Lyon Neuroscience Research Center (V.D., A.C., C. Bost, F.D., V.R., C. Blanc, O.P., J.-C.A., J.H.), INSERM U1028/CNRS UMR 5292; Université de Lyon-Université Claude Bernard Lyon 1 (V.D., A.C., C. Bost, F.D., V.R., C. Bardel, O.P., J.H.); the Department of Neurology D (V.D.) and French Reference Center on Paraneoplastic Neurological Syndrome (A.V., C. Bost, F.D., G.P., V.R., M.-O.C., C. Blanc, J.-C.A., J.-Y.D., J.H.), Hospices Civils de Lyon, Hôpital Neurologique, Bron; Service de Biostatistique (C. Bardel), Hospices Civils de Lyon, CNRS UMR 5558; Laboratoire de Biométrie et Biologie Evolutive (C. Bardel), Equipe Biostatistique-Santé, Villeurbanne; the Department of Biopathology (I.T.), Centre de Recherche en Cancérologie de Lyon; Service de Neurologie (J.-C.A.), CHU de Saint-Etienne et Université de Lyon, Saint-Etienne; and Service de Neurologie Mazarin (J.-Y.D.), Groupe Hospitalier Pitié-Salpêtrière, APHP, Université Pierre et Marie Curie-Paris 6, Centre de Recherche de l'Institut du Cerveau et de la Moelle Épinière, UMR S975, CNRS, UMR 7225, Paris, France.
Neurol Neuroimmunol Neuroinflamm. 2015 Oct 29;2(6):e166. doi: 10.1212/NXI.0000000000000166. eCollection 2015 Dec.
To evaluate the presence of immunoglobulin A (IgA) subtype of anti-NMDA receptor (NMDAR) antibodies (IgA-NMDAR-Abs) in the CSF of patients with immunoglobulin G (IgG)-NMDAR-Ab encephalitis and to describe the potential association with a specific clinical pattern.
The retrospective analysis for the presence of IgA-NMDAR-Abs in 94 CSF samples from patients with anti-NMDAR encephalitis diagnosed between October 2007 and February 2014 was conducted at the French Reference Centre on Paraneoplastic Neurological Syndrome. This observational study compared 39 patients with both IgA- and IgG-NMDAR-Abs to 55 patients with only IgG-NMDAR-Abs.
In the retrospective cohort, 41% of the patients with NMDAR-Ab encephalitis had both CSF IgG- and IgA-NMDAR-Abs. Approximately half of the IgA-NMDAR-Ab-positive patients (18/38, 49%) definitively possessed associated tumors, primarily ovarian teratomas (17/18, 94%), compared with only 5% (3/55) of the patients in the IgA-NMDAR-Ab-negative group (p < 0.001). In the adult female population at risk for ovarian teratoma, the detection of CSF IgA-NMDAR-Ab positivity showed 85% sensitivity, 70% specificity, a 57% positive predictive value, and a 90% negative predictive value for the diagnosis of ovarian teratoma. No other specific clinical features or clinical outcome were associated with CSF IgA-NMDAR-Ab positivity.
These results suggest that in patients with IgG-NMDAR-Ab encephalitis, CSF IgA-NMDAR-Abs could be used as a biological marker for the presence of an ovarian teratoma.
评估免疫球蛋白 A（IgA）亚型抗 N-甲基-D-天冬氨酸受体（NMDAR）抗体（IgA-NMDAR-Abs）在 IgG-NMDAR-Ab 脑炎患者脑脊液中的存在情况，并描述其与特定临床特征的潜在关联。
对 2007 年 10 月至 2014 年 2 月法国神经肿瘤综合征参考中心诊断的抗 NMDAR 脑炎患者的 94 份脑脊液样本进行了 IgA-NMDAR-Abs 存在情况的回顾性分析。这项观察性研究比较了 39 例同时存在 IgA 和 IgG-NMDAR-Abs 的患者与 55 例仅存在 IgG-NMDAR-Abs 的患者。
在回顾性队列中，41%的 NMDAR-Ab 脑炎患者脑脊液中同时存在 IgG 和 IgA-NMDAR-Abs。大约一半的 IgA-NMDAR-Ab 阳性患者（18/38，49%）确实存在相关肿瘤，主要为卵巢畸胎瘤（17/18，94%），而 IgA-NMDAR-Ab 阴性组患者仅有 5%（3/55）（p<0.001）。在存在卵巢畸胎瘤风险的成年女性人群中，脑脊液 IgA-NMDAR-Ab 阳性检测对卵巢畸胎瘤的诊断具有 85%的敏感性、70%的特异性、57%的阳性预测值和 90%的阴性预测值。与脑脊液 IgA-NMDAR-Ab 阳性无关的其他特定临床特征或临床结局。
这些结果表明，在 IgG-NMDAR-Ab 脑炎患者中，脑脊液 IgA-NMDAR-Abs 可作为存在卵巢畸胎瘤的生物学标志物。
