta_blockers_can_worsen (AAN 2024) - Hold_pyridostigmine_during_crisis_intubation (AAN 2024) - Check_IgA_before_IVIG (AAN 2024) - Meningococcal_vaccine_before_complement_inhibitor (AAN 2024) - Steroids_can_transiently_worsen_in_first_week (AAN 2024)
Monitoring
Regimen monitoring: - FVC NIF q4-6h during crisis (AAN 2024) - IVIG AE watch TRALI aseptic meningitis thrombosis (AAN 2024) - PLEX central line complications (AAN 2024) - response curve to IVIG PLEX at days 5 10 (AAN 2024) - thymoma screen chest CT at diagnosis (AAN 2024) Setting (outpatient) monitoring: - MG-ADL / QMG q3–6 months (AAN 2024) - Drug-specific labs (CBC for AZA, BUN/Cr for MMF, IgG for FcRn, complement panel for eculizumab) (AAN 2024) Follow-up plan: Long-term immunosuppression (steroid + steroid-sparing — azathioprine, MMF, rituximab; new agents efgartigimod/rozanolixizumab/zilucoplan); thymectomy if thymoma or AChR + age <65 (AAN 2024) - Close-out criterion: outpatient neuromuscular plan + chronic IS regimen set Monitoring phase: Serial FVC/MIP q4–6h, ABG, swallow assessment daily, response curve to IVIG/PLEX (AAN 2024)
Disposition
Current setting: outpatient — Maintain remission, prevent crisis, manage chronic IS, monitor for relapse (AAN 2024) Disposition criteria: - Continue maintenance with q3-month neuromuscular review (AAN 2024) Escalation triggers (move to higher acuity): - Bulbar weakness, dyspnea → ED for crisis assessment (AAN 2024) - Infection → urgent care, avoid offending antibiotics (AAN 2024)
Patient Action Plan
**Myasthenia gravis emergency action plan (AAN 2024)** Personalised values: baseline_function, maintenance_immunosuppressant (AAN 2024), pyridostigmine_schedule (AAN 2024), triggers_to_avoid. **At baseline (AAN 2024)** (green): Triggers: - Stable strength (AAN 2024) - Normal swallow + speech - Adequate breathing Actions: - Take pyridostigmine and immunosuppressant as prescribed - Avoid known offending meds (fluoroquinolones, aminoglycosides, macrolides, β-blockers, magnesium) - Carry MGFA emergency wallet card - Keep follow-up appointments **Caution — worsening symptoms (AAN 2024)** (yellow): Triggers: - Increased fatigability - New mild dysphagia or slurred speech - Mild dyspnea on exertion - Recent infection / fever - Started new medication Actions: - Contact neurology team within 24 h - Review medication list for offenders - Treat infection promptly with safe antibiotics - Monitor breathing carefully - Do NOT increase pyridostigmine without team guidance (cholinergic crisis risk) Contact provider when: - Any change persisting >24 h - New medication exposure - Fever or infection **Medical alert — myasthenic crisis warning signs (AAN 2024)** (red): Triggers: - Severe difficulty breathing or shortness of breath at rest - Cannot swallow saliva / drooling / aspiration - Cannot speak above a whisper - Cannot lift head off pillow - Sudden generalised severe weakness - Weak cough / cannot clear secretions Actions: - Call 911 / go to ED immediately - Bring MGFA wallet card and medication list - Tell ED team: "myasthenic crisis suspected — avoid succinylcholine, magnesium, aminoglycosides" - Sit upright; do not lie flat Contact provider when: - Any red zone symptom — ED now
Earlier-Return Triggers
Return-precaution thresholds (watch for): - [LIFE_THREATENING] Full myasthenic crisis — FVC <15 mL/kg OR NIF magnitude <20 cmH2O OR severe bulbar weakness with aspiration OR hypercapnia → mechanical ventilation indicated (MGFA 2020 Update PMID 33144515) - [SEVERE] Impending myasthenic crisis — FVC 15–20 mL/kg AND/OR NIF magnitude 20–30 cmH2O AND/OR bulbar progression with single-breath count <15; act BEFORE mechanical ventilation required (MGFA 2020 Update Int Consensus PMID 33144515) - [SEVERE] Cholinergic crisis from pyridostigmine overdose — SLUDGE (Salivation, Lacrimation, Urination, Defecation, GI cramping, Emesis) + fasciculations + miosis + bradycardia mimicking myasthenic weakness (MGFA 2020)
Citations
- International Consensus Guidance for Management of Myasthenia Gravis: 2020 Update (Narayanaswami, Sanders, Wolfe et al, Neurology 2020;96(3):114-122, PMID 33144515) — primary guideline floor; trial-grade anchors MGTX (Wolfe NEJM 2016), REGAIN (eculizumab), ADAPT (efgartigimod), MycarinG (rozanolixizumab), RAISE (zilucoplan), Díaz-Manera 2012 (MuSK rituximab), Barth 2011 (IVIg vs PLEX), Vivacity-MG3 (nipocalimab 2025) [PMID:33144515](https://pubmed.ncbi.nlm.nih.gov/33144515/) - Cited evidence (PMID 27509100) [PMID:27509100](https://pubmed.ncbi.nlm.nih.gov/27509100/) - Cited evidence (PMID 29066163) [PMID:29066163](https://pubmed.ncbi.nlm.nih.gov/29066163/) - Cited evidence (PMID 34146511) [PMID:34146511](https://pubmed.ncbi.nlm.nih.gov/34146511/) - Cited evidence (PMID 37059507) [PMID:37059507](https://pubmed.ncbi.nlm.nih.gov/37059507/) Last reconciled with current guidelines: 2026-05-18.
- International Consensus Guidance for Management of Myasthenia Gravis: 2020 Update (Narayanaswami, Sanders, Wolfe et al, Neurology 2020;96(3):114-122, PMID 33144515) — primary guideline floor; trial-grade anchors MGTX (Wolfe NEJM 2016), REGAIN (eculizumab), ADAPT (efgartigimod), MycarinG (rozanolixizumab), RAISE (zilucoplan), Díaz-Manera 2012 (MuSK rituximab), Barth 2011 (IVIg vs PLEX), Vivacity-MG3 (nipocalimab 2025) — PMID:33144515
- Cited evidence (PMID 27509100) — PMID:27509100
- Cited evidence (PMID 29066163) — PMID:29066163
- Cited evidence (PMID 34146511) — PMID:34146511
- Cited evidence (PMID 37059507) — PMID:37059507