Czech National Guillain-Barré Syndrome Registry

Česká verzia

Authors: M. Škorňa ¹; J. Bednařík ¹; J. Junkerová ²; J. Staněk ³; E. Ehler ⁴; R. Mazanec ⁵; J. Haberlová ⁶; P. Ridzoň ⁷; P. Otruba ⁸; J. Kuchyňka ⁹; L. Strmisková ⁹; T. Božovský ¹⁰; M. Forgáč ¹¹; P. Vaško ¹²; E. Minks ¹³ ; D. Kvasničková ¹; M. Pátá ¹⁴; M. Suchý ¹⁴
Authors place of work: Neurologická klinika LF MU a FN Brno ¹; Neurologická klinika LF OU a FN Ostrava ²; Oddělení dětské neurologie, FN Ostrava ³; Neurologická klinika FZS UP a Pardubické krajské nemocnice, a. s. ⁴; Neurologická klinika 2. LF UK a FN Motol, Praha ⁵; Klinika dětské neurologie 2. LF UK a FN Motol, Praha ⁶; Neurologické oddělení, Thomayerova nemocnice, Praha ⁷; Neurologická klinika LF UP a FN Olomouc ⁸; Neurologická klinika LF UK a FN Hradec Králové ⁹; Neurologická klinika LF UK a FN Plzeň ¹⁰; Neurologická klinika 1. LF UK a VFN v Praze ¹¹; Neurologická klinika 3. LF UK a FN Královské Vinohrady, Praha ¹²; I. neurologická klinika LF MU a FN u sv. Anny v Brně ¹³; Inaverz, o. p. s. ¹⁴
Published in the journal: Cesk Slov Neurol N 2017; 80/113(4): 418-427
Category: Původní práce
doi: https://doi.org/10.14735/amcsnn2017418

Summary

Introduction:
The Guillain-Barré syndrome is an acute inflammatory autoimmune polyradiculo-neuropathy and belongs among rare neurologic diseases. Since poliomyelitis has been almost completely eliminated, it is currently the most frequent cause of acute flaccid paralysis worldwide. The registry was formed in the Czech Republic in 2012 and is an important source of prospectively recorded data.

Aim:
The goal of the registry is to follow up the clinical profile, treatment and 6-month prognosis of Guillain-Barré syndrome patients. Methods: The data are collected prospectively by persons delegated by neuromuscular centres in the Czech Republic. The clinical course is assessed on the basis of the GBS disability scale and muscle strength according to the Medical Research Council sum score for facial muscles and extremity muscles. Detailed clinical investigation is done on admission, before the treatment, immediately after the treatment, 1 month and 6 months after the end of the treatment.

Results:
We enrolled a total 272 cases of Guillain-Barré syndrome patients in the period from 1^st January 2012 to 17^th August 2016. We have complete data for 6 months in 137 of them. 52 were treated with intravenous human immunoglobulin and 85 with plasma exchange. A good clinical outcome after 6 months (ability to walk unassisted) was achieved in 85% of patients. Severe residual neurological deficit persisted after 6 months in 11% of patients. The remaining 6 patients died. We did not find any difference in the efficiency of immunoglobulin vs. plasma exchange in the whole group, in the subgroup of the severely disabled patients and according to sex.

Conclusion:
Medical care centralisation and adherence to the standard of care lead to a good clinical outcome in Guillain-Barré syndrome patients, which is comparable with relevant sources.

Key words:
Guillain-Barré syndrome – intravenous human immunoglobulin – plasma exchange

The authors declare they have no potential conflicts of interest concerning drugs, products, or services used in the study.

The Editorial Board declares that the manuscript met the ICMJE “uniform requirements” for biomedical papers.

Chinese summary - 摘要

捷克国家格林-巴利综合征登记处

介绍：

格林-巴利综合征是一种急性炎症性自身免疫多神经根性神经病变，属于罕见的神经系统疾病。鉴于脊髓灰质炎几乎完全消除，它是全球性急性弛缓麻痹的最常见原因。 2012年，在捷克共和国成立了相关研究联盟，是前瞻性记录数据的重要来源。

目标：

该研究联盟主要采集格林-巴利综合征患者的临床表现、治疗以及6个月预后的相关数据。

方法：数据经由捷克共和国神经肌肉中心授权前瞻性研究人员收集。根据GBS残疾量表对临床症状进行评估，并根据医学研究委员会开发的面部肌肉和四肢肌肉综合评分对肌肉力量进行评估。在患者入院时、治疗前、治疗完成当天、治疗结束后1个月以及6个月进行详细的临床调查。

结果：

在2012年1月1日至2016年8月17日期间，我们共招募了272例格林-巴利综合征患者，其中137位患者拥有6个月的完整数据，52例患者进行了静脉注射人免疫球蛋白，85例患者进行了血浆置换。经过6个月治疗，85％的患者获得了良好的临床疗效（能够独立行走）， 11％的患者仍有严重的残余性神经缺陷，其中还有6名患者死亡。在整个小组、重度残疾患者亚组以及性别分组亚组中，我们没有发现免疫球蛋白与血浆置换疗效存在任何差异。

结论：

与已有数据相比较，集中化医疗和坚持标准化护理将有助于格林-巴利综合征患者的取得良好的临床治疗效果。

关键词：

格林-巴利综合征 - 静脉内人免疫球蛋白 - 血浆置换

Zdroje

1. Katirji B, Koontz D. Disorders of Peripheral Nerves. In: Daroff RB, Fenichel GM, Jankovic J, eds. Bradley’s Neurology in Clinical Practice. 6th ed. Philadelphia: Elsevier Saunders 2012:1955– 64.

2. Willison HJ, Jacobs BC, van Doorn PA. Guillain--Barré syndrome. Lancet 2016;388(10045):717– 27. doi: 10.1016/ S0140-6736(16)00339-1.

3. Yuki N, Hartung HP. Guillain-Barré syndrome. N Engl J Med 2012;366(24):2294– 304. doi: 10.1056/ NEJMra1114525.

4. Wakerley BR, Uncini A, Yuki N, et al. Guillain-Barré and Miller Fisher syndromes – new diagnostic classification. Nat Rev Neurol 2014;10(9):537– 44. doi: 10.1038/ nrneurol.2014.138.

5. Bednařík J, Voháňka S, Ehler E, et al. Standard pro léčbu pacientů s autoimunitními nervosvalovými onemocněními intravenózním lidským imunoglobulinem a plazmaferézou. Cesk Slov Neurol N 2010;73/ 106(5):579– 89.

6. Léčba AutoImunitních NervoSvalových Onemocnění. [cit. 2017 Čer 12]. Dostupné z URL: http:/ / ainso.cz/.

7. Hughes RA, Newsom-Davis JM, Perkin GD, et al. Controlled trial prednisolone in acute polyneuropathy. Lancet 1978;2(8093):750– 3.

8. Kleyweg RP, van der Meché FG, Schmitz PI. Interobserver agreement in the assessment of muscle strength and functional abilities in Guillain-Barré syndrome. Muscle Nerve 1991;14(11):1103– 9.

9. Raphaël JC, Chevret S, Hughes RA, et al. Plasma exchange for Guillain-Barré syndrome. Cochrane Database Syst Rev 2012;7:CD001798. doi: 10.1002/ 14651858.CD001798.pub2.

10. Hughes RA, Swan AV, van Doorn PA. Intravenous immunoglobulin for Guillain-Barré syndrome. Cochrane Database Syst Rev 2014;9:CD002063.

11. 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– 9.

12. 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– 30.

13. Bril V, Ilse WK, Pearce R, et al. Pilot trial of immunoglobulin versus plasma exchange in patients with Guillain-Barré syndrome. Neurology 1996;46(1):100– 3.

14. Diener HC, Haupt WF, Kloss TM, et al. A preliminary, randomized, multicenter study comparing intravenous immunoglobulin, plasma exchange, and immune adsorption in Guillain-Barré syndrome. Eur Neurol 2001;46(2):107– 9.

15. Nomura K, Hamaguchi K, Hosokawa T, et al. A randomized controlled trial comparing intravenous immunoglobulin and plasmapheresis in Guillain-Barré syndrome. Neurological Therapeutics 2001;18(1):69– 81.

16. El-Bayoumi MA, El-Refaey AM, Abdelkader AM, et al. Comparison of intravenous immunoglobulin and plasma exchange in treatment of mechanically ventilated children with Guillain Barré syndrome: a randomized study. Crit Care 2011;15(4):R164. doi: 10.1186/ cc10305.

17. Wang R, Feng A, Sun W, et al. Intravenous immunoglobulin therapy in children with Guillain-Barre syndrome. J Appl Clin Pediatrics 2001;16(4):223– 4.

18. Yuki N, Ang CW, Koga M, et al. Clinical features and response to treatment in Guillain-Barré syndrome associated with antibodies to GM1b ganglioside. Ann Neurol 2000;47(3):314– 21.

19. Kuwabara S, Mori M, Ogawara K, et al. Indicators of rapid clinical recovery in Guillain-Barré syndrome. J Neurol Neurosurg Psychiatry 2001;70(4):560– 2.

20. Jacobs BC, van Doorn PA, Schmitz PI, et al. Campylobacter jejuni infections and anti-GM1 antibodies in Guillain-Barré syndrome. Ann Neurol 1996;40(2):181– 7.

21. van Doorn PA. Diagnosis, treatment and prognosis of Guillain-Barré syndrome (GBS). Presse Med 2013;42(6 Pt 2):e193– 201. doi: 10.1016/ j.lpm.2013.02.328.

22. Chen A, Kim J, Henderson G, et al. Posterior reversible encephalopathy syndrome in Guillain-Barré syndrome. J Clin Neurosci 2015;22(5):914– 6. doi: 10.1016/ j.jocn.2014.11.004.

23. Nabi S, Rajput HM, Badshah M, et al. Posterior reversible encephalopathy syndrome (PRES) as a complication of Guillain-Barre‘ syndrome (GBS). BMJ Case Rep 2016; pii: bcr2016216757. doi: 10.1136/ bcr-2016-216757.

24. Lawn N, Wijdicks EF, Burritt MF. Intravenous immune globulin and pseudohyponatremia. N Engl J Med 1998;339(9):632.

25. Saifudheen K, Jose J, Gafoor VA, et al. Guillain-Barre syndrome and SIADH. Neurology 2011;76(8):701– 4. doi: 10.1212/ WNL.0b013e31820d8b40.

26. Fokke C, van den Berg B, Drenthen J, et al. Diagnosis of Guillain-Barré syndrome and validation of Brighton criteria. Brain 2014;137(1):33– 43. doi: 10.1093/ brain/ awt285.

27. Verma R, Chaudhari TS, Raut TP, et al. Clinico-electrophysiological profile and predictors of functional outcome in Guillain-Barre syndrome (GBS). J Neurol Sci 2013;335(1– 2):105– 11. doi: 10.1016/ j.jns.2013.09.002.

28. Walgaard C, Lingsma HF, Ruts L, et al. Prediction of respiratory insufficiency in Guillain-Barré syndrome. Ann Neurol 2010;67(6):781– 7. doi: 10.1002/ ana.21976.

29. de Boisanger L. Outcomes for patients with Guillain-Barré syndrome requiring mechanical ventilation: a literature review. Ir J Med Sci 2016;185(1):11– 5. doi: 10.1007/ s11845-015-1365-7.

30. Netto AB, Taly AB, Kulkarni GB, et al. Prognosis of patients with Guillain-Barré syndrome requiring mechanical ventilation. Neurol India 2011;59(5):707– 11. doi: 10.4103/ 0028-3886.86545.

31. Fletcher DD, Lawn ND, Wolter TD, et al. Long-term outcome in patients with Guillain-Barré syndrome requiring mechanical ventilation. Neurology 2000;54(12):2311– 5.

32. Witsch J, Galldiks N, Bender A, et al. Long-term outcome in patients with Guillain-Barré syndrome requiring mechanical ventilation. J Neurol 2013;260(5):1367– 74. doi: 10.1007/ s00415-012-6806-x.

33. Köhrmann M, Huttner HB, Nowe T, et al. Mechanical ventilation in Guillain-Barré syndrome: does age influence functional outcome? Eur Neurol 2009;61(6):358– 63. doi: 10.1159/ 000210548.

34. Levin KH. Variants and mimics of Guillain Barré Syndrome. Neurologist 2004;10(2):61– 74.

35. Anadani M, Katirji B. Acute-onset chronic inflammatory demyelinating polyneuropathy: an electrodiagnostic study. Muscle Nerve 2015;52(5):900– 5. doi: 10.1002/ mus.24667.

36. Pacheco LD, Saad AF, Hankins GD, et al. Guillain-Barré Syndrome in pregnancy. Obstet Gynecol 2016;128(5):1105– 10.

37. Večeřa L, Bednařík J. Kazuistika Guillainova-Barrého syndromu u pacienta s renálním karcinomem. Cesk Slov Neurol N 2012;75/ 108(1):84– 7.

38. Vigliani MC, Magistrello M, Polo P, et al. Risk of cancer in patients with Guillain-Barré syndrome (GBS). A population-based study. J Neurol 2004;251(3):321– 6.

39. Walgaard C, Lingsma HF, Ruts L, et al. Early recognition of poor prognosis in Guillain-Barre syndrome. Neurology 2011;76(11):968– 75. doi: 10.1212/ WNL.0b013e3182104407.

40. Fitzpatrick AM, Mann CA, Barry S, et al. An open label clinical trial of complement inhibition in multifocal motor neuropathy. J Peripher Nerv Syst 2011;16(2):84– 91. doi: 10.1111/ j.1529-8027.2011.00328.x.

41. Halstead SK, Zitman FM, Humphreys PD, et al. Eculizumab prevents anti-ganglioside antibody-mediated neuropathy in a murine model. Brain 2008;131(5):1197– 208.

42. Yamaguchi N, Misawa S, Sato Y, et al. A Prospective, Multicenter, Randomized Phase II Study to Evaluate the Efficacy and Safety of Eculizumab in Patients with Guillain-Barré Syndrome (GBS): Protocol of Japanese Eculizumab Trial for GBS (JET-GBS). JMIR Res Protoc 2016;5(4):e210.