University of Bonn Medical Centre, Department of Epileptology, Germany.
Seizure. 2013 Mar;22(2):136-40. doi: 10.1016/j.seizure.2012.12.013. Epub 2013 Jan 11.
In people with suspected inflammatory CNS disease, cerebrospinal fluid (CSF) is commonly analyzed. Antibody-associated limbic encephalitis (ab-LE) and anti-NMDAR-encephalitis are recognized as two major syndromes of autoimmune epilepsies. Here, we investigated the diagnostic value of CSF findings in these two entities.
We reviewed patients from our tertiary epilepsy centre with ab-LE and anti-NMDAR-encephalitis in whom CSF examination including oligoclonal bands (OCB) was performed. Ab-LE patients were subdivided according to antibodies (voltage-gated potassium channels, VGKC; glutamic acid decarboxylase, GAD) or presence of onconeural antibodies/presence of tumour into three groups: VGKC-LE, GAD-LE or paraneoplastic LE (PLE). As controls, patients with CSF investigations in whom autoimmune origin was initially assumed but not confirmed later on were included. In addition, a review of published ab-LE and anti-NMDAR-encephalitis cases with reported CSF data was performed.
55 ab-LE (23 VGKC-LE, 25 GAD-LE, 7 PLE) and 14 anti-NMDAR-encephalitis patients were identified at our centre. OCB were significantly more frequent in ab-LE and anti-NMDAR-encephalitis than in controls. Literature review identified 150 ab-LE and 95 NMDAR cases. Analysis of pooled data confirmed that presence of OCB was significantly more frequent in ab-LE and anti-NMDAR-encephalitis (especially in people with GAD-LE and anti-NMDAR encephalitis) as compared to controls. Sensitivity and specificity of OCB in the pooled ab-LE and anti-NMDAR-encephalitis patients was 34% and 96%, respectively. In patients with ab-LE and anti-NMDAR-encephalitis, the likelihood of OCB in CSF was 8.5-fold higher as compared to controls. Furthermore, in the pooled ab-LE and anti-NMDAR-encephalitis patients, cell counts in CSF were more frequently elevated (especially in those with anti-NMDAR encephalitis) than in controls, whereas protein content of CSF was not different between the groups.
OCB, and to a lesser extent cell counts in CSF, appear to be helpful additional CSF markers in the diagnostic evaluation of people presenting with a constellation suggestive for GAD-LE, PLE and anti-NMDAR-encephalitis, prompting subsequent analysis of specific antibodies.
在疑似中枢神经系统炎症性疾病的患者中,通常会对脑脊液(CSF)进行分析。抗体相关边缘性脑炎(ab-LE)和抗 NMDAR 脑炎被认为是两种主要的自身免疫性癫痫综合征。在这里,我们研究了这两种疾病的 CSF 检查结果的诊断价值。
我们回顾了我院三级癫痫中心的 ab-LE 和抗 NMDAR 脑炎患者的资料,这些患者均进行了包括寡克隆带(OCB)在内的 CSF 检查。根据抗体(电压门控钾通道,VGKC;谷氨酸脱羧酶,GAD)或存在神经瘤相关抗体/存在肿瘤,将 ab-LE 患者分为三个亚组:VGKC-LE、GAD-LE 或副肿瘤性 LE(PLE)。作为对照,我们纳入了最初被认为是自身免疫性疾病但后来未被证实的患者。此外,我们还对已发表的 ab-LE 和抗 NMDAR 脑炎病例的 CSF 数据进行了回顾。
我们中心共发现 55 例 ab-LE(23 例 VGKC-LE、25 例 GAD-LE、7 例 PLE)和 14 例抗 NMDAR 脑炎患者。OCB 在 ab-LE 和抗 NMDAR 脑炎患者中比对照组更常见。文献回顾共纳入 150 例 ab-LE 和 95 例 NMDAR 病例。对汇总数据的分析证实,OCB 在 ab-LE 和抗 NMDAR 脑炎患者中更为常见(尤其是在 GAD-LE 和抗 NMDAR 脑炎患者中),对照组中则较少见。OCB 在汇总的 ab-LE 和抗 NMDAR 脑炎患者中的敏感性和特异性分别为 34%和 96%。与对照组相比,ab-LE 和抗 NMDAR 脑炎患者 CSF 中 OCB 的可能性高 8.5 倍。此外,在汇总的 ab-LE 和抗 NMDAR 脑炎患者中,CSF 中的细胞计数更常升高(尤其是抗 NMDAR 脑炎患者),而各组的 CSF 蛋白含量则无差异。
OCB,以及在较小程度上 CSF 中的细胞计数,似乎是有助于诊断疑似 GAD-LE、PLE 和抗 NMDAR 脑炎患者的有价值的 CSF 标志物,提示随后进行特定抗体的分析。