Cerebrospinal fluid findings in aquaporin-4 antibody positive neuromyelitis optica: Results from 211 lumbar punctures
Cerebrospinal fluid findings in aquaporin-4 antibody positive neuromyelitis optica: Results from 211 lumbar punctures
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DOI：
10.1016/j.jns.2011.03.038
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发表时间：
2011-07-15
影响因子：
4.4
通讯作者：
Wildemann, B.
中科院分区：
文献类型：
--
作者：
Jarius, S.;Paul, F.;Wildemann, B.
背景：视神经脊髓炎（NMO，Devic 病）是一种严重致残的中枢神经系统自身免疫性疾病，几十年来一直被认为是多发性硬化症 (MS) 的一个亚型。然而，最近在 NMO 患者亚群 (60-80%) 中发现了高度特异性的血清自身抗体（称为 NMO-IgG 或 AQP4-Ab）。这些抗体随后被证明直接参与该病症的发病机制。 AQP4-Ab 阳性 NMO 现在被认为是一种免疫病理遗传学上独特的疾病。然而，迄今为止，对AQP4-Ab阳性NMO的脑脊液（CSF）知之甚少。目的：系统描述AQP4-Ab阳性NMO或其截头状、纵向广泛性脊髓炎和视神经炎患者的CSF特征。材料和方法：对 89 名 AQP4-Ab 阳性患者的 211 次腰椎穿刺的细胞学和蛋白质生化结果进行回顾性分析，这些患者大多为白种人，患有视神经脊髓炎谱系疾病 (NMOSD)。 结果：大多数患者不存在脑脊液限制性寡核苷酸 IgG 带，这是 MS 的标志。如果存在的话。鞘内 IgG（以及更罕见的 IgM）合成量低且短暂，而且重要的是仅限于急性复发。大约 50% 的样本中存在 CSF 细胞增多，主要是轻微的（中位数为 19 个细胞/病；范围 6-380），并且经常包括中性粒细胞、嗜酸性粒细胞、活化的淋巴细胞和/或浆细胞。急性脊髓炎患者的脑脊液白蛋白/血清比率、总蛋白和脑脊液 L-乳酸水平与疾病活动度以及脊髓病变长度显着相关。腰椎穿刺时，急性脊髓炎患者和急性视神经炎患者的脑脊液检查结果存在显着差异。一些患者在缓解期间也存在细胞增多和血脑脊液屏障功能障碍，可能表明持续的亚临床疾病活动。结论：AQP4-Ab 阳性 NMOSD 的特征是脑脊液特征与 MS 不同。我们的发现对于 MS 和 NMOSD 的鉴别诊断非常重要，并增进了我们对这种破坏性疾病的免疫发病机制的理解。 (C) 2011 Elsevier B.V. 保留所有权利。
Background: Neuromyelitis optica (NMO, Devic disease) is a severely disabling autoimmune disorder of the CNS, which was considered a subtype of multiple sclerosis (MS) for many decades. Recently, however, highly specific serum autoantibodies (termed NMO-IgG or AQP4-Ab) have been discovered in a subset (60-80%) of patients with NMO. These antibodies were subsequently shown to be directly involved in the pathogenesis of the condition. AQP4-Ab positive NMO is now considered an immunopathogenetically distinct disease in its own right. However, to date little is known about the cerebrospinal fluid (CSF) in AQP4-Ab positive NMO.Objective: To describe systematically the CSF profile of AQP4-Ab positive patients with NMO or its formes frustes, longitudinally extensive myelitis and optic neuritis. Material and methods: Cytological and protein biochemical results from 211 lumbar punctures in 89 AQP4-Ab positive patients of mostly Caucasian origin with neuromyelitis optica spectrum disorders (NMOSD) were analysed retrospectively.Results: CSF-restricted oligodonal IgG bands, a hallmark of MS, were absent in most patients. If present. intrathecal IgG (and, more rarely, IgM) synthesis was low, transient, and, importantly, restricted to acute relapses. CSF pleocytosis was present in around 50% of samples, was mainly mild (median, 19 cells/ill; range 6-380), and frequently included neutrophils, eosinophils, activated lymphocytes, and/or plasma cells. Albumin CSF/serum ratios, total protein and CSF L-lactate levels correlated significantly with disease activity as well as with the length of the spinal cord lesions in patients with acute myelitis. CSF findings differed significantly between patients with acute myelitis and patients with acute optic neuritis at the time of LP. Pleocytosis and blood CSF barrier dysfunction were also present during remission in some patients, possibly indicating sustained subclinical disease activity.Conclusion: AQP4-Ab positive NMOSD is characterized by CSF features that are distinct from those in MS. Our findings are important for the differential diagnosis of MS and NMOSD and add to our understanding of the immunopathogenesis of this devastating condition. (C) 2011 Elsevier B.V. All rights reserved.