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neuro.gbs-aidp.v1PRODUCTION
neuro.gbs-aidp.v1

Guillain-Barré Syndrome — AIDP (classic demyelinating)

neurologyacuteadultpediatricpregnancy
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12/12 authored

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

Current phase

Frame

Detailed

Confirm AIDP — symmetric ascending areflexic weakness + preceding infection + demyelinating NCS (Brighton 2011 PMID 24163275; van Doorn 2011 PMID 25023340)

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Advance rule
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Advance when

AIDP-compatible pattern; cord compression excluded

Patient inputs (12)

Demyelinating pattern confirms AIDP vs AMAN/AMSAN (van Doorn 2011 PMID 25023340)

Age + pregnancy/pediatric status drive treatment selection and dose (EAN/PNS 2023)

Preceding infection in 60-70% of AIDP; informs variant + tx urgency (van Doorn 2011 PMID 25023340)

Ascending symmetric pattern + areflexia is Brighton level 1 anchor for AIDP (Brighton 2011 PMID 24163275)

Albumino-cytologic dissociation — present in ~50% wk1, 75% wk2 (AAN 2012)

IgA deficiency screen before IVIG (anaphylaxis risk) (EAN/PNS 2023)

Exclude transverse myelitis / cord compression; enhancing nerve roots support GBS (NICE 2024)

Required pre-IVIG/PLEX in reproductive-age females (EAN/PNS 2023)

20-30-40 rule — FVC <20 mL/kg or NIF magnitude <30 cmH2O triggers elective intubation (AAN 2012)

Autonomic — paroxysmal tachy/brady, asystole risk (IGOS Doets 2018)

Autonomic BP swings — labile HTN/hypotension (IGOS Doets 2018)

MRC sum 0-60 quantifies severity + tracks progression (AAN 2012)

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

Severity triggers (8)

8 need judgement
  • informationallife_threateningrespiratory_failure_FVC<20
    Respiratory failure — FVC <20 mL/kg OR NIF magnitude <30 cmH2O OR severe bulbar weakness; ~1/3 AIDP need MV (AAN 2012; Walgaard 2009 PMID 20517939)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereclassic_ascending_areflexia
    Classic AIDP — symmetric ascending areflexic weakness + glove-and-stocking paresthesia + preceding infection 1-6 wk; demyelinating NCS pattern (Brighton 2011 PMID 24163275; van Doorn 2011 PMID 25023340)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverebulbar_aspiration
    Bulbar weakness with dysphagia / dysarthria / choking — aspiration risk + airway compromise (AAN 2012)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereautonomic_arrhythmia_BP_lability
    Paroxysmal bradycardia/tachycardia + BP swings >40 mmHg + ileus + urinary retention; ~70% AIDP have autonomic dysfunction (IGOS Doets 2018 PMID 30247567)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevererapidly_progressive_24-48h
    Rapid progression to nadir within 24-48 h OR loss of ambulation within 7 d of onset → high ventilation risk (IGOS Doets 2018 PMID 30247567)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverepregnancy_aidp
    AIDP during pregnancy — IVIG preferred over PLEX (logistic + fetal monitoring); MFM consultation + delivery planning (EAN/PNS 2023)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverepediatric_aidp
    Pediatric AIDP (typically 4-10 y) — same IVIG dose 0.4 g/kg/d × 5 d; consider weight-based dosing precision; PLEX possible but technically harder in small children (EAN/PNS 2023)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderateCIDP_transition_>8wk
    Recurrent or progressive weakness >8 wk from initial AIDP onset with relapsing-remitting pattern → CIDP transition (~5% AIDP) (van Doorn 2011 PMID 25023340)
    Trigger could not be auto-evaluated — needs clinician judgement.

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RISK_STRATIFICATIONoptionalDrives severity classification
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Recommended regimen

AIDP — IVIG/PLEX + respiratory support + supportive bundle (Hughes Cochrane 2014 PMID 25238327; EAN/PNS 2023)
axis: aidp_acute_immunomodulation_supportivestep 1 - Step 1 — Acute immunomodulation (within 2-4 wk of onset)
Selected step "Step 1 — Acute immunomodulation (within 2-4 wk of onset)" — AIDP Brighton 1-3 with progressive weakness or non-ambulatory; IgA documented
  • ivig
    first line
    pooled_human_IgG
    0.4 g/kg/d × 5 d (total 2 g/kg) • IV • daily × 5 d (max: 2 g/kg cumulative)
    triggers: brighton_1_3, non_ambulatory_or_progressive
    Hughes Cochrane 2014 PMID 25238327 — IVIG and PLEX equivalent within 2 wk; check IgA pre-IVIG (EAN/PNS 2023)
    rxcui 1426680
  • plasmapheresis (PLEX)
    first line
    apheresis
    5 sessions over 7-10 d (q48h) • IV • 5 sessions q48h
    triggers: iga_deficient, ivig_unavailable, rapidly_progressive
    Raphael Cochrane PMID 22786475 — PLEX equivalent to IVIG; do NOT combine (Hughes Cochrane 2014)

outpatient playbook — drug actions (1)

  1. 1. gabapentin / pregabalin taper
    Per pain response • PO • taper as tolerated
    trigger: Pain resolving
    Most AIDP pain resolves over months (NICE 2024)

Auto-drafted A&P note

outpatient

Subjective

- Possible entry pathways: Classic ascending symmetric areflexic weakness + glove-and-stocking paresthesia (Brighton 2011 PMID 24163275); Preceding infection 1-6 wk prior (Campylobacter / CMV / EBV / Mycoplasma / Zika) (van Doorn 2011 PMID 25023340); NCS demyelinating pattern: segmental slowing + conduction block + prolonged distal latencies + temporal dispersion + reduced F-wave persistence (van Doorn 2011 PMID 25023340).

Objective

- No vitals, labs, or imaging entered for this encounter.

Assessment

**Guillain-Barré Syndrome — AIDP (classic demyelinating)** (neuro.gbs-aidp.v1).
Phenotype framing: AIDP (this engine) vs AMAN (route gbs-aman) vs MFS (route gbs-miller-fisher) vs MG crisis vs botulism vs tick paralysis vs CIDP (>8 wk) (van Doorn 2011)
Scope: Confirm AIDP — symmetric ascending areflexic weakness + preceding infection + demyelinating NCS (Brighton 2011 PMID 24163275; van Doorn 2011 PMID 25023340)

No severity triggers fired against current inputs.

Plan

Regimen axis: **AIDP — IVIG/PLEX + respiratory support + supportive bundle (Hughes Cochrane 2014 PMID 25238327; EAN/PNS 2023)** — step "Step 1 — Acute immunomodulation (within 2-4 wk of onset)".
1. ivig 0.4 g/kg/d × 5 d (total 2 g/kg) IV daily × 5 d (pooled_human_IgG, first line) — Hughes Cochrane 2014 PMID 25238327 — IVIG and PLEX equivalent within 2 wk; check IgA pre-IVIG (EAN/PNS 2023)
2. plasmapheresis (PLEX) 5 sessions over 7-10 d (q48h) IV 5 sessions q48h (apheresis, first line) — Raphael Cochrane PMID 22786475 — PLEX equivalent to IVIG; do NOT combine (Hughes Cochrane 2014)

Setting playbook (outpatient) — Post-acute AIDP — rehab + CIDP surveillance + neuropathic pain titration + vaccine-delay advice (NICE 2024)
3. gabapentin / pregabalin taper Per pain response PO taper as tolerated — Pain resolving (Most AIDP pain resolves over months (NICE 2024))

Non-pharmacologic actions:
- Continued PT/OT (NICE 2024)
- Vaccine timing discussion with neurology (delay live vaccines; influenza vaccine acceptable risk-benefit per CDC ACIP) (NICE 2024)
- Driving + return-to-work assessment (NICE 2024)

AVOID / contraindication checks:
- No_steroids_alone_for_GBS_AIDP (Hughes Cochrane 2014)
- Check_IgA_before_IVIG (EAN/PNS 2023)
- Avoid_succinylcholine_use_rocuronium_with_sugammadex (AAN 2012)
- DVT_prophylaxis_high_immobility_risk (EAN/PNS 2023)
- Do_not_combine_IVIG_and_PLEX (Hughes Cochrane 2014)
- Vaccination_delay_post_AIDP_check_with_neurology (NICE 2024)

Monitoring

Regimen monitoring:
- FVC NIF q4-6h during progression (AAN 2012)
- continuous ECG for autonomic arrhythmias (IGOS Doets 2018)
- BP q1-2h during unstable phase (IGOS Doets 2018)
- MRC sum score daily (AAN 2012)
- IVIG AE TRALI aseptic meningitis thrombosis (EAN/PNS 2023)
- watch for treatment related fluctuation weeks 2-3 (EAN/PNS 2023)

Setting (outpatient) monitoring:
- Functional recovery at 6 wk, 3 mo, 6 mo, 1 yr (NICE 2024)
- CIDP red-flag watch (van Doorn 2011 PMID 25023340)

Follow-up plan: Neuro rehab, vaccine-delay advice, CIDP surveillance (>8 wk recurrence), psychology (NICE 2024)
- Close-out criterion: Rehab + outpatient neurology follow-up scheduled

Monitoring phase: q4-6h FVC/NIF, continuous ECG (autonomic), serial MRC + disability score; watch treatment-related fluctuation wk 2-3 (EAN/PNS 2023)

Disposition

Current setting: outpatient — Post-acute AIDP — rehab + CIDP surveillance + neuropathic pain titration + vaccine-delay advice (NICE 2024)

Disposition criteria:
- Routine follow-up; annual neuromuscular review (NICE 2024)

Escalation triggers (move to higher acuity):
- Worsening / new weakness >8 wk → CIDP workup (van Doorn 2011)

Earlier-Return Triggers

Return-precaution thresholds (watch for):
- [LIFE_THREATENING] Respiratory failure — FVC <20 mL/kg OR NIF magnitude <30 cmH2O OR severe bulbar weakness; ~1/3 AIDP need MV (AAN 2012; Walgaard 2009 PMID 20517939)
- [SEVERE] Classic AIDP — symmetric ascending areflexic weakness + glove-and-stocking paresthesia + preceding infection 1-6 wk; demyelinating NCS pattern (Brighton 2011 PMID 24163275; van Doorn 2011 PMID 25023340)
- [SEVERE] Bulbar weakness with dysphagia / dysarthria / choking — aspiration risk + airway compromise (AAN 2012)

Citations

- 2023 EAN/PNS GBS Diagnosis & Treatment Guideline + Hughes Cochrane 2014 IVIG + Brighton 2011 (Sejvar) + van den Berg/van Doorn 2014 Nat Rev Neurol [PMID:25238327](https://pubmed.ncbi.nlm.nih.gov/25238327/)
- Cited evidence (PMID 22786475) [PMID:22786475](https://pubmed.ncbi.nlm.nih.gov/22786475/)
- Cited evidence (PMID 25023340) [PMID:25023340](https://pubmed.ncbi.nlm.nih.gov/25023340/)
- Cited evidence (PMID 24163275) [PMID:24163275](https://pubmed.ncbi.nlm.nih.gov/24163275/)
- Cited evidence (PMID 20517939) [PMID:20517939](https://pubmed.ncbi.nlm.nih.gov/20517939/)

Last reconciled with current guidelines: 2026-05-22.
References
  • 2023 EAN/PNS GBS Diagnosis & Treatment Guideline + Hughes Cochrane 2014 IVIG + Brighton 2011 (Sejvar) + van den Berg/van Doorn 2014 Nat Rev NeurolPMID:25238327
  • Cited evidence (PMID 22786475)PMID:22786475
  • Cited evidence (PMID 25023340)PMID:25023340
  • Cited evidence (PMID 24163275)PMID:24163275
  • Cited evidence (PMID 20517939)PMID:20517939