Danish Multiple Sclerosis Center, Department of Neurology, Copenhagen University Hospital - Rigshospitalet, Valdemar Hansens Vej 1-23, 2600 Glostrup, Denmark.
Department of Neurology, Odense University Hospital, J. B. Winsløws Vej 4, 5000 Odense, Denmark; Department of Clinical Research, University of Southern Denmark, Campusvej 55, 5220, Odense, Denmark.
J Neurol Sci. 2024 Nov 15;466:123226. doi: 10.1016/j.jns.2024.123226. Epub 2024 Sep 10.
Autoimmune encephalitis (AE) comprises a group of rare, severe neuroinflammatory conditions. Current biomarkers of neuroinflammation are often normal in AE which therefore can be difficult to rule out in patients with seizures, cognitive and/or neuropsychiatric symptoms. Cerebrospinal fluid (CSF) soluble CD27 (sCD27) and soluble B-cell maturation antigen (sBCMA) have high sensitivity for neuroinflammation in other neuroinflammatory conditions. In this exploratory study we investigate the potential of sCD27 and sBCMA in CSF as biomarkers of neuroinflammation in AE.
Concentrations of sCD27 and sBCMA were measured in CSF from 40 AE patients (20 patients were untreated (12 with anti-N-Methyl-d-Aspartate receptor antibodies (NMDA) and 8 with anti-Leucine-rich Glioma-Inactivated 1 antibodies (LGI1)), and 37 symptomatic controls (SCs).
CSF concentrations of sCD27 were increased in untreated NMDA AE patients (median 1571 pg/ml; p < 0.001) and untreated LGI1 AE patients (median 551 pg/ml; p < 0.05) compared to SCs (median 250 pg/ml). CSF sBCMA was increased in untreated NMDA AE patients (median 832 pg/ml) compared to SCs (median 429 pg/ml). CSF sCD27 and sBCMA correlated with the CSF cell count. Receiver operating characteristic curve analysis of untreated AE patients versus SCs showed an area under the curve of 0.97 for sCD27 and 0.76 for sBCMA.
CSF sCD27 is a suitable biomarker of neuroinflammation in AE with an ability to discriminate patients with NMDA AE and LGI1 AE from symptomatic controls. CSF sCD27 may be suited for ruling out AE and other neuroinflammatory conditions in the early phase of the diagnostic work-up.
自身免疫性脑炎(AE)是一组罕见的严重神经炎症性疾病。目前的神经炎症生物标志物在 AE 中通常正常,因此在有癫痫发作、认知和/或神经精神症状的患者中难以排除 AE。脑脊液(CSF)可溶性 CD27(sCD27)和可溶性 B 细胞成熟抗原(sBCMA)在其他神经炎症性疾病中具有较高的神经炎症敏感性。在这项探索性研究中,我们研究了 CSF 中 sCD27 和 sBCMA 作为 AE 神经炎症生物标志物的潜力。
测量了 40 名 AE 患者(20 名未经治疗的患者(12 名抗 N-甲基-D-天冬氨酸受体抗体(NMDA)和 8 名抗亮氨酸丰富胶质瘤失活 1 抗体(LGI1))和 37 名症状性对照者(SCs)的 CSF 中 sCD27 和 sBCMA 的浓度。
未经治疗的 NMDA AE 患者(中位数 1571pg/ml;p<0.001)和未经治疗的 LGI1 AE 患者(中位数 551pg/ml;p<0.05)的 CSF sCD27 浓度高于 SCs(中位数 250pg/ml)。未经治疗的 NMDA AE 患者的 CSF sBCMA 高于 SCs(中位数 832pg/ml)。CSF sCD27 和 sBCMA 与 CSF 细胞计数相关。未经治疗的 AE 患者与 SCs 的受试者工作特征曲线分析显示,sCD27 的曲线下面积为 0.97,sBCMA 为 0.76。
CSF sCD27 是 AE 神经炎症的一种合适的生物标志物,能够区分 NMDA AE 和 LGI1 AE 患者与症状性对照者。CSF sCD27 可能适合在诊断工作的早期阶段排除 AE 和其他神经炎症性疾病。