Phys · neurological
Guillain-Barre Syndrome
Also known as GBS · acute inflammatory demyelinating polyradiculoneuropathy · AIDP · Landry ascending paralysis · Miller Fisher syndrome · AMAN · AMSAN · Bickerstaff brainstem encephalitis
Consultant-physician-depth guide to Guillain-Barre syndrome — immune-mediated demyelination of peripheral nerves via molecular mimicry and anti-ganglioside antibodies, ascending weakness with areflexia, CSF albuminocytologic dissociation and nerve conduction studies, IVIG versus plasma exchange, respiratory and autonomic monitoring, and the CIDP boundary — for FRACP DWE and DCE preparation.
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Red flags
- Progressive ascending weakness with areflexia over hours to days — Guillain-Barre until proven otherwise
- Falling vital capacity — intubate electively at FVC below 20 mL/kg or MIP more negative than -30 cmH2O, before desaturation
- Autonomic instability (arrhythmia, blood pressure swings) — a leading cause of death in ventilated patients; needs continuous cardiac monitoring
- Bilateral lower motor neuron facial palsy — a classic GBS sign that should never be attributed to bilateral Bell palsy without considering GBS
- Bulbar weakness and weak cough — imminent aspiration and ventilatory failure
- Weakness still progressing beyond 8 weeks — re-diagnose as CIDP, management changes to corticosteroids
Guillain-Barre Syndrome
The answer first
Guillain-Barre syndrome (GBS) is an acute, immune-mediated polyradiculoneuropathy. The clinical signature is progressive, symmetric, ascending weakness with areflexia that reaches its nadir within 4 weeks. It is the most common cause of acute generalized paralysis since polio was eradicated, and it is a medical emergency: around one in four patients needs mechanical ventilation, and autonomic failure causes a substantial share of deaths. [1]
Three facts organise everything else: [1]
- It is treatable. Both intravenous immunoglobulin (IVIG) and plasma exchange speed recovery if given within 2-4 weeks of onset. Corticosteroids do not work and may worsen the disease.
- The threat to life is respiratory and autonomic, not the weakness itself. Bedside lung function monitoring (FVC, MIP, MEP), not the strength of the grip, decides who goes to ICU and who is intubated.
- The diagnosis is clinical and supported by CSF and nerve conduction studies. A normal CSF in the first week does not exclude GBS — albuminocytologic dissociation appears after 1-2 weeks. [1]
The organising principle on the ward is: diagnose early, monitor lung function relentlessly, treat with IVIG or plasma exchange (not both), never give steroids, and watch the clock for the 8-week boundary that converts the diagnosis to CIDP. [1]
References15ShowHide
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- [2]Leonhard SE, Mandarakas MR, Gondim FAA, et al. Diagnosis and management of Guillain-Barré syndrome in ten steps Nat Rev Neurol, 2019.PMID 31541214
- [3]van den Berg B, Walgaard C, Drenthen J, et al. Guillain-Barré syndrome: pathogenesis, diagnosis, treatment and prognosis Nat Rev Neurol, 2014.PMID 25023340
- [4]The Guillain-Barre Syndrome Study Group Plasmapheresis and acute Guillain-Barré syndrome. The Guillain-Barré syndrome Study Group Neurology, 1985.PMID 4022342
- [5]van der Meche FGA, Schmitz PIM 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
- [6]Plasma Exchange/Sandoglobulin Guillain-Barre Syndrome Trial Group Randomised trial of plasma exchange, intravenous immunoglobulin, and combined treatments in Guillain-Barré syndrome. Plasma Exchange/Sandoglobulin Guillain-Barré Syndrome Trial Group Lancet, 1997.PMID 9014908
- [7]van Koningsveld R, Schmitz PIM, van der Meche FGA, et al. Effect of methylprednisolone when added to standard treatment with intravenous immunoglobulin for Guillain-Barré syndrome: randomised trial Lancet, 2004.PMID 14738791
- [8]Hughes RA, Brassington R, Gunn AA, van Doorn PA Corticosteroids for Guillain-Barré syndrome Cochrane Database Syst Rev, 2016.PMID 27775812
- [9]van Koningsveld R, Steyerberg EW, Hughes RA, et al. A clinical prognostic scoring system for Guillain-Barré syndrome Lancet Neurol, 2007.PMID 17537676
- [10]Walgaard C, Lingsma HF, Ruts L, et al. Prediction of respiratory insufficiency in Guillain-Barré syndrome Ann Neurol, 2010.PMID 20517939
- [11]Fokke C, van den Berg B, Drenthen J, et al. Diagnosis of Guillain-Barré syndrome and validation of Brighton criteria Brain, 2014.PMID 24163275
- [12]Chiba A, Kusunoki S, Obata H, et al. Serum anti-GQ1b IgG antibody is associated with ophthalmoplegia in Miller Fisher syndrome and Guillain-Barré syndrome: clinical and immunohistochemical studies Neurology, 1993.PMID 8413947
- [13]Kusunoki S, Kaida K, Ueda M Antibodies against gangliosides and ganglioside complexes in Guillain-Barré syndrome: new aspects of research Biochim Biophys Acta, 2008.PMID 17976386
- [14]Ang CW, De Klerk MA, Endtz HP, Jacobs BC, et al. Guillain-Barré syndrome- and Miller Fisher syndrome-associated Campylobacter jejuni lipopolysaccharides induce anti-GM1 and anti-GQ1b Antibodies in rabbits Infect Immun, 2001.PMID 11254608
- [15]Cao-Lormeau VM, Blake A, Mons S, et al. Guillain-Barré Syndrome outbreak associated with Zika virus infection in French Polynesia: a case-control study Lancet, 2016.PMID 26948433