Kollár Katalin, Liptai Zoltán, Rosdy Beáta, Móser Judit
Heim Pál Gyermekkórház, Neurológiai Osztály, Budapest.
Ideggyogy Sz. 2009 Nov 30;62(11-12):399-404.
Guillain-Barré syndrome (GBS) is clinically well known since 1916. It can occur at any age. Its main characteristic is acute rapidly ascending flaccid paresis. It is a neuro-immunologic disorder with heterogeneous background. In Hungary we could not find reports about big paediatric population with GBS.
We analysed retrospectively the data of 38 children diagnosed and treated with GBS at the Neurological Department of Paul Heim Children's Hospital or at the Paediatric Department of St. László Hospital from January 2000 till April 2008. We analysed the clinical characteristics, seriousness of clinical signs, laboratory results, and electrophysiological features of them as well documented the preceding illness. We observed the effectiveness of our treatment; we measured the speed and time of the healing process and documented the residual clinical signs.
35 children could be classified as having acute inflammatory demyelinating polyneuropathy (AIDP), 2 as having acute motor axonal neuropathy (AMAN) and 1 as Miller-Fisher syndrome. By those patients who at the very beginning did not show the characteristic clinical signs, electrophysiology helped in establishing the diagnosis. By one child spinal MRI with gadolinium supported our diagnosis. Those children, who lost their ambulation, got immunotherapy: intravenous immunoglobulin (IVIG) or plasmapheresis (PEX). Both method seemed to be effective. None of our patients died. All were cured. By five patients residual clinical symptoms could be found.
The disease process, the relative incidence of each subtype of GBS is nearly similar to that in Western Europe and North America according to the literature. By the currently used immune therapy most of the pediatric patients recover fully within a short time.
吉兰 - 巴雷综合征(GBS)自1916年起在临床上就广为人知。它可发生于任何年龄。其主要特征是急性快速进展的弛缓性麻痹。它是一种背景各异的神经免疫性疾病。在匈牙利,我们未找到关于大量儿童GBS患者的报道。
我们回顾性分析了2000年1月至2008年4月期间在保罗·海姆儿童医院神经科或圣拉兹洛医院儿科诊断并接受治疗的38例GBS患儿的数据。我们分析了他们的临床特征、临床症状的严重程度、实验室检查结果以及电生理特征,并记录了前驱疾病。我们观察了治疗效果;测量了恢复过程的速度和时间,并记录了残留的临床症状。
35例患儿可归类为急性炎症性脱髓鞘性多发性神经病(AIDP),2例为急性运动轴索性神经病(AMAN),1例为米勒 - 费希尔综合征。对于那些一开始未表现出特征性临床症状的患者,电生理检查有助于确诊。有1例患儿的脊髓钆增强磁共振成像(MRI)支持了我们的诊断。那些失去行走能力的患儿接受了免疫治疗:静脉注射免疫球蛋白(IVIG)或血浆置换(PEX)。两种方法似乎都有效。我们的患者均无死亡。全部治愈。有5例患者存在残留临床症状。
根据文献,GBS的疾病进程、各亚型的相对发病率与西欧和北美几乎相似。通过目前使用的免疫治疗,大多数儿科患者在短时间内可完全康复。