Background

Paraneoplastic neurological syndromes (PNS) are a group of syndromes that affect the central and peripheral neuromuscular system in association with cancer. Specific antibodies may assist in the diagnosis of PNS. The antibodies tested can be classified into those directed against intracellular neuronal proteins (“well characterized” PNS: Hu, Yo, RI, CV2, amphiphysin, Ma1, Ma2) and those directed against neural surface antigens (autoimmune encephalitis syndromes: NMDA, AMPA, LGI1, CASPR2, GABAR). We aimed to characterize patients with unexplained neuropsychiatric symptoms, in whom positive PNS antibodies were detected in the Sheba medical center, a large referral hospital.

Methods

Clinical and demographic data of patients with positive PNS antibodies were collected during the years 2002–2016. Antibodies were tested by either Line immunoassay or by cell-based indirect immunofluorscent assay.

Results

During the follow up of 14 years, 4010 PNS tests were performed in patients with unexplained neuropsychiatric symptoms. Seventy-two were found to be positive; among them we had full clinical data access to 44. The most frequent antibodies were anti-Hu (31.8%), anti-Yo (18.2%), anti-CV2 (13.6%), and anti-NMDA (9.1%), and the most common cancers were small-cell lung (SCLC) and ovarian cancers. In the well characterized paraneoplastic group, cancer was diagnosed in 55.9% of the patients, and in the autoimmune encephalitis group, 40.0% were diagnosed with cancer. A positive correlation between antibody titer and the presence of cancer was found. Ninety percent of the tests in patients who were found positive were ordered by a neurologist or neuro-oncologist.

Conclusions

The titers of PNS auto-antibodies can predict cancer in patients whom anti-PNS antibodies are tested. In addition, consultation with a specialist should be considered before this test is ordered.

中文翻译：

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肿瘤旁神经系统自身抗体的大屏幕；诊断和预测价值

背景

副肿瘤神经综合症（PNS）是一组与癌症相关的综合症，会影响中枢和周围神经肌肉系统。特异性抗体可能有助于PNS的诊断。所测试的抗体可以分为针对细胞内神经元蛋白的抗体（“特征明确的” PNS：Hu，Yo，RI，CV2，两亲，Ma1，Ma2）和针对神经表面抗原的抗体（自身免疫性脑炎综合征：NMDA，AMPA， LGI1，CASPR2，GABAR）。我们旨在表征患有无法解释的神经精神症状的患者，这些患者在一家大型转诊医院Sheba医疗中心检测到PNS抗体阳性。

方法

在2002–2016年期间收集了PNS抗体阳性患者的临床和人口统计学数据。通过线免疫测定或通过基于细胞的间接免疫荧光测定来测试抗体。

结果

在14年的随访期间，对无法解释的神经精神症状的患者进行了4010次PNS测试。发现有72人是阳性。其中我们拥有44种完整的临床数据。最常见的抗体是抗Hu（31.8％），抗Yo（18.2％），抗CV2（13.6％）和抗NMDA（9.1％），以及最常见的癌症是小细胞肺癌（SCLC）和卵巢癌。在特征明确的副肿瘤治疗组中，有55.9％的患者被诊断出患有癌症，而在自身免疫性脑炎组中，有40.0％的患者被诊断出患有癌症。发现抗体效价与癌症的存在呈正相关。被发现为阳性的患者中有90％的测试是由神经科医生或神经肿瘤学家下令进行的。

结论

PNS自身抗体的效价可以预测接受抗PNS抗体检测的患者的癌症。此外，在订购此测试之前，应考虑与专家协商。