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ICU TopicsNeurocritical Care

ICU · Neurocritical Care

Guillain-Barré syndrome and neuromuscular emergencies

Also known as Guillain-Barré syndrome (GBS) · Acute inflammatory demyelinating polyneuropathy (AIDP) · Myasthenia gravis crisis · Miller Fisher syndrome · IVIG vs plasmapheresis

Guillain-Barré syndrome in the ICU — clinical variants and antecedent infections, the 20/30/40 rule and EGRIS for respiratory failure, ICU admission criteria, autonomic dysfunction, CSF and electrodiagnostic support (Brighton validation), EAN/PNS 2023 treatment (IVIg 0.4 g/kg/day for 5 days within 2 weeks or plasma exchange 12-15 L within 4 weeks; no corticosteroids, no second IVIg course, no PE followed by IVIg), treatment-related fluctuations versus A-CIDP, prognosis (mEGOS) and myasthenic crisis.

high21 referencesUpdated 8 Oct 20268 min readSource-verified · Oct 2026

Checked against its sources on 8 Oct 2026

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CICMFFICMEDIC

Red flags

  • 20/30/40 rule: vital capacity under 20 mL/kg, MIP under 30 cmH2O or MEP under 40 cmH2O signals risk of respiratory failure
  • Rapid progression, bulbar dysfunction, bilateral facial weakness or dysautonomia make ventilation highly likely
  • Autonomic involvement (arrhythmias, blood-pressure instability) contributes to mortality of 3-10%
  • Normal CSF protein does not exclude GBS — it is normal in 30-50% in week 1
  • EAN/PNS 2023: IVIg or plasma exchange — no corticosteroids, no second IVIg course, no PE followed by IVIg
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Your progress

Saved on this device.

Target exams

CICMFFICMEDIC

Red flags

  • 20/30/40 rule: vital capacity under 20 mL/kg, MIP under 30 cmH2O or MEP under 40 cmH2O signals risk of respiratory failure
  • Rapid progression, bulbar dysfunction, bilateral facial weakness or dysautonomia make ventilation highly likely
  • Autonomic involvement (arrhythmias, blood-pressure instability) contributes to mortality of 3-10%
  • Normal CSF protein does not exclude GBS — it is normal in 30-50% in week 1
  • EAN/PNS 2023: IVIg or plasma exchange — no corticosteroids, no second IVIg course, no PE followed by IVIg
The one-line answer

GBS is an acute polyradiculoneuropathy; the severe generalised form with respiratory failure affects 20 to 30 per cent. Anticipate the tube with serial vital capacity and respiratory pressures (the 20/30/40 rule) and the EGRIS score; watch for autonomic arrhythmia and blood-pressure instability, which contribute to a 3-10 per cent mortality. Treat patients unable to walk unaided with IVIg 0.4 g/kg/day for 5 days within 2 weeks or plasma exchange 12-15 L in four to five exchanges within 4 weeks — no corticosteroids, no second IVIg course, no plasma exchange followed by IVIg (EAN/PNS 2023).[2][1][10]

Meet the patient

A 45-year-old man presents with four days of progressive leg weakness two weeks after a diarrhoeal illness. He has flaccid weakness in all four limbs, absent reflexes and facial diplegia; vital capacity is falling and his heart rate and blood pressure are swinging. Two questions decide his next hour: is respiratory failure coming? and is the autonomic system unstable?[1]

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References21ShowHide
  1. [1]Leonhard SE, Mandarakas MR, Gondim FAA, et al. Diagnosis and management of Guillain-Barré syndrome in ten steps Nat Rev Neurol, 2019.PMID 31541214
  2. [2]Willison HJ, Jacobs BC, van Doorn PA. Guillain-Barré syndrome Lancet, 2016.PMID 26948435
  3. [3]Lacomis D. Myasthenic crisis Neurocrit Care, 2005.PMID 16377829
  4. [4]van der Meche FG, Schmitz PI; Dutch Guillain-Barre Study Group. A randomized trial comparing intravenous immune globulin and plasma exchange in Guillain-Barré syndrome. Dutch Guillain-Barré Study Group N Engl J Med, 1992.PMID 1552913
  5. [5]French Cooperative Group on Plasma Exchange in Guillain-Barre syndrome. Efficiency of plasma exchange in Guillain-Barré syndrome: role of replacement fluids. French Cooperative Group on Plasma Exchange in Guillain-Barré syndrome Ann Neurol, 1987.PMID 2893583
  6. [6]Walgaard C, Lingsma HF, Ruts L, et al. Prediction of respiratory insufficiency in Guillain-Barré syndrome Ann Neurol, 2010.PMID 20517939
  7. [7]Walgaard C, Lingsma HF, Ruts L, et al. Early recognition of poor prognosis in Guillain-Barre syndrome Neurology, 2011.PMID 21403108
  8. [8]Ruts L, Drenthen J, Jacobs BC, et al. Distinguishing acute-onset CIDP from fluctuating Guillain-Barre syndrome: a prospective study Neurology, 2010.PMID 20427754
  9. [9]Lawn ND, Fletcher DD, Henderson RD, et al. Anticipating mechanical ventilation in Guillain-Barré syndrome Arch Neurol, 2001.PMID 11405803
  10. [10]van Doorn PA, Van den Bergh PYK, Hadden RDM, et al. European Academy of Neurology/Peripheral Nerve Society Guideline on diagnosis and treatment of Guillain-Barré syndrome Eur J Neurol, 2023.PMID 37814552
  11. [11]Plasma Exchange/Sandoglobulin Guillain-Barré Syndrome Trial Group Randomised trial of plasma exchange, intravenous immunoglobulin, and combined treatments in Guillain-Barré syndrome Lancet, 1997.PMID 9014908
  12. [12]Lawn ND, Wijdicks EF Tracheostomy in Guillain-Barré syndrome Muscle Nerve, 1999.PMID 10417787
  13. [13]Fokke C, van den Berg B, Drenthen J, et al. Diagnosis of Guillain-Barré syndrome and validation of Brighton criteria Brain, 2014.PMID 24163275
  14. [14]Hughes RA, Swan AV, van Doorn PA Intravenous immunoglobulin for Guillain-Barré syndrome Cochrane Database Syst Rev, 2014.PMID 25238327
  15. [15]Hughes RA, Brassington R, Gunn AA, et al. Corticosteroids for Guillain-Barré syndrome Cochrane Database Syst Rev, 2016.PMID 27775812
  16. [16]Wijdicks EF, Roy TK BiPAP in early Guillain-Barré syndrome may fail Can J Neurol Sci, 2006.PMID 16583732
  17. [17]Shang P, Zhu M, Baker M, et al. Mechanical ventilation in Guillain-Barré syndrome Expert Rev Clin Immunol, 2020.PMID 33112177
  18. [18]van den Berg B, Walgaard C, Drenthen J, et al. Guillain-Barré syndrome: pathogenesis, diagnosis, treatment and prognosis Nat Rev Neurol, 2014.PMID 25023340
  19. [19]Reilly M, Hutchinson M Suxamethonium is contraindicated in the Guillain-Barré syndrome J Neurol Neurosurg Psychiatry, 1991.PMID 1800653
  20. [20]Hor JY Cardiac arrhythmia after succinylcholine administration in a patient with Guillain-Barré syndrome--a case report Middle East J Anaesthesiol, 2010.PMID 21526679
  21. [21]van Doorn PA, Ruts L, Jacobs BC Clinical features, pathogenesis, and treatment of Guillain-Barré syndrome Lancet Neurol, 2008.PMID 18848313
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