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伴有显著脑脊液细胞增多或脑脊液中多形核粒细胞比例显著的吉兰-巴雷综合征:5例神经病理学研究及鉴别诊断综述

Guillain-Barré syndrome with marked pleocytosis or a significant proportion of polymorphonuclear granulocytes in the cerebrospinal fluid: neuropathological investigation of five cases and review of differential diagnoses.

作者信息

Rauschka H, Jellinger K, Lassmann H, Braier F, Schmidbauer M

机构信息

Department of Neurology, Hospital Lainz, Vienna, Austria.

出版信息

Eur J Neurol. 2003 Sep;10(5):479-86. doi: 10.1046/j.1468-1331.2003.00644.x.

DOI:10.1046/j.1468-1331.2003.00644.x
PMID:12940826
Abstract

In cases with otherwise clinically typical Guillain-Barré syndrome (GBS), pronounced cerebrospinal fluid (CSF) pleocytosis or the mere presence of CSF-polymorphonuclear granulocytes should alert the physician to consider alternative diagnoses. Therefore, we retrospectively studied the neuropathology of central and peripheral nervous system in two cases with a CSF cell count of more than 50/microl and in three cases with a significant proportion of polymorphonuclear granulocytes in the CSF sediment. All cases fulfilled the required criteria for the diagnosis of GBS, the duration from onset to death ranged from 4 to 100 days. Neuropathological investigations included routine staining procedures and immunohistochemistry for antigens of glial and haematopoetic cells as well as for products of relevant neurotropic viruses. Demyelinating polyradiculitis was present in four cases, in one patient with a survival time of 4 days the type of damage to myelinated fibres was unclassifiable. In the central nervous system a consistent finding was diffuse activation of microglia, only one case showed mild meningeal and lower brainstem inflammation. Viral products were generally absent. In summary, the neuropathological findings confirm that marked CSF pleocytosis or the presence of polymorphonuclear granulocytes does not rule out the diagnosis of GBS.

摘要

在临床上具有典型吉兰-巴雷综合征(GBS)表现的病例中,显著的脑脊液(CSF)细胞增多或脑脊液中仅存在多形核粒细胞应提醒医生考虑其他诊断。因此,我们回顾性研究了2例脑脊液细胞计数超过50/微升以及3例脑脊液沉淀物中多形核粒细胞比例显著的病例的中枢和周围神经系统神经病理学。所有病例均符合GBS诊断的必要标准,从发病到死亡的时间为4至100天。神经病理学检查包括常规染色程序以及针对胶质细胞和造血细胞抗原以及相关嗜神经病毒产物的免疫组织化学检查。4例存在脱髓鞘性多发性神经根炎,1例存活4天的患者中,有髓纤维的损伤类型无法分类。在中枢神经系统中,一致的发现是小胶质细胞弥漫性激活,仅1例显示轻度脑膜和脑干下部炎症。通常未发现病毒产物。总之,神经病理学结果证实,显著的脑脊液细胞增多或多形核粒细胞的存在并不排除GBS的诊断。

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