Guillain-Barré Syndrome: Experience of an Intensive Care Unit and literature review
Keywords:
Guillain-Barré syndrome, acute inflammatory demyelinating polyneuropathy, axonal neuropathy, Miller-Fisher syndrome, intravenous immunoglobulin, plasmapheresisAbstract
Introduction and objective:Guillain-Barré syndrome (GBS) is a historically well defined entity, however, the advance of scientific knowledge has proven to be a heterogeneous syndrome, with multiple variants,
each one with distinct clinical features and specific pathophysiology. Except for theMiller-Fishervariant of GBS, it is characterized bythe absence of osteotendinous reflexes and ascending flaccid muscle
paralysis of variable magnitude, which may progress to respiratory
failure by paralysis of respiratory muscles. Analytically, the main
characteristic finding is the cerebro-spinal-fluid albumin-cytologic dissociation. The electromyogram helps the diagnosis, but
it’s more useful in identifying the clinical variant. The authors
present the cases of GBS requiring hospitalization in the Intensive
Care Unit (ICU) in our hospital. The aim of this study was to
evaluate the demographic, clinical disease, type of variant
and response to therapy. We also analyzed the complications and
hospitalization time and its relation to the clinical variant of the
disease.
Material and Methods: A retrospective study of GBS cases diagnosed in the last 10 years, requiring hospitalization in the Intensive
Care Unit (from the 31st January 2001 to the 31st January 2011),
through the analysis of their clinical files.
Results: Of the twenty-one patients diagnosed with GBS in the
selected period,seven patients required admission to the ICU, all due
to respiratory failure. It were five men and two women. The mean
age was 50.8 years. A precipitating event was identified in two
patients. Four patients evolved rapidly (less than 5 days to hospitalization in ICU) and all of these had axonal variants. There were
signs of autonomic dysfunction in 43% of patients. All had albumincytologic dissociation. The mean duration of mechanical ventilation
(MV) was 24.4 days, and this was higher in axonal forms.The average
time of stay in ICU and hospital were, respectively, 29.7 days and 93.4
days, being higher in axonal forms. All patients received intravenous
immunoglobulin. There were no complications associated with
treatment.
Conclusion:Axonal variants are associated with a quicker disease
progression and severe neurological deficits, which are reflected in
prolonged time of MV and hospitalization. These are also associated
with lower recovery rates and increased morbidity and mortality.Treatment was directed towards modifying the course of the disease,
and in this respect both plasmapheresis and intravenous human
immunoglobulin have similar efficacy. Supportive care, including respiratory care and treatment of autonomic dysfunction are extremely
important to avoid potentially fatal complications.
Downloads
References
Orlikowski D. Prigent H. Sharshar T. Lofaso F. Raphael JC. Respiratory dysfunction in Guillain-Barré Syndrome. Neurocrit Care 2004; 1(4): 415-422.
Dourado ME. et al. Clinical characteristics of Guillain-Barré syndrome in a tropical country: a Brazilian experience. Acta Neurol Scand 2012;125(1):47-53. (Epub 2011 Mar 24)
Fonseca T. Cardoso T. Perdigão S. Sarmento A. Morgado R. Costa MM. Sindrome de Guillain-Barré. Acta Med Port 2004; 17(2):119-122.
Aladro-Benito Y. et all. Guillain-Barré syndrome in the northern area of Gran Canaria and the island of Lanzarote. Rev Neurol 2002; 35(8):705-710.
Soysal A. et all. Clínico-electrophysiological findings and prognosis of Guillain-Barré syndrome-10 years’experience. Acta Neurol Scand 2011;
(3):181-186.
http://www.gbs.org.uk/history.html - acedido em 31/08/2011
Miller Fisher C. An Unusual Variant of Acute Idiopathic Polyneuritis (Syndrome of Ophthalmoplegia, Ataxia and Areflexia). New Eng J Med 1956; 255:57-65.
Ho TW. et al. Guillain-Barré syndrome in northern China. Relationship to Campylobacter jejuni infection and anti-glycolipid antibodies. Brain 1995; 118(3):597-605.
Ropper, AH. The Guillain-Barré syndrome. N Engl J Med 1992; 326:1130.
Fisher, M. An unusual variant of acute idiopathic polyneuritis (syndrome of ophthalmoplegia, ataxia and areflexia). N Engl J Med 1956; 255:257.
Lo YL. Clinical and immunological spectrum of the Miller Fisher syndrome. Muscle Nerve. 2007;36(5):615-627.
Feasby TE, Gilbert JJ, Brown WF, Bolton CF, Hahn AF, Koopman WF, Zochodne DW. An acute axonal form of Guillain-Barré polyneuropathy. Brain. 1986;109 ( Pt 6):1115-1126.
Ang CW, Jacobs BC, Laman JD. The Guillain-Barre Syndrome: a true case of molecular mimicry. Trends in Immunology. 2004;25(2):61-66.
Griffin JW, Li CY, Ho TW, Tian M, Gao CY, Xue P, Mishu B, Cornblath DR, Macko C, McKhann GM, Asbury AK. Pathology of the motor-sensory axonal Guillain-Barré syndrome. Ann Neurol. 1996;39(1):17-28.
Ropper AH. Unusual clinical variants and signs in Guillain-Barré syndrome. Arch Neurol. 1986;43(11):1150-1152.
Alshekhlee A, Hussain Z, Sultan B, Katirji B. Guillain-Barré syndrome: incidence and mortality rates in US hospitals. Neurology.2008;70(18):1608-1613.
Jacobs BC, Rothbarth PH, van der Mech FG, Herbrink P, Schmitz PI, de Klerk MA, van Doorn PA. The spectrum of antecedent infections in Guillain-Barré syndrome: a case-control study. Neurology. 1998;51(4):1110-1115.
Pritchard, J, Appleton, R, Howard, R, Hughes, RA. Guillain-Barré syndrome seen in users of isotretinoin. BMJ 2004; 328:1537.
Shin IS, Baer AN, Kwon HJ, Papadopoulos EJ, Siegel JN. Guillain-Barré and Miller Fisher syndromes occurring with tumor necrosis factor alpha antagonist therapy. Arthritis Rheum. 2006;54(5):1429-1434.
Zochodne DW. Autonomic involvement in Guillain-Barré syndrome: a review. Muscle Nerve. 1994;17(10):1145-1155.
Ruts L, Drenthen J, Jacobs BC, van Doorn PA, Dutch GBS Study Group. Distinguishing acute-onset CIDP from fluctuating Guillain-Barré syndrome: a prospective study. Neurology. 2010;74(21):1680-1686.
Pritchard J. Guillain-Barré syndrome. Clin Med. 2010;10(4):399-401.
Shah D.N. The spectrum of Guillain-Barré syndrome. Dis Mon. 2010;56(5):262-265.
Raphaél JC, Chevret S, Hughes RA, Annane D. Plasma exchange for Guillain-Barré syndrome. Cochrane Database Syst Rev. 2002; (2):CD001798.
Hughes RA, Swan AV, Raphaél JC, Annane D, van Koningsveld R, van Doorn PA. Immunotherapy for Guillain-Barré syndrome: a systematic review. Brain. 2007;130(Pt 9):2245-2257.
Szczepiorkowski Zbigniew M. et al. Guidelines on the Use of Therapeutic Apheresis in Clinical Practice-Evidence-Based Approach from the Apheresis Applications Committee of the American Society for Apheresis. Journal of Clinical Apheresis 2010. 25:83-177.
Raphael JC, Chevret S, Harboun M, Jars-Guincestre MC, French Guillain-Barré Syndrome Cooperative Group. Intravenous immune globulins in patients with Guillain-Barré syndrome and contraindications to plasma exchange: 3 days versus 6 days. J Neurol Neurosurg Psychiatry. 2001;71(2):235-238.
Fergusson D, Hutton B, Sharma M, Tinmouth A, Wilson K, Cameron DW, Hebert PC. Use of intravenous immunoglobulin for treatment of neurologic conditions: a systematic review. Transfusion. 2005;45(10):1640-1657.
Hughes RA, Swan AV, van Koningsveld R, van Doorn PA. Corticosteroids for Guillain-Barré Syndrome. Cochrane Database Syst Rev. 2006;19;(2):CD001446.
Pritchard J, Gray IA, Idrissova ZR, Lecky BR, Sutton IJ, Swan AV, Willison HJ, Winer JB. A randomized controlled trial of recombinant interferon-beta 1a in Guillain-Barré syndrome. Neurology. 2003;61(9):1282-1284.
Zhang X, Xia J, Ye H. Effect of tripterygium polyglycoside on interleukin-6 in patients with Guillain-Barré syndrome. Chung-Kuo Chung Hsi Chieh Ho Tsa Chih 2000;20:332-334.
Wollinsky KH, et al. CSF filtration is an effective treatment of Guillain-Barré syndrome: a randomized clinical trial. Neurology 2001;57:774-780.
Lawn ND, Fletcher DD, Henderson RD, Wolter TD, Wijdicks EF. Anticipating mechanical ventilation in Guillain-Barré syndrome. Arch Neurol. 2001;58(6):893-898.
Sharshar T, Chevret S, Bourdain F, Raphaél 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(1):278-283.
Fourrier F, Robriquet L, Hurtvent JF, Spagnolo S. A simple functional marker to predict the need for prolonged mechanical ventilation in patients with Guillain-Barré syndrome. Crit Care. 2011; 15 (1): R65.
Hughes RA, Wijdicks EF, Benson E, Cornblath DR, Hahn AF, Meythaler JM, Sladky JT, Barohn RJ, Stevens JC. Supportive care for patients with Guillain-Barré Syndrome. Arch Neurol. 2005;62(8):1194-1198.
Hund EF, Borel CO, Cornblath DR, Hanley DF, McKhann GM. Intensive management and treatment of severe Guillain-Barré syndrome. Crit Care Med. 1993;21(3):433-446.
Pandey CK, Bose N, Garg G, Singh N, Baronia A, Agarwal A, Singh PK, Singh U. Gabapentin for the treatment of pain in guillain-barré syndrome: a double-blinded, placebo-controlled, crossover study. Anesth Analg. 2002;95(6):1719-1723.
Moulin DE, Hagen N, Feasby TE, Amireh R, Hahn A. Pain in Guillain-Barré syndrome. Neurology. 1997;48(2):328-331.
Weiss H, Rastan V, Mullges W, Wagner RF, Toyka KV. Psychotic symptoms and emotional distress in patients with Guillain-Barré syndrome. Eur Neurol 2002;47(2):74-78.
Meythaler JM. Rehabilitation of Guillain-Barré Syndrome. Arch Phys Med Rehabil. 1997;78(8):872-879.
American Neurological Association. Criteria for diagnosis of Guillain-Barré syndrome. Annals of Neurology 1978;3: 565–566.
Hughes RA. et al. Practice paramenter: immunotherapy for Guillain-Barré syndrome: report of the Quality Standards Subcommittee of the American Academy of Neurology. Neurology 2003; 23;61(6):736-740
Additional Files
Published
How to Cite
Issue
Section
License
This work is licensed under a Creative Commons Attribution 4.0 International License.
Copyright (c) 2023 Medicina Interna
Open Access
