CC BY 4.0 · J Neuroanaesth Crit Care 2024; 11(02): 077-085
DOI: 10.1055/s-0044-1782509
Review Article

Critical Care in Guillain–Barré Syndrome

G. S. Umamaheswara Rao
1   Former Senior Professor, Department of Neuroanaesthesia and Neurocritical Care, National Institute of Mental Health and Neurosciences (NIMHANS), Bangalore, Karnataka, India
› Author Affiliations

Abstract

Guillain–Barré syndrome (GBS) is an autoimmune polyneuropathy characterized by hyporeflexic neuromuscular paralysis and albuminocytologic dissociation in the cerebrospinal fluid. It is a postinfectious disorder. The most common antecedent illnesses are respiratory tract infection and Campylobacter jejuni infection. After the antecedent infection, specific antibodies are generated that cross-react with gangliosides in the host culminating in demyelination of the peripheral nerves or nerve roots. Complement activation also contributes to nerve degeneration. Bilateral symmetrical progression of the limb weakness occurs over a period of a few days followed by a plateau phase, after which a recovery phase follows. Generalized hypotonia and hyporeflexia characterize the limb weakness. Cerebrospinal fluid analysis shows albuminocytologic dissociation. About one-third of patients develop respiratory failure. Neuropathic pain is a disturbing symptom in GBS. Dysautonomia is very characteristic of GBS. Erasmus GBS respiratory insufficiency score predicts the need for mechanical ventilation. The weaning process from mechanical ventilation mainly depends on the recovery of vital capacity and inspiratory force. The definitive treatment for GBS consists of plasma exchange or intravenous immunoglobulin therapy, both of which are equally efficacious. Seasonal variation has been observed in the occurrence and recovery of GBS. Prognosis of GBS varies widely. Erasmus GBS outcome scale scoring system predicts the ability of the patient to walk independently after 6 months. Several GBS cases have been reported globally during recent pandemic of coronavirus disease 2019. Though GBS is a self-limiting disease, there are quite a few research questions that still remain to be answered.



Publication History

Article published online:
02 May 2024

© 2024. The Author(s). This is an open access article published by Thieme under the terms of the Creative Commons Attribution License, permitting unrestricted use, distribution, and reproduction so long as the original work is properly cited. (https://creativecommons.org/licenses/by/4.0/)

Thieme Medical and Scientific Publishers Pvt. Ltd.
A-12, 2nd Floor, Sector 2, Noida-201301 UP, India

 
  • References

  • 1 Guillain G, Barré JA, Strohl A. Sur un syndrome de radiculonévrite avec hyperalbuminose du liquide céphalo-rachidien sans réaction cellulaire: remarques sur les caractères cliniques et graphiques des réflexes tendineux. Bulletins et mémoires de la Société des Médecins des Hôpitaux de Paris 1916; 40: 1462-1470
  • 2 Afifi AK. The Landry-Guillain-Barré Strohl syndrome 1859 to 1992 a historical perspective. J Family Community Med 1994; 1 (01) 30-34
  • 3 Goodfellow JA, Willison HJ. Guillain-Barré syndrome: a century of progress. Nat Rev Neurol 2016; 12 (12) 723-731
  • 4 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
  • 5 Auger N, Quach C, Healy-Profitós J, Dinh T, Chassé M. Early predictors of Guillain-Barré syndrome in the life course of women. Int J Epidemiol 2018; 47 (01) 280-288
  • 6 Doets AY, Verboon C, van den Berg B. et al; IGOS Consortium. Regional variation of Guillain-Barré syndrome. Brain 2018; 141 (10) 2866-2877
  • 7 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
  • 8 Jacobs BC, Rothbarth PH, van der Meché FG. et al. The spectrum of antecedent infections in Guillain-Barré syndrome: a case-control study. Neurology 1998; 51 (04) 1110-1115
  • 9 van Doorn PA, Kuitwaard K, Walgaard C, van Koningsveld R, Ruts L, Jacobs BC. IVIG treatment and prognosis in Guillain-Barré syndrome. J Clin Immunol 2010; 30 (Suppl 1, Suppl 1): S74-S78
  • 10 Tam CC, Rodrigues LC, Petersen I, Islam A, Hayward A, O'Brien SJ. Incidence of Guillain-Barré syndrome among patients with Campylobacter infection: a general practice research database study. J Infect Dis 2006; 194 (01) 95-97
  • 11 Kuwabara S, Yuki N. Axonal Guillain-Barré syndrome: concepts and controversies. Lancet Neurol 2013; 12 (12) 1180-1188
  • 12 Willison HJ, Yuki N. Peripheral neuropathies and anti-glycolipid antibodies. Brain 2002; 125 (Pt 12): 2591-2625
  • 13 Ogawara K, Kuwabara S, Mori M, Hattori T, Koga M, Yuki N. Axonal Guillain-Barré syndrome: relation to anti-ganglioside antibodies and Campylobacter jejuni infection in Japan. Ann Neurol 2000; 48 (04) 624-631
  • 14 Visser LH, Van der Meché FG, Van Doorn PA. et al; Dutch Guillain-Barré Study Group. Guillain-Barré syndrome without sensory loss (acute motor neuropathy). A subgroup with specific clinical, electrodiagnostic and laboratory features. Brain 1995; 118 (Pt 4): 841-847
  • 15 Yuki N, Ho TW, Tagawa Y. et al. Autoantibodies to GM1b and GalNAc-GD1a: relationship to Campylobacter jejuni infection and acute motor axonal neuropathy in China. J Neurol Sci 1999; 164 (02) 134-138
  • 16 Chiba A, Kusunoki S, Shimizu T, Kanazawa I. Serum IgG antibody to ganglioside GQ1b is a possible marker of Miller Fisher syndrome. Ann Neurol 1992; 31 (06) 677-679
  • 17 Yuki N. Fisher syndrome and Bickerstaff brainstem encephalitis (Fisher-Bickerstaff syndrome). J Neuroimmunol 2009; 215 (1-2): 1-9
  • 18 Hafer-Macko C, Hsieh ST, Li CY. et al. Acute motor axonal neuropathy: an antibody-mediated attack on axolemma. Ann Neurol 1996; 40 (04) 635-644
  • 19 McGonigal R, Rowan EG, Greenshields KN. et al. Anti-GD1a antibodies activate complement and calpain to injure distal motor nodes of Ranvier in mice. Brain 2010; 133 (Pt 7): 1944-1960
  • 20 Susuki K, Rasband MN, Tohyama K. et al. Anti-GM1 antibodies cause complement-mediated disruption of sodium channel clusters in peripheral motor nerve fibers. J Neurosci 2007; 27 (15) 3956-3967
  • 21 Kuitwaard K, van Koningsveld R, Ruts L, Jacobs BC, van Doorn PA. Recurrent Guillain-Barré syndrome. J Neurol Neurosurg Psychiatry 2009; 80 (01) 56-59
  • 22 Schonberger LB, Bregman DJ, Sullivan-Bolyai JZ. et al. Guillain-Barre syndrome following vaccination in the National Influenza Immunization Program, United States, 1976–1977. Am J Epidemiol 1979; 110 (02) 105-123
  • 23 Salmon DA, Proschan M, Forshee R. et al; H1N1 GBS Meta-Analysis Working Group. Association between Guillain-Barré syndrome and influenza A (H1N1) 2009 monovalent inactivated vaccines in the USA: a meta-analysis. Lancet 2013; 381 (9876) 1461-1468
  • 24 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
  • 25 Inloo S, Katibeh P. Guillain-Barre syndrome presenting with bilateral facial nerve palsy. Iran J Child Neurol 2014; 8: 70-72 PubMed
  • 26 Azarisman SMS, Shahrin TCA, Marzuki AO, Fatnoon NNA, Rathor MY. Bilateral facial nerve palsy secondary to an atypical presentation of Guillain-Barré syndrome. IMJ 2009; 8: 41-44
  • 27 Burakgazi AZ, Höke A. Respiratory muscle weakness in peripheral neuropathies. J Peripher Nerv Syst 2010; 15 (04) 307-313
  • 28 Hughes RA, Wijdicks EF, Benson E. et al; Multidisciplinary Consensus Group. Supportive care for patients with Guillain-Barré syndrome. Arch Neurol 2005; 62 (08) 1194-1198
  • 29 Bazán-Rodríguez L, Ruiz-Avalos JA, de Saráchaga AJ, Martinez-Jimenez E, López-Hernández JC, Vargas-Cañas S. Dysautonomia and related outcomes in Guillain-Barre syndrome. Auton Neurosci 2023; 245: 103070
  • 30 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
  • 31 Tan CY, Shahrizaila N, Tan HT, Goh KJ, Tan MP. Cardiovascular autonomic assessment in Guillain-Barré syndrome: a longitudinal study. Neurol India 2022; 70 (05) 1856-1859
  • 32 Song X, Chi C, Song J, Zhu J. Isolated right ventricular Takotsubo cardiomyopathy in Guillain-Barré syndrome. BMC Cardiovasc Disord 2022; 22 (01) 531
  • 33 Peña L, Moreno CB, Gutierrez-Alvarez AM. Pain management in Guillain-Barre syndrome: a systematic review. Neurología 2015; 30 (07) 433-438
  • 34 Kuroda H, Fujihara K, Nakashima I. et al. Pregabalin attenuates dysautonomia as well as painful dysesthesia caused by Guillain–Barré syndrome. Clin Exp Neuroimmunol 2013; 4: 75-78
  • 35 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
  • 36 Lawn ND, Fletcher DD, Henderson RD, Wolter TD, Wijdicks EF. Anticipating mechanical ventilation in Guillain-Barré syndrome. Arch Neurol 2001; 58 (06) 893-898
  • 37 Paul BS, Bhatia R, Prasad K, Padma MV, Tripathi M, Singh MB. Clinical predictors of mechanical ventilation in Guillain-Barré syndrome. Neurol India 2012; 60 (02) 150-153
  • 38 Doets AY, Walgaard C, Lingsma HF. et al; IGOS Consortium. International validation of the Erasmus Guillain-Barré syndrome respiratory insufficiency score. Ann Neurol 2022; 91 (04) 521-531
  • 39 Vianello A, Bevilacqua M, Arcaro G, Gallan F, Serra E. Non-invasive ventilatory approach to treatment of acute respiratory failure in neuromuscular disorders. A comparison with endotracheal intubation. Intensive Care Med 2000; 26 (04) 384-390
  • 40 Plummer AL, Gracey DR. Consensus conference on artificial airways in patients receiving mechanical ventilation. Chest 1989; 96 (01) 178-180
  • 41 Lawn ND, Wijdicks EF. Post-intubation pulmonary function test in Guillain-Barré syndrome. Muscle Nerve 2000; 23 (04) 613-616
  • 42 Shang P, Zhu M, Baker M, Feng J, Zhou C, Zhang HL. Mechanical ventilation in Guillain-Barré syndrome. Expert Rev Clin Immunol 2020; 16 (11) 1053-1064
  • 43 The Guillain-Barré Syndrome Study Group. Plasmapheresis and acute Guillain-Barré syndrome. The Guillain-Barré syndrome Study Group. Neurology 1985; 35 (08) 1096-1104
  • 44 Chevret S, Hughes RA, Annane D. Plasma exchange for Guillain-Barré syndrome. Cochrane Database Syst Rev 2017; 2 (02) CD001798
  • 45 Bauer PR, Ostermann M, Russell L. et al; Nine-I Investigators. Plasma exchange in the intensive care unit: a narrative review. Intensive Care Med 2022; 48 (10) 1382-1396
  • 46 Kaplan AA. Therapeutic plasma exchange: core curriculum 2008. Am J Kidney Dis 2008; 52 (06) 1180-1196
  • 47 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
  • 48 van der Meché FGA, Schmitz PIM. 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
  • 49 Farcas P, Avnun L, Frisher S, Herishanu YO, Wirguin I. Efficacy of repeated intravenous immunoglobulin in severe unresponsive Guillain-Barré syndrome. Lancet 1997; 350 (9093) 1747
  • 50 Orbach H, Katz U, Sherer Y, Shoenfeld Y. Intravenous immunoglobulin: adverse effects and safe administration. Clin Rev Allergy Immunol 2005; 29 (03) 173-184
  • 51 Arumugham VB, Rayi A. Intravenous Immunoglobulin (IVIG). In: StatPearls [Internet]. Treasure Island (FL):: StatPearls Publishing;; 2023. Jan
  • 52 Bondi M, Engel-Haber E, Wolff J, Grosman-Rimon L, Bloch A, Zeilig G. Functional outcomes following inpatient rehabilitation of Guillain-Barré syndrome patients: intravenous immunoglobulins versus plasma exchange. NeuroRehabilitation 2021; 48 (04) 543-551
  • 53 Hughes RA, Swan AV, van Doorn PA. Intravenous immunoglobulin for Guillain-Barré syndrome. Cochrane Database Syst Rev 2014; 2014 (09) CD002063
  • 54 Verboon C, van den Berg B, Cornblath DR. et al; IGOS Consortium. Original research: Second IVIg course in Guillain-Barré syndrome with poor prognosis: the non-randomised ISID study. J Neurol Neurosurg Psychiatry 2020; 91 (02) 113-121
  • 55 Netto AB, Kulkarni GB, Taly AB, Rao GU, Periyavan S, Rao S. A comparison of immunomodulation therapies in mechanically ventilated patients with Guillain Barré syndrome. J Clin Neurosci 2012; 19 (12) 1664-1667
  • 56 Hughes RA, Brassington R, Gunn AA, van Doorn PA. Corticosteroids for Guillain-Barré syndrome. Cochrane Database Syst Rev 2016; 10 (10) CD001446
  • 57 Yuki N, Hartung HP. Guillain-Barré syndrome. N Engl J Med 2012; 366 (24) 2294-2304
  • 58 Halstead SK, Zitman FM, Humphreys PD. et al. Eculizumab prevents anti-ganglioside antibody-mediated neuropathy in a murine model. Brain 2008; 131 (Pt 5): 1197-1208
  • 59 Pineda AA, Minohara M, Kawamura N. et al. Preventive and therapeutic effects of the selective Rho-kinase inhibitor Fasudil on experimental autoimmune neuritis. J Neurol Sci 2011; 306 (1-2): 115-120
  • 60 Zhang G, Lehmann HC, Bogdanova N, Gao T, Zhang J, Sheikh KA. Erythropoietin enhances nerve repair in anti-ganglioside antibody-mediated models of immune neuropathy. PLoS One 2011; 6 (10) e27067
  • 61 Webb AJ, Brain SA, Wood R, Rinaldi S, Turner MR. Seasonal variation in Guillain-Barré syndrome: a systematic review, meta-analysis and Oxfordshire cohort study. J Neurol Neurosurg Psychiatry 2015; 86 (11) 1196-1201
  • 62 Sriganesh K, Netto A, Kulkarni GB, Taly AB, Umamaheswara Rao GS. Seasonal variation in the clinical recovery of patients with Guillain Barré syndrome requiring mechanical ventilation. Neurol India 2013; 61 (04) 349-354
  • 63 Korinthenberg R, Trollmann R, Felderhoff-Müser U. et al. Diagnosis and treatment of Guillain-Barré Syndrome in childhood and adolescence: an evidence- and consensus-based guideline. Eur J Paediatr Neurol 2020; 25: 5-16
  • 64 Nagappa M, Rahul W, Sinha S. et al. Guillain Barre Syndrome in the elderly: experience from a tertiary-care hospital in India. J Clin Neurosci 2017; 46: 45-49
  • 65 López-Hernández JC, Briseño-Godinez ME, Pérez-Valdez EY. et al. Inpatient delirium in Guillain-Barré syndrome: frequency and clinical characteristics in a Mexican hospital. Cureus 2021; 13 (11) e19260
  • 66 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
  • 67 van Koningsveld R, Steyerberg EW, Hughes RA, Swan AV, van Doorn PA, Jacobs BC. A clinical prognostic scoring system for Guillain-Barré syndrome. Lancet Neurol 2007; 6 (07) 589-594
  • 68 Walgaard C, Lingsma HF, Ruts L, van Doorn PA, Steyerberg EW, Jacobs BC. Early recognition of poor prognosis in Guillain-Barre syndrome. Neurology 2011; 76 (11) 968-975
  • 69 Agarwal E, Bhagat A, Srivastava K. et al. Clinical and electrophysiological factors predicting prolonged recovery in children with Guillain-Barré syndrome. Indian J Pediatr 2022; 89 (05) 452-458
  • 70 Netto AB, Taly AB, Kulkarni GB, Rao UG, Rao S. Mortality in mechanically ventilated patients of Guillain Barré syndrome. Ann Indian Acad Neurol 2011; 14 (04) 262-266
  • 71 Nagappa M, Netto AB, Taly AB. et al. Electrophysiological observations in critically ill Guillain-Barre syndrome. Neurol India 2016; 64 (05) 914-920
  • 72 Dhamne MC, Benny R, Singh R. et al. Guillian–Barre' syndrome in patients with SARS-CoV-2: a multicentric study from Maharashtra, India. Ann Indian Acad Neurol 2021; 24 (03) 339-346
  • 73 Bentley SA, Ahmad S, Kobeissy FH, Toklu HZ. Concomitant Guillain-Barré syndrome and COVID-19: a meta-analysis of cases. Medicina (Kaunas) 2022; 58 (12) 1835
  • 74 Abolmaali M, Rezania F, Behnagh AK, Hamidabad NM, Gorji A, Mirzaasgari Z. Guillain-Barré syndrome in association with COVID-19 vaccination: a systematic review. Immunol Res 2022; 70 (06) 752-764
  • 75 Chan LY, Tsui MH, Leung TN. Guillain-Barré syndrome in pregnancy. Acta Obstet Gynecol Scand 2004; 83 (04) 319-325
  • 76 Feldman JM. Cardiac arrest after succinylcholine administration in a pregnant patient recovered from Guillain-Barré syndrome. Anesthesiology 1990; 72 (05) 942-944
  • 77 Papri N, Doets AY, Mohammad QD. et al Validation and adjustment of modified Erasmus GBS outcome score in Bangladesh. Ann Clin Transl Neurol 2022; 9 (08) 1264-1275