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Semin Neurol 2023; 43(05): 791-798
DOI: 10.1055/s-0043-1775750
Review Article

Neuroprognostication: Guillain–Barré Syndrome

Rebecca Traub
1   Department of Neurology, University of North Carolina, Chapel Hill, North Carolina
,
Vinay Chaudhry
1   Department of Neurology, University of North Carolina, Chapel Hill, North Carolina
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Abstract

Guillain–Barré syndrome is an immune-mediated disease of the peripheral nerves characterized by rapidly progressing symmetric weakness, areflexia, and albuminocytological dissociation. Most patients reach their nadir within 2 weeks. Disease severity can be mild to severe, with 20% of patients requiring mechanical ventilation. Intravenous immunoglobulin and plasma exchange are equally effective treatments. Monitoring strength, respiratory function, blood pressure, and heart rate, as well as pain management and rehabilitative therapy are important aspects of management. About 20% of patients require assistance to walk at 6 months. Older age, preceding diarrhea, and lower Medical Research Council (MRC) sum scores predict poor outcome. Death from cardiovascular and respiratory complications can occur in the acute or recovery phases of the illness in 3 to 7% of the patients. Risk factors for mortality include advanced age and disease severity at onset. Neuropathic pain, weakness, and fatigue can be residual symptoms; risk factors for these include axonal loss, sensory involvement, and severity of illness.

Keywords

Guillain–Barré syndrome - acute inflammatory demyelinating polyneuropathy - acute motor axonal neuropathy - acute motor sensory axonal neuropathy


Publication History

Article published online:
03 October 2023

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  • References

  • 1 Rees JH, Soudain SE, Gregson NA, Hughes RA. Campylobacter jejuni infection and Guillain-Barré syndrome. N Engl J Med 1995; 333 (21) 1374-1379
    CrossrefPubMedGoogle Scholar
  • 2 Leonhard SE, van der Eijk AA, Andersen H. et al; IGOS Consortium. An International Perspective on Preceding Infections in Guillain-Barré Syndrome: The IGOS-1000 Cohort. Neurology 2022; 99 (12) e1299-e1313
    CrossrefPubMedGoogle Scholar
  • 3 Sivadon-Tardy V, Orlikowski D, Porcher R. et al. Guillain-Barré syndrome and influenza virus infection. Clin Infect Dis 2009; 48 (01) 48-56
    CrossrefPubMedGoogle Scholar
  • 4 Brannagan III TH, Zhou Y. HIV-associated Guillain-Barré syndrome. J Neurol Sci 2003; 208 (1-2): 39-42
    CrossrefPubMedGoogle Scholar
  • 5 Rudant J, Dupont A, Mikaeloff Y, Bolgert F, Coste J, Weill A. Surgery and risk of Guillain-Barré syndrome: a French nationwide epidemiologic study. Neurology 2018; 91 (13) e1220-e1227
    CrossrefPubMedGoogle Scholar
  • 6 Möhn N, Beutel G, Gutzmer R, Ivanyi P, Satzger I, Skripuletz T. Neurological immune related adverse events associated with nivolumab, ipilimumab, and pembrolizumab therapy-review of the literature and future outlook. J Clin Med 2019; 8 (11) 1777
    CrossrefPubMedGoogle Scholar
  • 7 Chiò A. Cocito D, Leone M. et al. Guillain-Barré syndrome a prospective, population-based incidence and outcome survey. Neurology 2003; 60 (07) 1146-1150
    CrossrefPubMedGoogle Scholar
  • 8 Sejvar JJ, Baughman AL, Wise M, Morgan OW. Population incidence of Guillain-Barré syndrome: a systematic review and meta-analysis. Neuroepidemiology 2011; 36 (02) 123-133
    CrossrefPubMedGoogle Scholar
  • 9 McGrogan A, Madle GC, Seaman HE, de Vries CS. The epidemiology of Guillain-Barré syndrome worldwide. A systematic literature review. Neuroepidemiology 2009; 32 (02) 150-163
    CrossrefPubMedGoogle Scholar
  • 10 Fokke C, van den Berg B, Drenthen J, Walgaard C, van Doorn PA, Jacobs BC. Diagnosis of Guillain-Barré syndrome and validation of Brighton criteria. Brain 2014; 137 (Pt 1): 33-43
    CrossrefPubMedGoogle Scholar
  • 11 Doets AY, Verboon C, van den Berg B. et al; IGOS Consortium. Regional variation of Guillain-Barré syndrome. Brain 2018; 141 (10) 2866-2877
    CrossrefPubMedGoogle Scholar
  • 12 Ruts L, Drenthen J, Jacobs BC, van Doorn PA. Dutch GBS Study Group. Distinguishing acute-onset CIDP from fluctuating Guillain-Barre syndrome: a prospective study. Neurology 2010; 74 (21) 1680-1686
    CrossrefPubMedGoogle Scholar
  • 13 Hughes RAC, Cornblath DR. Guillain-Barré syndrome. Lancet 2005; 366 (9497) 1653-1666
    CrossrefPubMedGoogle Scholar
  • 14 van Doorn PA, Ruts L, Jacobs BC. Clinical features, pathogenesis, and treatment of Guillain-Barré syndrome. Lancet Neurol 2008; 7 (10) 939-950
    CrossrefPubMedGoogle Scholar
  • 15 Yuki N, Hartung HP. Guillain-Barré syndrome. N Engl J Med 2012; 366 (24) 2294-2304
    CrossrefPubMedGoogle Scholar
  • 16 Kuwabara S, Yuki N. Axonal Guillain-Barré syndrome: concepts and controversies. Lancet Neurol 2013; 12 (12) 1180-1188
    CrossrefPubMedGoogle Scholar
  • 17 Mori M, Kuwabara S, Yuki N. Fisher syndrome: clinical features, immunopathogenesis and management. Expert Rev Neurother 2012; 12 (01) 39-51
    CrossrefPubMedGoogle Scholar
  • 18 van den Berg B, Walgaard C, Drenthen J, Fokke C, Jacobs BC, van Doorn PA. Guillain-Barré syndrome: pathogenesis, diagnosis, treatment and prognosis. Nat Rev Neurol 2014; 10 (08) 469-482
    CrossrefPubMedGoogle Scholar
  • 19 Sejvar JJ, Kohl KS, Gidudu J. et al; Brighton Collaboration GBS Working Group. Guillain-Barré syndrome and Fisher syndrome: case definitions and guidelines for collection, analysis, and presentation of immunization safety data. Vaccine 2011; 29 (03) 599-612
    CrossrefPubMedGoogle Scholar
  • 20 Chevret S, Hughes RAC, Annane D. Plasma exchange for Guillain-Barré syndrome. Cochrane Database Syst Rev 2017; 2 (02) CD001798
    CrossrefPubMedGoogle Scholar
  • 21 Gajdos P, Chevret S, Toyka K. Plasma exchange for myasthenia gravis. Cochrane Database Syst Rev 2002; 2002 (04) CD002275
    PubMedGoogle Scholar
  • 22 van der Meché FG, Schmitz PI. Dutch Guillain-Barré Study Group. A randomized trial comparing intravenous immune globulin and plasma exchange in Guillain-Barré syndrome. N Engl J Med 1992; 326 (17) 1123-1129
    CrossrefPubMedGoogle Scholar
  • 23 Plasma Exchange/Sandoglobulin Guillain-Barré Syndrome Trial Group. Randomised trial of plasma exchange, intravenous immunoglobulin, and combined treatments in Guillain-Barré syndrome. Lancet 1997; 349 (9047) 225-230
    CrossrefPubMedGoogle Scholar
  • 24 Walgaard C, Jacobs BC, Lingsma HF. et al; Dutch GBS Study Group. Second intravenous immunoglobulin dose in patients with Guillain-Barré syndrome with poor prognosis (SID-GBS): a double-blind, randomised, placebo-controlled trial. Lancet Neurol 2021; 20 (04) 275-283
    CrossrefPubMedGoogle Scholar
  • 25 Rajabally YA, Uncini A. Outcome and its predictors in Guillain-Barre syndrome. J Neurol Neurosurg Psychiatry 2012; 83 (07) 711-718
    CrossrefPubMedGoogle Scholar
  • 26 Islam Z, Papri N, Ara G. et al. Risk factors for respiratory failure in Guillain-Barré syndrome in Bangladesh: a prospective study. Ann Clin Transl Neurol 2019; 6 (02) 324-332
    CrossrefPubMedGoogle Scholar
  • 27 Walgaard C, Lingsma HF, Ruts L. et al. Prediction of respiratory insufficiency in Guillain-Barré syndrome. Ann Neurol 2010; 67 (06) 781-787
    CrossrefPubMedGoogle Scholar
  • 28 Durand MC, Porcher R, Orlikowski D. et al. Clinical and electrophysiological predictors of respiratory failure in Guillain-Barré syndrome: a prospective study. Lancet Neurol 2006; 5 (12) 1021-1028
    CrossrefPubMedGoogle Scholar
  • 29 Wu X, Li C, Zhang B. et al. Predictors for mechanical ventilation and short-term prognosis in patients with Guillain-Barré syndrome. Crit Care 2015; 19 (01) 310
    CrossrefPubMedGoogle Scholar
  • 30 Sharshar T, Chevret S, Bourdain F, Raphaël JC. French Cooperative Group on Plasma Exchange in Guillain-Barré Syndrome. Early predictors of mechanical ventilation in Guillain-Barré syndrome. Crit Care Med 2003; 31 (01) 278-283
    CrossrefPubMedGoogle Scholar
  • 31 Cheng Y, Liu K, Li C, Zhang W, Wu X, Fang S. Risk factors for mechanical ventilation in patients with Guillain-Barré Syndrome. Neurocrit Care 2022; 37 (01) 121-128
    CrossrefPubMedGoogle Scholar
  • 32 Randhawa MS, Iyer R, Bansal A. et al. Clinical features associated with need for mechanical ventilation in children with Guillain-Barré Syndrome: retrospective cohort from India. Pediatr Crit Care Med 2022; 23 (05) 378-382
    CrossrefPubMedGoogle Scholar
  • 33 International Guillain Barre Outcomes Study (IGOS). . GBS Prognosis Tool. Accessed April 11, 2023 at: https://gbstools.erasmusmc.nl/prognosis-tool
    PubMed
  • 34 Nguyen TN, Badjatia N, Malhotra A, Gibbons FK, Qureshi MM, Greenberg SA. Factors predicting extubation success in patients with Guillain-Barré syndrome. Neurocrit Care 2006; 5 (03) 230-234
    CrossrefPubMedGoogle Scholar
  • 35 Lawn ND, Wijdicks EF. Tracheostomy in Guillain-Barré syndrome. Muscle Nerve 1999; 22 (08) 1058-1062
    CrossrefPubMedGoogle Scholar
  • 36 Fletcher DD, Lawn ND, Wolter TD, Wijdicks EF. Long-term outcome in patients with Guillain-Barré syndrome requiring mechanical ventilation. Neurology 2000; 54 (12) 2311-2315
    CrossrefPubMedGoogle Scholar
  • 37 van den Berg B, Storm EF, Garssen MJP, Blomkwist-Markens PH, Jacobs BC. Clinical outcome of Guillain-Barré syndrome after prolonged mechanical ventilation. J Neurol Neurosurg Psychiatry 2018; 89 (09) 949-954
    CrossrefPubMedGoogle Scholar
  • 38 Alshekhlee A, Hussain Z, Sultan B, Katirji B. Guillain-Barré syndrome: incidence and mortality rates in US hospitals. Neurology 2008; 70 (18) 1608-1613
    CrossrefPubMedGoogle Scholar
  • 39 van den Berg B, Bunschoten C, van Doorn PA, Jacobs BC. Mortality in Guillain-Barre syndrome. Neurology 2013; 80 (18) 1650-1654
    CrossrefPubMedGoogle Scholar
  • 40 Reyes-Vega MF, Soto-Cabezas MG, Soriano-Moreno AN. et al. Clinical features of Guillain-Barré syndrome and factors associated with mortality during the 2019 outbreak in Peru. J Neurol 2023; 270 (01) 369-376
    CrossrefPubMedGoogle Scholar
  • 41 The Italian Guillain-Barré Study Group. The prognosis and main prognostic indicators of Guillain-Barré syndrome. A multicentre prospective study of 297 patients. Brain 1996; 119 (Pt 6): 2053-2061
    CrossrefPubMedGoogle Scholar
  • 42 Hadden RD, Karch H, Hartung HP. et al; Plasma Exchange/Sandoglobulin Guillain-Barré Syndrome Trial Group. Preceding infections, immune factors, and outcome in Guillain-Barré syndrome. Neurology 2001; 56 (06) 758-765
    CrossrefPubMedGoogle Scholar
  • 43 Cornblath DR, Mellits ED, Griffin JW. et al. Motor conduction studies in Guillain-Barré syndrome: description and prognostic value. Ann Neurol 1988; 23 (04) 354-359
    CrossrefPubMedGoogle Scholar
  • 44 Visser LH, Schmitz PI, Meulstee J, van Doorn PA, van der Meché FG. Dutch Guillain-Barré Study Group. Prognostic factors of Guillain-Barré syndrome after intravenous immunoglobulin or plasma exchange. Neurology 1999; 53 (03) 598-604
    CrossrefPubMedGoogle Scholar
  • 45 van Koningsveld R, Steyerberg EW, Hughes RAC, Swan AV, van Doorn PA, Jacobs BC. A clinical prognostic scoring system for Guillain-Barré syndrome. Lancet Neurol 2007; 6 (07) 589-594
    CrossrefPubMedGoogle Scholar
  • 46 Walgaard C, Lingsma HF, Ruts L, van Doorn PA, Steyerberg EW, Jacobs BC. Early recognition of poor prognosis in Guillain-Barré Syndrome. Neurology 2011; 76 (11) 968-975
    CrossrefPubMedGoogle Scholar
  • 47 Doets AY, Lingsma HF, Walgaard C. et al; IGOS Consortium. Predicting outcome in Guillain-Barré Syndrome: international validation of the Modified Erasmus GBS Outcome Score. Neurology 2022; 98 (05) e518-e532
    CrossrefPubMedGoogle Scholar
  • 48 Tan CY, Razali SNO, Goh KJ, Shahrizaila N. The utility of Guillain-Barré syndrome prognostic models in Malaysian patients. J Peripher Nerv Syst 2019; 24 (02) 168-173
    CrossrefPubMedGoogle Scholar
  • 49 Yamagishi Y, Suzuki H, Sonoo M. et al. Markers for Guillain-Barré syndrome with poor prognosis: a multi-center study. J Peripher Nerv Syst 2017; 22 (04) 433-439
    CrossrefPubMedGoogle Scholar
  • 50 McKhann GM, Griffin JW, Cornblath DR, Mellits ED, Fisher RS, Quaskey SA. Plasmapheresis and Guillain-Barré syndrome: analysis of prognostic factors and the effect of plasmapheresis. Ann Neurol 1988; 23 (04) 347-353
    CrossrefPubMedGoogle Scholar
  • 51 Sung EJ, Kim DY, Chang MC, Ko EJ. Prediction of functional outcome in axonal Guillain-Barre Syndrome. Ann Rehabil Med 2016; 40 (03) 481-488
    CrossrefPubMedGoogle Scholar
  • 52 Kuwabara S, Mori M, Ogawara K, Hattori T, Yuki N. Indicators of rapid clinical recovery in Guillain-Barré syndrome. J Neurol Neurosurg Psychiatry 2001; 70 (04) 560-562
    CrossrefPubMedGoogle Scholar
  • 53 Zaeem Z, Siddiqi ZA, Zochodne DW. Autonomic involvement in Guillain-Barré syndrome: an update. Clin Auton Res 2019; 29 (03) 289-299
    CrossrefPubMedGoogle Scholar
  • 54 Chakraborty T, Kramer CL, Wijdicks EFM, Rabinstein AA. Dysautonomia in Guillain-Barré Syndrome: prevalence, clinical spectrum, and outcomes. Neurocrit Care 2020; 32 (01) 113-120
    CrossrefPubMedGoogle Scholar
  • 55 Korinthenberg R, Schessl J, Kirschner J. Clinical presentation and course of childhood Guillain-Barré syndrome: a prospective multicentre study. Neuropediatrics 2007; 38 (01) 10-17
    Article in Thieme ConnectPubMedGoogle Scholar
  • 56 Estrade S, Guiomard C, Fabry V. et al. Prognostic factors for the sequelae and severity of Guillain-Barré syndrome in children. Muscle Nerve 2019; 60 (06) 716-723
    CrossrefPubMedGoogle Scholar
  • 57 Luo H, Hong S, Li M, Wang L, Jiang L. Risk factors for mechanical ventilation in children with Guillain-Barré syndrome. Muscle Nerve 2020; 62 (02) 214-218
    CrossrefPubMedGoogle Scholar
  • 58 Kalita J, Kumar M, Misra UK. Prospective comparison of acute motor axonal neuropathy and acute inflammatory demyelinating polyradiculoneuropathy in 140 children with Guillain-Barré syndrome in India. Muscle Nerve 2018; 57 (05) 761-765
    CrossrefPubMedGoogle Scholar
  • 59 Roodbol J, de Wit MCY, Aarsen FK, Catsman-Berrevoets CE, Jacobs BC. Long-term outcome of Guillain-Barré syndrome in children. J Peripher Nerv Syst 2014; 19 (02) 121-126
    CrossrefPubMedGoogle Scholar
  • 60 Fokkink WR, Walgaard C, Kuitwaard K, Tio-Gillen AP, van Doorn PA, Jacobs BC. Association of albumin levels with outcome in intravenous immunoglobulin-treated Guillain-Barré Syndrome. JAMA Neurol 2017; 74 (02) 189-196
    CrossrefPubMedGoogle Scholar
  • 61 Altmann P, De Simoni D, Kaider A. et al. Increased serum neurofilament light chain concentration indicates poor outcome in Guillain-Barré syndrome. J Neuroinflammation 2020; 17 (01) 86
    CrossrefPubMedGoogle Scholar
  • 62 Martín-Aguilar L, Camps-Renom P, Lleixà C. et al. Serum neurofilament light chain predicts long-term prognosis in Guillain-Barré syndrome patients. J Neurol Neurosurg Psychiatry 2020; jnnp-2020-323899
    PubMedGoogle Scholar
  • 63 Jin M, Liu K, Zhao L. et al. Cerebrospinal fluid neurofilament light chain predicts short-term prognosis in pediatric Guillain-Barré syndrome. Front Neurol 2022; 13: 972367
    CrossrefPubMedGoogle Scholar
  • 64 Kuitwaard K, de Gelder J, Tio-Gillen AP. et al. Pharmacokinetics of intravenous immunoglobulin and outcome in Guillain-Barré syndrome. Ann Neurol 2009; 66 (05) 597-603
    CrossrefPubMedGoogle Scholar
  • 65 Yamagishi Y, Kuwahara M, Suzuki H. et al. Serum IgG anti-GD1a antibody and mEGOS predict outcome in Guillain-Barré syndrome. J Neurol Neurosurg Psychiatry 2020; 91 (12) 1339-1342
    CrossrefPubMedGoogle Scholar
  • 66 Merkies ISJ, Kieseier BC. Fatigue, pain, anxiety and depression in Guillain-Barré syndrome and chronic inflammatory demyelinating polyradiculoneuropathy. Eur Neurol 2016; 75 (3-4): 199-206
    CrossrefPubMedGoogle Scholar
  • 67 Garssen MPJ, Van Koningsveld R, Van Doorn PA. Residual fatigue is independent of antecedent events and disease severity in Guillain-Barré syndrome. J Neurol 2006; 253 (09) 1143-1146
    CrossrefPubMedGoogle Scholar
  • 68 Drenthen J, Jacobs BC, Maathuis EM, van Doorn PA, Visser GH, Blok JH. Residual fatigue in Guillain-Barre syndrome is related to axonal loss. Neurology 2013; 81 (21) 1827-1831
    CrossrefPubMedGoogle Scholar
  • 69 Ruts L, Drenthen J, Jongen JLM. et al.; On behalf of the Dutch GBS Study Group. Pain in Guillain-Barré Syndrome a long-term follow-up study. Neurology 2010; 75 (16) 1439-1447
    CrossrefPubMedGoogle Scholar