Neuronal surface antigen antibodies in limbic encephalitis: clinical-immunologic associations.
Neuronal surface antigen antibodies in limbic encephalitis: clinical-immunologic associations.
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DOI：
10.1212/01.wnl.0000325917.48466.55
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发表时间：
2008-09-16
期刊：
影响因子：
9.9
通讯作者：
Dalmau J
中科院分区：
文献类型：
--
作者：
Graus F;Saiz A;Lai M;Bruna J;López F;Sabater L;Blanco Y;Rey MJ;Ribalta T;Dalmau J
目的探讨边缘脑炎(LE)患者抗神经元表面抗原(NSA-ab)抗体的出现频率和类型。分析45例副肿瘤(23例)或特发性(22例)LE患者的临床特征、神经病理学表现，并用免疫组织化学方法检测大鼠组织和神经元培养中NSA-ab的表达。29例患者中检出NsA-ab(%；副肿瘤12例，特发性17例)。13例患者有电压门控性钾通道(VGKC)-ab，11例新的NSA(NNSA)-ab，5例NMDA受体(NMDAR)-ab。NNSA-ab不识别共同的抗原，在副肿瘤中的出现率高于特发性LE(39%对9%；p=0.03)。与VGKC-ab或NMDAR-ab相比，NNSA与神经元内抗体的相关性更高(11%对73%；p=0.001)。12例副肿瘤性LE和NSA-ab患者(NNSA-ab 9例，VGKC-ab 2例，NMDAR-ab 1例)中，9例(75%)同时出现神经元内抗体。这12名患者在接受免疫治疗后均无好转。其中三人的尸检显示，神经元丢失，小胶质细胞增多，细胞毒性T细胞在海马区和杏仁体中渗透。这些发现与T细胞介导的神经元损伤是一致的。17例特发性LE合并NSA-ab患者中13例(76%)临床症状改善(P=0.04)。在副肿瘤性边缘脑炎(LE)中，抗神经元表面抗原(NNSA-ab)的新型抗体(NNSA-ab)频繁出现，并与抗细胞内抗原的抗体共存，这些病例对免疫治疗难以奏效。在特发性LE中，有NSA-ab抗体的患者改善的可能性明显高于没有抗体的患者。
To report the frequency and type of antibodies against neuronal surface antigens (NSA-ab) in limbic encephalitis (LE). Analysis of clinical features, neuropathologic findings, and detection of NSA-ab using immunochemistry on rat tissue and neuronal cultures in a series of 45 patients with paraneoplastic (23) or idiopathic (22) LE. NSA-ab were identified in 29 patients (64%; 12 paraneoplastic, 17 idiopathic). Thirteen patients had voltage-gated potassium channels (VGKC)-ab, 11 novel NSA (nNSA)-ab, and 5 NMDA receptor (NMDAR)-ab. nNSA-ab did not identify a common antigen and were more frequent in paraneoplastic than idiopathic LE (39% vs 9%; p = 0.03). When compared with VGKC-ab or NMDAR-ab, the nNSA associated more frequently with intraneuronal antibodies (11% vs 73%; p = 0.001). Of 12 patients (9 nNSA-ab, 2 VGKC-ab, 1 NMDAR-ab) with paraneo-plastic LE and NSA-ab, concomitant intraneuronal antibodies occurred in 9 (75%). None of these 12 patients improved with immunotherapy. The autopsy of three of them showed neuronal loss, microgliosis, and cytotoxic T cell infiltrates in the hippocampus and amygdala. These findings were compatible with a T-cell mediated neuronal damage. In contrast, 13 of 17 (76%) patients with idiopathic LE and NSA-ab (8 VGKC-ab, 4 NMDAR-ab, 1 nNSA-ab) and 1 of 5 (20%) without antibodies had clinical improvement (p = 0.04). In paraneoplastic limbic encephalitis (LE), novel antibodies against neuronal surface antigens (nNSA-ab) occur frequently, coexist with antibodies against intracellular antigens, and these cases are refractory to immunotherapy. In idiopathic LE, the likelihood of improvement is significantly higher in patients with NSA-ab than in those without antibodies.
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影响因子：
11
作者：
Bataller, L.;Kleopa, K. A.;Dalmau, J.
通讯作者：
Dalmau, J.
影响因子：
9.9
作者：
Bien, C. G.;Urbach, H.;Elger, C. E.
通讯作者：
Elger, C. E.
影响因子：
9.9
作者：
Saiz, A;Arpa, J;Graus, F
通讯作者：
Graus, F
影响因子：
9.9
作者：
CHALK, CH;MURRAY, NMF;SPIRO, SG
通讯作者：
SPIRO, SG
影响因子：
3.3
作者：
Graus, F;Vincent, A;Dalmau, J
通讯作者：
Dalmau, J