GABAB receptor autoantibody frequency in service serologic evaluation

GABAB receptor autoantibody frequency in service serologic evaluation
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DOI:
10.1212/wnl.0b013e3182a35271
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发表时间:
2013-09-03
期刊:
影响因子:
9.9
通讯作者:
McKeon, Andrew
McKeon, Andrew
中科院分区:
医学1区
文献类型:
--
作者:
Jeffery, Oliver J.;Lennon, Vanda A.;McKeon, Andrew

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目的:小细胞肺癌(SCLC)和边缘脑炎是公认的g-氨基丁酸-B受体(GABA(B)R)自身抗体伴生。我们试图在诊断血清学实验室中确定GABA(B) r -免疫球蛋白G (IgG)的频率和伴随(神经学、肿瘤学和血清学)。方法:用间接免疫荧光法检测3组患者血清和脑脊液中GABA(B)R-IgG的含量,并转染HEK293细胞。第1组包括3,989例在疑似自身免疫性脑病服务评估中检测GABA(B)R-IgG的患者。第2组包括49例未分类的CNS突触IgG检测(先于GABA(B)R自身抗体描述)。第三组包括384例患者,检测到>= 1的sclc预测自身抗体。结果:17例患者(血清14例,脑脊液11例)检测到GABA(B) r特异性IgG。1、2组血清阳性患者均有n型钙通道抗体与GABA(B)R-IgG共存。第1组3989例患者中有7例阳性(0.2%)。所有人都患有边缘脑炎;5人患有SCLC。4例患者接受免疫治疗,神经功能得到改善。2组49例患者中5例阳性(10%)。3例边缘脑炎,1例快速进行性脑脊髓病,1例小脑性共济失调。2例为小细胞肺癌,1例为多发性骨髓瘤。第3组384例患者中,5例阳性(1.3%);滴度低(仅通过转染细胞试验检测)。神经系统表现多样,可归因于共存的t细胞介导的自身免疫(由CRMP-5 IgG [2], ANNA-1[2]和ANNA-3[2]表示),而不是GABA(B)R-IgG。结论:GABA(B)R自身抗体是一种罕见但可治疗的副肿瘤神经系统疾病的标志物,通常发生在边缘脑炎和SCLC。
Objective: Small-cell lung carcinoma (SCLC) and limbic encephalitis are recognized g-aminobutyric acid-B receptor (GABA(B)R) autoantibody accompaniments. We sought to determine in a diagnostic serology laboratory the frequency and accompaniments (neurologic, oncologic, and serologic) of GABA(B)R-immunoglobulin G (IgG).Methods: We tested stored serum and CSF specimens from 3 patient groups for GABA(B)R-IgG by indirect immunofluorescence on mouse brain tissue and transfected HEK293 cells. Group 1 included 3,989 patients tested for GABA(B)R-IgG in service evaluation for suspected autoimmune encephalopathy. Group 2 included 49 patients with an unclassified CNS synaptic IgG detected (antedating descriptions of GABA(B)R autoantibody). Group 3 included 384 patients in whom >= 1 SCLC-predictive autoantibodies had been detected.Results: GABA(B)R-specific IgG was detected in 17 patients (serum, 14; CSF, 11). N-type calcium channel antibody coexisted with GABA(B)R-IgG in all seropositive patients of groups 1 and 2. In group 1, 7 of 3,989 patients were positive (0.2%). All had limbic encephalitis; 5 had SCLC. Four patients received immunotherapy and improved neurologically. In group 2, 5 of 49 patients were positive (10%). Three had limbic encephalitis, 1 had rapidly progressive encephalomyelopathy, and 1 had cerebellar ataxia. Two patients had SCLC and 1 had multiple myeloma. In group 3, 5 of 384 patients were positive (1.3%); titers were low (detected only by transfected cell assay). The neurologic presentations were diverse and attributable to coexisting T-cell-mediated autoimmunity (indicated by CRMP-5 IgG [2], ANNA-1 [2], and ANNA-3 [2]), rather than to GABA(B)R-IgG.Conclusion: GABA(B)R autoantibody is a marker of an uncommon but treatable paraneoplastic neurologic disorder, usually occurring in the setting of limbic encephalitis and SCLC.