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neuro.gbs-aman.v1PRODUCTION
neuro.gbs-aman.v1

Guillain-Barré Syndrome — AMAN (acute motor axonal)

neurologyacuteadultpediatric
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Encounter flow

12/12 authored

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

Current phase

Frame

Detailed

Confirm AMAN — pure motor ascending areflexic weakness + preceding Campylobacter + axonal NCS pattern (Brighton 2011 PMID 24163275; van Doorn 2011 PMID 25023340)

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Advance rule
Set
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AMAN-compatible pattern; cord compression excluded

Patient inputs (13)

Anti-GM1 / GD1a in ~60-80% AMAN; supports diagnosis (Yuki 2007 PMID 22694000)

Reduced CMAP without slowing + preserved sensory NCS confirms AMAN (van Doorn 2011 PMID 25023340)

Age + geographic/ethnic context informs AMAN likelihood (van Doorn 2011 PMID 25023340)

Campylobacter gastroenteritis 1-3 wk prior strong AMAN risk factor (Yuki 2007 PMID 22694000)

Pure motor pattern (preserved sensation) pivots AMAN from AIDP (van Doorn 2011)

Albumino-cytologic dissociation — present in ~50% wk 1, 75% wk 2 (AAN 2012)

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

Exclude transverse myelitis / cord compression (NICE 2024)

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

Autonomic — paroxysmal tachy/brady (IGOS Doets 2018)

Autonomic BP swings (IGOS Doets 2018)

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

Stool Campylobacter culture / PCR — confirms preceding infection (Yuki 2007)

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

Severity triggers (7)

7 need judgement
  • informationallife_threateningrapidly_progressive_quadriparesis
    AMAN often more rapid than AIDP — quadriparesis within 24-48 h; high EGRIS risk → immediate ICU (IGOS Doets 2018 PMID 30247567; Walgaard 2009 PMID 20517939)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverepure_motor_axonal_NCS
    AMAN signature: reduced CMAP without slowing + preserved sensory NCS = pure motor axonal pattern; pivots away from AIDP demyelinating (van Doorn 2011 PMID 25023340)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverepost_campylobacter
    Preceding Campylobacter jejuni gastroenteritis 1-3 wk prior — strong AMAN risk; molecular mimicry between LOS and GM1/GD1a gangliosides (Yuki 2007 PMID 22694000)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereanti-GM1_GD1a_positive
    Anti-GM1 and/or anti-GD1a antibodies positive — supports AMAN diagnosis (~60-80% positivity in AMAN) (Yuki 2007 PMID 22694000)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverepediatric_aman_high
    Pediatric AMAN — high prevalence in Asian children especially during Campylobacter outbreaks (van Doorn 2011 PMID 25023340)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderateslower_recovery_axonal
    AMAN slower long-term recovery than AIDP due to axonal regeneration time (~1 mm/day); extended rehab + IRF preferred over SNF (Walgaard 2011 PMID 21403108)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalmoderatepreserved_sensation_pivot
    AMAN signature: PRESERVED sensation despite areflexic ascending weakness — key clinical pivot from AIDP glove-and-stocking (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

AMAN — IVIG/PLEX (same dose as AIDP) + extended rehab planning (Hughes Cochrane 2014 PMID 25238327; van Doorn 2011)
axis: aman_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)" — AMAN 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: aman_brighton_1_3, non_ambulatory_or_progressive
    Hughes Cochrane 2014 PMID 25238327 — IVIG and PLEX equivalent in AMAN; 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 — equivalent; do NOT combine

outpatient playbook — drug actions (1)

  1. 1. gabapentin / pregabalin taper
    Per pain response • PO • taper
    trigger: Pain resolving
    NICE 2024

Auto-drafted A&P note

outpatient

Subjective

- Possible entry pathways: Pure motor ascending symmetric weakness with PRESERVED sensation; areflexia (van Doorn 2011 PMID 25023340); Preceding Campylobacter jejuni gastroenteritis (diarrhea) 1-3 wk prior — strong AMAN association (Yuki 2007 PMID 22694000); NCS pure motor axonal pattern: reduced CMAP amplitudes WITHOUT slowing; preserved sensory NCS (van Doorn 2011 PMID 25023340).

Objective

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

Assessment

**Guillain-Barré Syndrome — AMAN (acute motor axonal)** (neuro.gbs-aman.v1).
Phenotype framing: AMAN (this engine) vs AIDP (route gbs-aidp; demyelinating + sensory) vs MFS (route gbs-miller-fisher) vs AMSAN (encoded in parent severity) vs botulism vs tick paralysis (van Doorn 2011)
Scope: Confirm AMAN — pure motor ascending areflexic weakness + preceding Campylobacter + axonal NCS pattern (Brighton 2011 PMID 24163275; van Doorn 2011 PMID 25023340)

No severity triggers fired against current inputs.

Plan

Regimen axis: **AMAN — IVIG/PLEX (same dose as AIDP) + extended rehab planning (Hughes Cochrane 2014 PMID 25238327; van Doorn 2011)** — 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 in AMAN; 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 — equivalent; do NOT combine

Setting playbook (outpatient) — Post-acute AMAN — extended rehab (axonal loss → slower recovery); residual disability monitoring; CIDP surveillance; vaccine-delay advice (van Doorn 2011 PMID 25023340)
3. gabapentin / pregabalin taper Per pain response PO taper — Pain resolving (NICE 2024)

Non-pharmacologic actions:
- Continued PT/OT — likely longer course than AIDP (NICE 2024)
- Vaccine timing discussion with neurology (NICE 2024)
- Driving + return-to-work assessment (NICE 2024)

AVOID / contraindication checks:
- No_steroids_alone_for_GBS_AMAN (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)
- Extended_rehab_for_AMAN_axonal_loss (van Doorn 2011)

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)
- extended rehab trajectory AMAN (Walgaard 2011 PMID 21403108)

Setting (outpatient) monitoring:
- Functional recovery at 6 wk, 3 mo, 6 mo, 1 yr, 2 yr (NICE 2024)
- CIDP red-flag watch (van Doorn 2011)

Follow-up plan: Neuro rehab (often longer than AIDP due to axonal loss), CIDP surveillance, psychology (NICE 2024)
- Close-out criterion: Extended rehab + neurology follow-up scheduled

Monitoring phase: q4-6h FVC/NIF, continuous ECG, MRC + disability score; AMAN has slower recovery — extended rehab planning (Walgaard 2011 PMID 21403108)

Disposition

Current setting: outpatient — Post-acute AMAN — extended rehab (axonal loss → slower recovery); residual disability monitoring; CIDP surveillance; vaccine-delay advice (van Doorn 2011 PMID 25023340)

Disposition criteria:
- Routine follow-up; annual neuromuscular review with extended baseline (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] AMAN often more rapid than AIDP — quadriparesis within 24-48 h; high EGRIS risk → immediate ICU (IGOS Doets 2018 PMID 30247567; Walgaard 2009 PMID 20517939)
- [SEVERE] AMAN signature: reduced CMAP without slowing + preserved sensory NCS = pure motor axonal pattern; pivots away from AIDP demyelinating (van Doorn 2011 PMID 25023340)
- [SEVERE] Preceding Campylobacter jejuni gastroenteritis 1-3 wk prior — strong AMAN risk; molecular mimicry between LOS and GM1/GD1a gangliosides (Yuki 2007 PMID 22694000)

Citations

- 2023 EAN/PNS GBS Guideline + Hughes Cochrane 2014 + Yuki 2007 (Campylobacter molecular mimicry) [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