Czech National Guillain-Barré Syndrome Registry


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‘ workplace: Neurologická klinika LF MU a FN Brno 1;  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;  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
Published in: Cesk Slov Neurol N 2017; 80/113(4): 418-427
Category: Original Paper
doi: 10.14735/amcsnn2017418

Overview

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 1st January 2012 to 17th 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


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