İnflamatuvar Nöropatilerde Acil Tanı ve Tedavi Yöntemleri
Özet
Guillain-Barré Sendromu (GBS), genellikle enfeksiyon sonrasında gelişen, simetrik motor güçsüzlük ve arefleksi ile karakterize akut bir inflamatuvar poliradikülonöropatidir. Batı ülkelerinde yıllık insidansı 100.000 kişide 0,81 – 1,89 arasındadır ve yaşla birlikte artar. Vakaların %41'ine kadarından Campylobacter Jejuni sorumluyken, çeşitli virüsler de hastalığı tetikleyebilir. Klinik olarak, distalden proksimale ilerleyen güçsüzlük, fasiyal sinir tutulumu, otonomik disfonksiyon ve akut dönemde ağrı sık görülür. Tanıda beyin-omurilik sıvısında albüminositolojik disosiasyon ve elektrofizyolojik olarak periferik sinir demyelinizasyonu veya aksonal kayıp bulguları esastır. Akut Motor Aksonal Nöropati (AMAN), AMSAN ve Miller-Fisher Sendromu gibi varyantları bulunur. Tedavide solunum yetmezliği ve kardiyovasküler disotonomi gibi hayati komplikasyonların yakın takibi critical öneme sahiptir; immunoterapi olarak plazmaferez veya IVIg (intravenöz immunglobulin) ilk seçenekler arasında yer alırken kortikosteroidlerin etkinliği yoktur. Hastaların büyük kısmı iyileşirken, %10-15'inde ciddi rezidüel kısıtlılık kalır. Yoğun bakım hastalarında mortalite %7,5'e kadar yükselebilmekte olup ölümler genellikle solunum ve otonom komplikasyonlardan kaynaklanır.
Guillain-Barré Syndrome (GBS) is an acute inflammatory polyradiculoneuropathy characterized by symmetric motor weakness and areflexia, typically developing after an infection. Its annual incidence in Western countries ranges from 0.81 to 1.89 per 100,000 people and increases with age. While Campylobacter Jejuni is responsible for up to 41% of cases, various viruses can also trigger the disease. Clinically, weakness progressing from distal to proximal, facial nerve involvement, autonomic dysfunction, and pain in the acute phase are common. Diagnosis is fundamentally based on albuminocytologic dissociation in cerebrospinal fluid and electrophysiological findings of peripheral nerve demyelination or axonal loss. It includes variants such as Acute Motor Axonal Neuropathy (AMAN), AMSAN, and Miller-Fisher Syndrome. In treatment, close monitoring of vital complications like respiratory failure and cardiovascular dysautonomia is critical; plasmapheresis or IVIg (intravenous immunoglobulin) are preferred immunotherapies, whereas corticosteroids are ineffective. While most patients recover, 10-15% suffer severe residual disability. Mortality can rise up to 7.5% in intensive care patients, with deaths usually resulting from respiratory and autonomic complications.
Referanslar
Donofrio PD. Guillain-Barré Syndrome. Continuum (Minneap Minn). 2017;23:1295-1309. Doi: 10.1212/CON.0000000000000513.
Guillain G, Barré, JA, Strohl, A. Sur un syndrome de radiculonévrite avec hyper-albuminose du liquide céphalo-tachidien sans réaction cellulaire. Remarques sur les caractères cliniques et graphiques des réflexes tendineux. Bul Mém Soc Med Hôp Paris. 1916;40:1462-1470.
Sejvar JJ, Baughman AL, Wise M, et al. Population incidence of Guillain-Barré syndrome: a systematic review and meta-analysis. Neuroepidemiology. 2011;36(2):123-133. Doi:10.1159/000324710
Scallan Walter EJ, Crim SM, Bruce BB, et al. Incidence of Campylobacter-Associated Guillain-Barré Syndrome Estimated from Health Insurance Data. Foodborne Pathog Dis. 2020;17(1):23-28. Doi:10.1089/fpd.2019.2652
Hao Y, Wang W, Jacobs BC, et al. Antecedent infections in Guillain-Barré syndrome: a single-center, prospective study. Ann Clin Transl Neurol. 2019;6(12):2510-2517. Doi:10.1002/acn3.50946
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(4):1110-1115. Doi:10.1212/wnl.51.4.1110
Asbury AK, Cornblath DR. Assessment of current diagnostic criteria for Guillain-Barré syndrome. Ann Neurol. 1990;27 Suppl:S21-S24. Doi:10.1002/ana.410270707
Ropper AH. The Guillain-Barré syndrome. N Engl J Med. 1992;326(17):1130-1136. Doi:10.1056/NEJM199204233261706
Karimzadeh P, Bakhshandeh Bali MK, Nasehi MM, Taheri Otaghsara SM, Ghofrani M. Atypical findings of guillain-barré syndrome in children. Iran J Child Neurol. 2012;6(4):17-22.
Sharma K, Tengsupakul S, Sanchez O, et al. Guillain-Barré syndrome with unilateral peripheral facial and bulbar palsy in a child: A case report. SAGE Open Med Case Rep. 2019;7:2050313X19838750. Published 2019 Mar 21. Doi:10.1177/2050313X1983875
Ruts L, Drenthen J, Jongen JL, et al. Pain in Guillain-Barre syndrome: a long-term follow-up study. Neurology. 2010 Oct 19;75(16):1439-1447. Doi: 10.1212/WNL.0b013e3181f88345.
Mukerji S, Aloka F, Farooq MU, et al. Cardiovascular complications of the Guillain-Barré syndrome. Am J Cardiol. 2009;104(10):1452-1455. Doi:10.1016/j.amjcard.2009.06.069
Fryman E, Saleem S, Singh A. Guillain-Barré Syndrome Induced Dysautonomia Resulting in Cardiac Arrest. Cureus. 2020;12(12):e12149. Published 2020 Dec 18. Doi:10.7759/cureus.12149
Zaeem Z, Siddiqi ZA, Zochodne DW. Autonomic involvement in Guillain-Barré syndrome: an update. Clin Auton Res. 2019;29(3):289-299. Doi:10.1007/s10286-018-0542-y
Chakraborty T, Kramer CL, Wijdicks EFM, et al. Dysautonomia in Guillain-Barré Syndrome: Prevalence, Clinical Spectrum, and Outcomes. Neurocrit Care. 2020;32(1):113-120. Doi:10.1007/s12028-019-00781-w
Wong AH, Umapathi T, Nishimoto Y, et al. Cytoalbuminologic dissociation in Asian patients with Guillain-Barré and Miller Fisher syndromes. J Peripher Nerv Syst. 2015;20(1):47-51. Doi:10.1111/jns.12104
Fokke C, van den Berg B, Drenthen J, et al. Diagnosis of Guillain-Barré syndrome and validation of Brighton criteria. Brain. 2014;137(Pt 1):33-43. Doi:10.1093/brain/awt285
Willison HJ, Jacobs BC, van Doorn PA. Guillain-Barré syndrome. Lancet. 2016;388(10045):717-727. Doi:10.1016/S0140-6736(16)00339-1
Albers JW, Donofrio PD, McGonagle TK. Sequential electrodiagnostic abnormalities in acute inflammatory demyelinating polyradiculoneuropathy. Muscle Nerve. 1985;8(6):528-539. Doi:10.1002/mus.880080609
Vucic S, Cairns KD, Black KR, et al. Neurophysiologic findings in early acute inflammatory demyelinating polyradiculoneuropathy. Clin Neurophysiol. 2004;115(10):2329-2335. Doi:10.1016/j.clinph.2004.05.009
Preston DC., Shapiro BE. (2013). Polyneuropathy. In Electromyography and Neuromuscular Disorders içinde (p.384–416). Elsevier Inc.
Albers JW, Kelly JJ Jr. Acquired inflammatory demyelinating polyneuropathies: clinical and electrodiagnostic features. Muscle Nerve. 1989;12(6):435-451. Doi:10.1002/mus.880120602
Leonhard SE, Mandarakas MR, Gondim FAA, et al. Diagnosis and management of Guillain-Barré syndrome in ten steps. Nat Rev Neurol. 2019;15(11):671-683. Doi:10.1038/s41582-019-0250-9
van den Berg B, Walgaard C, Drenthen J, et al. Guillain-Barré syndrome: pathogenesis, diagnosis, treatment and prognosis. Nat Rev Neurol. 2014;10(8):469-482. Doi:10.1038/nrneurol.2014.121
Hughes, R. A. Guillain–Barré Syndrome p.124–130 Springer-Verlag, 1990.
Ropper AH, Brown RH. (2005) Adams and Victor’s Principles of Neurology (Eighth edit). New York: McGraw-Hill Medical. p. 1110 – 1177
Kuwabara S, Yuki N. Axonal Guillain-Barré syndrome: concepts and controversies. Lancet Neurol. 2013;12(12):1180-1188. Doi:10.1016/S1474-4422(13)70215-1
Yuki N, Kokubun N, Kuwabara S, et al. Guillain-Barré syndrome associated with normal or exaggerated tendon reflexes. J Neurol. 2012;259(6):1181-1190. Doi:10.1007/s00415-011-6330-4
Capasso M, Caporale CM, Pomilio F, et al. Acute motor conduction block neuropathy Another Guillain-Barré syndrome variant. Neurology. 2003;61(5):617-622. Doi:10.1212/wnl.61.5.617
Uncini A, Zappasodi F, Notturno F. Electrodiagnosis of GBS subtypes by a single study: not yet the squaring of the circle. J Neurol Neurosurg Psychiatry. 2015;86(1):5-8. Doi:10.1136/jnnp-2014-308220
Kuwabara S, Yuki N, Koga M, et al. IgG anti-GM1 antibody is associated with reversible conduction failure and axonal degeneration in Guillain-Barré syndrome. Ann Neurol. 1998;44(2):202-208. Doi:10.1002/ana.410440210
Griffin JW, Li CY, Ho TW, et al. Pathology of the motor-sensory axonal Guillain-Barré syndrome. Ann Neurol. 1996;39(1):17-28. Doi:10.1002/ana.410390105
Fisher M. An unusual variant of acute idiopathic polyneuritis (syndrome of ophthalmoplegia, ataxia and areflexia). N Engl J Med. 1956;255(2):57-65. Doi:10.1056/NEJM195607122550201
Mori M, Kuwabara S, Yuki N. Fisher syndrome: clinical features, immunopathogenesis and management. Expert Rev Neurother. 2012;12(1):39-51. Doi:10.1586/ern.11.182
Chiba A, Kusunoki S, Shimizu T, et al. Serum IgG antibody to ganglioside GQ1b is a possible marker of Miller Fisher syndrome. Ann Neurol. 1992;31(6):677-679. Doi:10.1002/ana.410310619
Ryabinkina YV, Piradov MA, Gnedovskaya EV, Suponeva NA, Prokazova PR. Zh Nevrol Psikhiatr Im S S Korsakova. 2016;116(5):13-18. doi:10.17116/jnevro20161165113-18
Hughes RA, Wijdicks EF, Benson E, et al. Supportive care for patients with Guillain-Barré syndrome. Arch Neurol. 2005;62(8):1194-1198. doi:10.1001/archneur.62.8.1194
Hund EF, Borel CO, Cornblath DR, et al. Intensive management and treatment of severe Guillain-Barré syndrome. Crit Care Med. 1993;21(3):433-446. Doi:10.1097/00003246-199303000-00023
Mehta S. Neuromuscular disease causing acute respiratory failure. Respir Care. 2006;51(9):1016-1023.
Pearse RM, Draper A, Grounds RM. Non-invasive ventilation to avoid tracheal intubation in a patient with Guillain-Barré syndrome. Br J Anaesth. 2003;91(6):913-916. Doi:10.1093/bja/aeg252
Vianello A, Bevilacqua M, Arcaro G, et al. Non-invasive ventilatory approach to treatment of acute respiratory failure in neuromuscular disorders. A comparison with endotracheal intubation. Intensive Care Med. 2000;26(4):384-390. Doi:10.1007/s001340051171
Craig VJ. Ford V. Chakrabarti B. Non Invasive Ventilation In The Weaning Of Patients With Acute Guillain Barre Syndrome Requiring Invasive Ventilation. Am J Respir Crit Care Med 2017;195:A3691
Yavagal DR, Mayer SA. Respiratory complications of rapidly progressive neuromuscular syndromes: Guillain-Barré syndrome and myasthenia gravis. Semin Respir Crit Care Med. 2002;23(3):221-229. Doi:10.1055/s-2002-33030
Shang P, Zhu M, Baker M, et al. Mechanical ventilation in Guillain-Barré syndrome. Expert Rev Clin Immunol. 2020;16(11):1053-1064.
Wongsurakiat P, Pierson DJ, Rubenfeld GD. Changing pattern of ventilator settings in patients without acute lung injury: changes over 11 years in a single institution. Chest. 2004;126(4):1281-1291.
Ali MI, Fernández-Pérez ER, Pendem S, et al. Mechanical ventilation in patients with Guillain-Barré syndrome. Respir Care. 2006;51(12):1403-1407.
Nguyen TN, Badjatia N, Malhotra A, et al. Factors predicting extubation success in patients with Guillain-Barré syndrome. Neurocrit Care. 2006;5(3):230-234. Doi:10.1385/NCC:5:3:230
Walgaard C, Lingsma HF, van Doorn PA, et al. Tracheostomy or Not: Prediction of Prolonged Mechanical Ventilation in Guillain-Barré Syndrome. Neurocrit Care. 2017;26(1):6-13. Doi:10.1007/s12028-016-0311-5
Greene-Chandos D, Torbey M. Critical Care of Neuromuscular Disorders. Continuum (Minneap Minn). 2018;24(6):1753-1775.
Cortese I, Chaudhry V, So YT, et al. Evidence-based guideline update: Plasmapheresis in neurologic disorders: report of the Therapeutics and Technology Assessment Subcommittee of the American Academy of Neurology. Neurology. 2011;76(3):294-300.
Appropriate number of plasma exchanges in Guillain-Barré syndrome. The French Cooperative Group on Plasma Exchange in Guillain-Barré Syndrome. Ann Neurol. 1997;41(3):298-306. Doi:10.1002/ana.410410304
Türk Nöroloji Derneği. Guillain-Barré Sendromunda Tedavi Rehberi (01/12/2021 tarihinde https://www.noroloji.org.tr/TNDData/Uploads/files/GBS%20Tedavi%20Rehberi.pdf adresinden ulaşılmıştır).
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;22(6):753-761.
Hughes RA, Swan AV, van Doorn PA. Intravenous immunoglobulin for Guillain-Barré syndrome. Cochrane Database Syst Rev. 2014;2014(9):CD002063. Published 2014 Sep 19.
Randomised trial of plasma exchange, intravenous immunoglobulin, and combined treatments in Guillain-Barré syndrome. Plasma Exchange/Sandoglobulin Guillain-Barré Syndrome Trial Group. Lancet. 1997 Jan 25;349(9047):225-30. PMID: 9014908
Brennan VM, Salomé-Bentley NJ, Chapel HM; Immunology Nurses Study. Prospective audit of adverse reactions occurring in 459 primary antibody-deficient patients receiving intravenous immunoglobulin. Clin Exp Immunol. 2003;133(2):247-251.
Dalakas MC. The use of intravenous immunoglobulin in the treatment of autoimmune neuromuscular diseases: evidence-based indications and safety profile. Pharmacol Ther. 2004;102(3):177-193.
Dalakas MC. High-dose intravenous immunoglobulin and serum viscosity: risk of precipitating thromboembolic events. Neurology. 1994;44(2):223-226. Doi:10.1212/wnl.44.2.223
Ahsan N. Intravenous immunoglobulin induced-nephropathy: a complication of IVIG therapy. J Nephrol. 1998;11(3):157-161.
Ellie E, Combe C, Ferrer X. High-dose intravenous immune globulin and acute renal failure. N Engl J Med. 1992;327(14):1032-1033. Doi:10.1056/nejm199210013271416
Björkander J, Hammarström L, Smith CI, et al. Immunoglobulin prophylaxis in patients with antibody deficiency syndromes and anti-IgA antibodies. J Clin Immunol. 1987;7(1):8-15. Doi:10.1007/BF00915419
Burks AW, Sampson HA, Buckley RH. Anaphylactic reactions after gamma globulin administration in patients with hypogammaglobulinemia. Detection of IgE antibodies to IgA. N Engl J Med. 1986;314(9):560-564. Doi:10.1056/NEJM198602273140907
Elovaara I, Apostolski S, van Doorn P, et al. EFNS guidelines for the use of intravenous immunoglobulin in treatment of neurological diseases: EFNS task force on the use of intravenous immunoglobulin in treatment of neurological diseases [published correction appears in Eur J Neurol. 2009 Apr;16(4):547]. Eur J Neurol. 2008;15(9):893-908. Doi:10.1111/j.1468-1331.2008.02246.x
Walgaard C, Jacobs BC, Lingsma HF, et al. 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(4):275-283.
Kleyweg RP, van der Meché FG. Treatment related fluctuations in Guillain-Barré syndrome after high-dose immunoglobulins or plasma-exchange. J Neurol Neurosurg Psychiatry. 1991;54(11):957-960. doi:10.1136/jnnp.54.11.957
Osterman PO, Fagius J, Säfwenberg J, Wikström B. Early relapse of acute inflammatory polyradiculoneuropathy after successful treatment with plasma exchange. Acta Neurol Scand. 1988;77(4):273-277. doi:10.1111/j.1600-0404.1988.tb05909.x
van Doorn PA, Ruts L, Jacobs BC. Clinical features, pathogenesis, and treatment of Guillain-Barré syndrome. Lancet Neurol. 2008;7(10):939-950. doi:10.1016/S1474-4422(08)70215-1
Verboon C, van Doorn PA, Jacobs BC. Treatment dilemmas in Guillain-Barré syndrome. J Neurol Neurosurg Psychiatry. 2017;88(4):346-352. doi:10.1136/jnnp-2016-314862
van der Meché FG, Schmitz PI. A randomized trial comparing intravenous immune globulin and plasma exchange in Guillain-Barré syndrome. Dutch Guillain-Barré Study Group. N Engl J Med. 1992;326(17):1123-1129. doi:10.1056/NEJM199204233261705
Hughes RA, Brassington R, Gunn AA, van Doorn PA. Corticosteroids for Guillain-Barré syndrome. Cochrane Database Syst Rev. 2016;10(10):CD001446. Published 2016 Oct 24.
van Koningsveld R, Schmitz PI, Meché FG, et al. Effect of methylprednisolone when added to standard treatment with intravenous immunoglobulin for Guillain-Barré syndrome: randomised trial. Lancet. 2004;363(9404):192-196. doi:10.1016/s0140-6736(03)15324-x
Bernsen RA, Jacobs HM, de Jager AE, et al. Residual health status after Guillain-Barré syndrome. J Neurol Neurosurg Psychiatry. 1997;62(6):637-640. Doi:10.1136/jnnp.62.6.637
de Jager AE, Minderhoud JM. Residual signs in severe Guillain-Barré syndrome: analysis of 57 patients. J Neurol Sci. 1991;104(2):151-156. Doi:10.1016/0022-510x(91)90304-p
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(1):310. Published 2015 Sep 2. Doi:10.1186/s13054-015-1037-z
González-Suárez I, Sanz-Gallego I, Rodríguez de Rivera FJ, Arpa J. Guillain-Barré syndrome: natural history and prognostic factors: a retrospective review of 106 cases. BMC Neurol. 2013;13:95. Published 2013 Jul 22. Doi:10.1186/1471-2377-13-95
Walgaard C, Lingsma HF, Ruts L, et al. Prediction of respiratory insufficiency in Guillain-Barré syndrome. Ann Neurol. 2010;67(6):781-787. Doi:10.1002/ana.21976
Luo H, Hong S, Li M, et al. Risk factors for mechanical ventilation in children with Guillain-Barré syndrome. Muscle Nerve. 2020;62(2):214-218. Doi:10.1002/mus.26905
Umer SR, Nisa Q, Kumari M, et al. Clinical Features Indicating the Need for Mechanical Ventilation in Patients with Guillain Barre Syndrome. Cureus. 2019;11(8):e5520. Published 2019 Aug 29. Doi:10.7759/cureus.5520
van den Berg B, Bunschoten C, van Doorn PA, et al. Mortality in Guillain-Barre syndrome. Neurology. 2013;80(18):1650-1654. doi:10.1212/WNL.0b013e3182904fcc
Randomised trial of plasma exchange, intravenous immunoglobulin, and combined treatments in Guillain-Barré syndrome. Plasma Exchange/Sandoglobulin Guillain-Barré Syndrome Trial Group. Lancet. 1997 Jan 25;349(9047):225-30. PMID: 9014908
Alshekhlee A, Hussain Z, Sultan B, et al. Guillain-Barré syndrome: incidence and mortality rates in US hospitals. Neurology. 2008;70(18):1608-1613. doi:10.1212/01.wnl.0000310983.38724.d4
Domínguez-Moreno R, Tolosa-Tort P, Patiño-Tamez A, et al. Mortalidad asociada al diagnóstico de síndrome de Guillain-Barré en adultos ingresados en instituciones del sistema sanitario Mexicano [Mortality associated with a diagnosis of Guillain-Barré syndrome in adults of Mexican health institutions]. Rev Neurol. 2014;58(1):4-10.
Chiò A, Cocito D, Leone M, et al. Guillain-Barré syndrome: a prospective, population-based incidence and outcome survey. Neurology. 2003;60(7):1146-1150. Doi:10.1212/01.wnl.0000055091.96905.d0
