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neuro.mg-crisis.core.v1

Myasthenic Crisis

neurologyacuteadultacuteinpatient
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Phase B contract-depth pass (2026-05-14): antibody/phenotype branching (AChR / MuSK / LRP4 / seronegative / refractory-failed-two-therapies / pregnancy / cholinergic-mimic / impending-vs-full-crisis / precipitant-medication-trigger) encoded as severity_triggers — pivot rationale: future subtype engines (e.g., neuro.mg.musk.v1) are Phase C/D expansion targets and the audit gate requires SiblingDifferentiation.sibling_engine_id to resolve in ALL_DOSSIERS (same encoding pivot as TOAST for neuro.ischaemic-stroke.v1 and Hunt-Hess for neuro.sah.core.v1). 5 setting playbooks span the full MG journey: home (patient action plan + caregiver crisis recognition + trigger avoidance + EMS pre-notification) → ed (FVC + NIF q15 min during decompensation; intubation criteria FVC <15 / NIF magnitude <20 / pH <7.32 / bulbar w/ aspiration; STAT IVIG vs PLEX decision; rocuronium + sugammadex NOT succinylcholine) → icu (mechanical ventilation; PLEX 5 sessions q48h OR IVIG 0.4 g/kg × 5 d; withhold AChE-I during ventilation) → inpatient (wean MV as MIP/MEP improve; restart pyridostigmine low; prednisone induction start 15–20 mg/d titrate up to avoid early steroid worsening; steroid-sparing initiation AZA/MMF/MTX/tacrolimus; thymectomy planning if AChR+ <60 y per MGTX) → outpatient (adherence + flare prevention; AChR-Ab titration q6–12 mo; PJP prophylaxis on chronic IS; bone health DEXA + Ca/vit-D + bisphosphonate on long steroid; pregnancy planning; drug-avoidance card; vaccination AVOID live virus on IS; q3–6 mo neuromuscular review). CL-3 depth-pass-2 (2026-05-18): ALL evidence PMIDs PubMed-MCP-VERIFIED — the prior 2026-05-14 8-PMID set was an UNVERIFIED pass with 7/8 wrong-article mis-attributions. Corrected verified anchor set: MGFA International Consensus 2020 Update 33144515 (was 32503870=Alzheimer reaching), MGTX 27509100 (was 27532387), REGAIN 29066163 (was 28893718=dental graft), ADAPT 34146511 (only pre-existing correct), MycarinG 37059507 (was 37236748=ACR aorta), RAISE 37059508 (was 37182524=loneliness), Díaz-Manera rituximab MuSK 22218276 (was 26975912=pelvic exam), Barth IVIg-vs-PLEX RCT 21562253 (was 21525393=IL-32 HIV) + added Vivacity-MG3 nipocalimab 39862879 as guideline-delta FcRn agent. §5.5.1 quantitative tightening: MGTX QMG 6.15 vs 8.99 + prednisone 44 vs 60 mg (p<0.001); ADAPT MG-ADL responder 68% vs 30% OR 4.95 (95% CI 2.21–11.53); Vivacity MG-ADL Δ −1.45 (95% CI −2.38 to −0.52); Barth 69% vs 65% improved; 20-30-40 intubation rule; ~15–20% crisis lifetime, ~75–90% ventilation rate. §5.5.2 Bayesian ros-ddx triple verified/extended (ros/differentials/finding-lrs). Guideline-delta: 2020 MGFA Update remains primary floor; 2025 added nipocalimab (Vivacity-MG3, FDA gMG ≥12 AChR/MuSK) + batoclimab phase-3 to the FcRn class. Sejvar Brighton GBS (seed 20951491) + Tao brainstem-stroke (seed 22678089) could not be positively re-verified via PubMed MCP → NEEDS_SOURCE_REVIEW (non-MG-core cross-reference rows). Full PMID-correction table in src/lib/dossiers/neuro.mg-crisis.core.v1._research-bundle.md. Schema-blocked calculators (surfaced as tickets in docs/framework-audit/shard-3-neuro-sym-state.md; shard-3 file scope forbids registry edits): calc.mgfa_classification (I–V — ocular vs generalised mild/moderate/severe + crisis), calc.mg_adl (Activities of Daily Living 0–24), calc.qmg (Quantitative Myasthenia Gravis 0–39). All three are standard MG severity instruments not yet in clinical-tools-registry.ts. AVOID list (operationalised in precipitant_medication_trigger severity_trigger + ed playbook): fluoroquinolones (cipro/levo/moxi), aminoglycosides (gent/tobra/amika), macrolides (azithro/erythro/telithro), magnesium IV (esp. obstetric), β-blockers (esp. propranolol/timolol), immune-checkpoint inhibitors (nivo/pembro — can induce de-novo fulminant MG → STOP ICI + high-dose steroid + IVIG/PLEX), procainamide, quinidine, D-penicillamine, succinylcholine (use rocuronium + sugammadex). Terminology values populated from canonical MG codes; reconcile with any future manifest update.

Entry points (4)

  • symptom
    Bulbar weakness — dysphagia / dysarthria / dysphonia (AAN 2024)
    bulbar_weakness
  • symptom
    Respiratory failure / paradoxical breathing (AAN 2024)
    respiratory_failure
  • symptom
    Rapidly worsening weakness in known MG (AAN 2024)
    rapidly_worsening_weakness_in_known_mg
  • symptom
    Fluctuating fatigable weakness with diurnal pattern (AAN 2024)
    fatigability

Required inputs (12)

  • agerequired
    demographic • used at CONTEXT
    Age + thymoma screening trigger; older onset (>50) more often AChR-positive (AAN 2024)
  • mg_known_diagnosisrequired
    history • used at CONTEXT
    Known MG with subtype (AChR/MuSK/seronegative) gates IVIG vs PLEX choice (PLEX preferred MuSK) (AAN 2024)
  • recent_infection_or_med_changerequired
    history • used at CONTEXT
    Crisis triggers — infection, surgery, fluoroquinolones, aminoglycosides, magnesium, beta-blockers (AAN 2024)
  • bulbar_scorerequired
    symptom • used at RED_FLAGS
    Bulbar weakness predicts aspiration + airway compromise (AAN 2024)
  • fvc_miprequired
    vital • used at RED_FLAGS
    20-30-40 rule — FVC <20 mL/kg or NIF magnitude <30 → intubation threshold (AAN 2024)
  • rrrequired
    vital • used at RED_FLAGS
    Tachypnoea → impending failure; paradoxical breathing is late (AAN 2024)
  • spo2required
    vital • used at RED_FLAGS
    Late marker; can be preserved until rapid decompensation (AAN 2024)
  • achr_antibody
    lab • used at INITIAL_WORKUP
    AChR-Ab positive 80–85% of generalised MG; informs efgartigimod / rozanolixizumab eligibility (AAN 2024)
  • musk_antibody
    lab • used at INITIAL_WORKUP
    MuSK-Ab → favour PLEX (IVIG less effective); rituximab early (AAN 2024)
  • iga_levelrequired
    lab • used at TREATMENT
    IgA deficiency screen before IVIG (anaphylaxis risk) (AAN 2024)
  • cxr_or_chest_ctrequired
    imaging • used at INITIAL_WORKUP
    Thymoma screen + aspiration / pneumonia trigger (AAN 2024)
  • current_medsrequired
    medication • used at CONTEXT
    Identify offending agents — fluoroquinolones, aminoglycosides, magnesium, beta-blockers, ICIs (AAN 2024)

12-phase flow (11)

  1. 1FRAME
    Acute neuromuscular respiratory failure in known/suspected MG — exclude cholinergic crisis + GBS + botulism (AAN 2024)
    inputs: mg_known_diagnosis
    advance: crisis classified vs cholinergic vs mimic
  2. 2ENTRY
    Recognise bulbar/respiratory failure with fatigability (AAN 2024)
    inputs: age, bulbar_score
    advance: crisis trigger captured
  3. 3CONTEXT
    MG history, antibody status, current immunosuppression, trigger identification (AAN 2024)
    inputs: mg_known_diagnosis, recent_infection_or_med_change, current_meds
    advance: baseline + trigger documented
  4. 4RED_FLAGS
    FVC <20 / MIP magnitude <30 / aspiration / hypercapnia → ICU + early elective intubation (avoid rapid-sequence sux) (AAN 2024)
    inputs: fvc_mip, rr, spo2, bulbar_score
    actions: acute_weakness
    advance: airway secured or stable + ICU bed
  5. 5INITIAL_WORKUP
    CXR/CT chest (thymoma + pneumonia), ABG, AChR/MuSK Ab if not on file, infection workup, IgA before IVIG (AAN 2024)
    inputs: cxr_or_chest_ct, achr_antibody, musk_antibody, iga_level
    advance: baseline labs + imaging back; IgA on chart before IVIG
  6. 6BRANCHING_WORKUP
    Cholinergic crisis (excessive AChE-I) — fasciculations, miosis, secretions; consider edrophonium / drug-holiday trial (AAN 2024)
    advance: cholinergic vs myasthenic distinguished
  7. 7DIFFERENTIAL
    AChR vs MuSK vs seronegative; LEMS; congenital myasthenic syndromes; ICI-induced MG (AAN 2024)
    inputs: achr_antibody, musk_antibody
    advance: subtype assigned
  8. 8TREATMENT
    IVIG 0.4 g/kg/d × 5d OR PLEX 5 sessions (PLEX favoured MuSK + faster onset); pulse high-dose steroids cautiously (transient worsening); HOLD pyridostigmine periintubation; treat trigger (AAN 2024)
    inputs: iga_level
    advance: immunomodulation started + trigger addressed
  9. 9DISPOSITION
    ICU mandatory; thoracic surgery referral if thymoma; neurology lead (AAN 2024)
    advance: ICU + neuro/thoracics consulted
  10. 10MONITORING
    Serial FVC/MIP q4–6h, ABG, swallow assessment daily, response curve to IVIG/PLEX (AAN 2024)
    inputs: fvc_mip
    advance: monitoring plan active
  11. 11FOLLOWUP
    Long-term immunosuppression (steroid + steroid-sparing — azathioprine, MMF, rituximab; new agents efgartigimod/rozanolixizumab/zilucoplan); thymectomy if thymoma or AChR + age <65 (AAN 2024)
    advance: outpatient neuromuscular plan + chronic IS regimen set