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neuro.mg-crisis.core.v1PRODUCTION
neuro.mg-crisis.core.v1

Myasthenic Crisis

neurologyacuteadult
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11/12 authored

Canonical 12-phase frame with authored status for this dossier.

Current phase

Frame

Detailed

Acute neuromuscular respiratory failure in known/suspected MG — exclude cholinergic crisis + GBS + botulism (AAN 2024)

Inputs
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Actions
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Advance rule
Set
Advance when

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)

* = hard-required. Engine cannot meaningfully run until these are filled.

Severity triggers (16)

16 need judgement
  • informationallife_threateningfull_crisis
    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)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereimpending_crisis
    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)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverecholinergic_crisis
    Cholinergic 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_risk
    Severe bulbar weakness with cough / swallow failure / drooling — leading cause of MG mortality (MGFA 2020)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseveremusk_phenotype
    MuSK-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 consensus
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereachr_phenotype
    Anti-AChR-Ab positive MG (~80% of generalised MG) — thymus pathology common (hyperplasia or thymoma); responsive to AChE-I, IVIG, PLEX, steroids, eculizumab, efgartigimod, rozanolixizumab, zilucoplan
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereseronegative_phenotype
    Seronegative MG (~5–10%) — standard AChR + MuSK Ab tests negative; clustered-AChR cell-based assay positive in subset; clinical features match generalised MG
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevererefractory_failed_two_therapies
    Refractory MG — failed ≥2 prior therapies (typically prednisone + a steroid-sparing IS or PLEX/IVIG); persistent generalised disease despite maximal conventional therapy
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverepregnancy_phenotype
    MG 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_trigger
    Crisis 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, succinylcholine
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverepregnancy_peripartum_magnesium_special_pop
    MG 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_pivot
    Acute 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_deficiency
    Total IgA <7 mg/dL pre-IVIG — anaphylaxis risk with standard IVIG products
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderatethymoma_detected
    Anterior mediastinal mass on CT/MRI chest — 10–15% of generalised MG; majority AChR-Ab positive
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderatelrp4_phenotype
    Anti-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 upgrade
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderateoffending_medication_exposure
    Fluoroquinolone / 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.

Workflow calculators

This dossier does not reference any calculators.

Recommended regimen

Myasthenic crisis immunomodulation + airway + chronic IS (AAN 2024)
axis: mg_crisis_immunomodulationstep 1 - Step 1 — Immunomodulation (IVIG OR PLEX)
Selected step "Step 1 — Immunomodulation (IVIG OR PLEX)" — Myasthenic crisis (FVC <20 mL/kg, NIF magnitude <30, severe bulbar weakness)
  • ivig
    first line
    pooled_human_IgG
    2 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_replete
    2020 MGFA — IVIG and PLEX equivalent for AChR; check IgA before infusion (anaphylaxis risk in IgA deficient)
    rxcui 1426680
  • plasmapheresis
    first line
    apheresis
    5 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_deficiency
    PLEX 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. 1. maintenance IS (AAN 2024)
    Azathioprine 2–3 mg/kg/d OR MMF 1 g BID (AAN 2024) • PO • daily
    trigger: Stable (AAN 2024)
    Maintenance (AAN 2024)
  2. 2. CGRP-class biologic if refractory (AAN 2024)
    Per agent (efgartigimod, rozanolixizumab, eculizumab, ravulizumab, zilucoplan, rituximab) (AAN 2024) • IV/SC • per agent
    trigger: Refractory gMG (AAN 2024)
    Newer agents (AAN 2024)

Auto-drafted A&P note

outpatient

Subjective

- 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.
References
  • 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