Clinical Features and Diagnostic Usefulness of Antibodies to Clustered Acetylcholine Receptors in the Diagnosis of Seronegative Myasthenia Gravis.
Clinical Features and Diagnostic Usefulness of Antibodies to Clustered Acetylcholine Receptors in the Diagnosis of Seronegative Myasthenia Gravis.
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DOI：
10.1001/jamaneurol.2015.0203
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发表时间：
2015-06
期刊：
影响因子：
29
通讯作者：
Palace J
中科院分区：
文献类型：
--
作者：
Rodríguez Cruz PM;Al-Hajjar M;Huda S;Jacobson L;Woodhall M;Jayawant S;Buckley C;Hilton-Jones D;Beeson D;Vincent A;Leite MI;Palace J
基于细胞的检测（cba）可提高重症肌无力（MG）患者乙酰胆碱受体（AChR）抗体的检测。在这里，我们询问这些检测是否能够帮助确定在常规临床实践中研究的患者的诊断。目的：确定cba在MG诊断中的诊断价值，并比较仅具有聚集性achr抗体的患者与血清阴性MG （SNMG）患者的临床特征。所有在2009年11月1日至2013年11月30日期间在英国牛津约翰拉德克利夫医院临床神经内科就诊的临床怀疑MG的患者。观察两组患者的血清抗体及临床特征。采用放射免疫沉淀法（RIPA）和CBA检测138例患者的标准AChR抗体和聚集性AChR抗体。所有可获得的SNMG患者样本回顾性检测脂蛋白受体相关蛋白4 （LRP4）抗体。人口统计学、临床、神经生理学和实验室数据。总共有138例患者接受了聚集性achr抗体检测，42例最终诊断为MG。聚集性AChR CBA在38.1%（16 / 42）的ripa阴性MG患者中检测到抗体，特异性为100%。所有SNMG患者的LRP4抗体检测（26例中有21例）均为CBA阴性。与SNMG患者相比，仅针对聚集性achr抗体的患者多发青春期前发病（62.5%[中位年龄，6岁；年龄范围，1-52岁]vs 11.5%[中位年龄，38岁；年龄范围，2-72岁]，P≤0.05），眼部MG患病率高（62.5%对42.3%），疾病严重程度较轻，球受累较少（25.0%对46.2%），无呼吸道症状（0%对23.1%）。治疗反应和预后良好，胸腺切除术的需求减少（6.3%对19.2%），缓解的患者比例很高（50.0%对8.3%,P≤0.05）。这些观察结果也适用于在大系列中看到的经典AChR MG表型。细胞为基础的分析是一个有用的程序，在常规诊断ripa阴性MG，特别是在儿童。只有聚集性achr抗体的患者似乎比其他MG患者更年轻，病情更轻。这些观察结果将对计划治疗产生影响。
Cell-based assays (CBAs) were shown to improve detection of acetylcholine receptor (AChR) antibodies in patients with myasthenia gravis (MG). Herein, we asked whether these assays were able to help determine the diagnosis in patients studied in routine clinical practice. To determine the diagnostic usefulness of CBAs in the diagnosis of MG and to compare the clinical features of patients with antibodies only to clustered AChRs with those of patients with seronegative MG (SNMG). All patients with clinical suspicion of MG who were seen within the Division of Clinical Neurology at the John Radcliffe Hospital in Oxford, England, between November 1, 2009, and November 30, 2013. Their serum antibodies and clinical features were studied. Radioimmunoprecipitation assay (RIPA) and CBA were used to test for standard AChR antibodies and antibodies to clustered AChRs in 138 patients. All available samples from patients with SNMG were retrospectively tested for lipoprotein receptor–related protein 4 (LRP4) antibodies. Demographic, clinical, neurophysiological, and laboratory data. In total, 138 patients were tested for antibodies to clustered AChRs, and 42 had a final diagnosis of MG. The clustered AChR CBA detected antibodies in 38.1% (16 of 42) of RIPA-negative patients with MG with 100% specificity. All patients with SNMG who were tested for LRP4 antibodies (21 of 26) were negative by CBA. Compared with patients with SNMG, patients with antibodies only to clustered AChRs had frequent prepubertal onset (62.5% [median age, 6 years; age range, 1-52 years] vs 11.5% [median age, 38 years; age range, 2-72 years], P ≤ .05), high prevalence of ocular MG (62.5% vs 42.3%), milder disease severity with less bulbar involvement (25.0% vs 46.2%), and absence of respiratory symptoms (0% vs 23.1%). Response to treatment and prognosis was good, with a reduced need for thymectomy (6.3% vs 19.2%) and a high proportion of patients going into remission (50.0% vs 8.3%, P ≤ .05). These observations also apply to the classic AChR MG phenotype seen in large series. Cell-based assay is a useful procedure in the routine diagnosis of RIPA-negative MG, particularly in children. Patients with antibodies only to clustered AChRs appear to be younger and have milder disease than other patients with MG. These observations will have implications in planning treatment.
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影响因子：
9.9
作者：
CHIU, HC;VINCENT, A;HUNG, TP
通讯作者：
HUNG, TP
影响因子：
5.1
作者：
Kostera-Pruszczyk, A.;Kaminska, A.;Kwiecinski, H.
通讯作者：
Kwiecinski, H.
影响因子：
82.9
作者：
Hoch, W;McConville, J;Vincent, A
通讯作者：
Vincent, A
影响因子：
3.5
作者：
Ashraf, V. V.;Taly, A. B.;Rao, S.
通讯作者：
Rao, S.
影响因子：
6
作者：
Pevzner, Alexandra;Schoser, Benedikt;Kroeger, Stephan
通讯作者：
Kroeger, Stephan