Berger Benjamin, Dersch Rick, Ruthardt Elisabeth, Rasiah Christiane, Rauer Sebastian, Stich Oliver
Department of Neurology and Neurophysiology, Medical Center - University of Freiburg, Faculty of Medicine, Breisacher Strasse 64, D-79106 Freiburg, Germany.
Department of Neurology and Neurophysiology, Medical Center - University of Freiburg, Faculty of Medicine, Breisacher Strasse 64, D-79106 Freiburg, Germany.
J Neurol Sci. 2016 Oct 15;369:342-346. doi: 10.1016/j.jns.2016.09.002. Epub 2016 Sep 4.
Anti-SOX1 antibodies are associated with small cell lung cancer (SCLC) and predict a paraneoplastic etiology in Lambert-Eaton myasthenic syndrome (LEMS). In 2010, a study described these antibodies in a small cohort of putative non-paraneoplastic, immune-mediated neuropathies. In this respect, we investigated the seroprevalence and specificity of anti-SOX1 antibodies in a large cohort of neurological disorders.
Overall, serum samples of 1493 consecutive patients were screened for anti-SOX1 reactivity by an ELISA: 471 with well-defined neurological disorders (multiple sclerosis, motor neuron disease, Guillain-Barré syndrome, and chronic inflammatory demyelinating polyneuropathy), 185 with polyneuropathy (PNP) of unknown origin, and 837 with neurological syndromes of suspicious paraneoplastic etiology. These were compared to eight positive controls with definite paraneoplastic neurological syndromes (PNS) and 92 healthy individuals. We also collected demographic and clinical data, including well-characterized onconeural antibodies in anti-SOX1-positive patients.
Fifteen patients (1.0%) showed anti-SOX1 reactivity: two with multiple sclerosis, two with PNP of unknown origin, and 11 suspicious PNS cases. Remarkably, 9/15 anti-SOX1-positive patients had a PNP. However, antibody concentrations were significantly lower compared to positive controls, and none additionally harbored well-characterized onconeural antibodies. During a follow-up of at least four years, only five patients had cancer: one thyroid, one Hodgkin lymphoma, two breast, and one patient had multiple malignancies - prostate, penis, cecum, liver, and non-small cell lung cancer. However, none had SCLC, typically associated with SOX1 antibodies.
The seroprevalence of anti-SOX1 antibodies in patients with various neurological disorders is low. These patients predominantly have PNPs, which might represent a group of immune-mediated diseases.
抗SOX1抗体与小细胞肺癌（SCLC）相关，并可预测兰伯特-伊顿肌无力综合征（LEMS）的副肿瘤病因。2010年，一项研究在一小群疑似非副肿瘤性免疫介导性神经病患者中描述了这些抗体。在这方面，我们调查了一大群神经系统疾病患者中抗SOX1抗体的血清阳性率和特异性。
总体而言，通过酶联免疫吸附测定（ELISA）对1493例连续患者的血清样本进行抗SOX1反应性筛查：471例患有明确的神经系统疾病（多发性硬化症、运动神经元病、格林-巴利综合征和慢性炎症性脱髓鞘性多发性神经病），185例患有不明原因的多发性神经病（PNP），837例患有疑似副肿瘤病因的神经系统综合征。将这些患者与8例确诊为副肿瘤性神经系统综合征（PNS）的阳性对照和92名健康个体进行比较。我们还收集了人口统计学和临床数据，包括抗SOX1阳性患者中特征明确的肿瘤神经抗体。
15例患者（1.0%）显示抗SOX1反应性：2例患有多发性硬化症，2例患有不明原因的PNP，11例为疑似PNS病例。值得注意的是，15例抗SOX1阳性患者中有9例患有PNP。然而，与阳性对照相比，抗体浓度显著较低，且无一例额外携带特征明确的肿瘤神经抗体。在至少四年的随访期间，只有5例患者患有癌症：1例甲状腺癌、1例霍奇金淋巴瘤、2例乳腺癌，1例患者患有多种恶性肿瘤——前列腺癌、阴茎癌、盲肠癌、肝癌和非小细胞肺癌。然而，无一例患有通常与SOX1抗体相关的SCLC。
各种神经系统疾病患者中抗SOX1抗体的血清阳性率较低。这些患者主要患有PNP，这可能代表一组免疫介导性疾病。
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