Berger Benjamin, Hottenrott Tilman, Rauer Sebastian, Stich Oliver
Department of Neurology and Neurophysiology, Medical Center-University of Freiburg, Faculty of Medicine, Breisacher Strasse 64, D-79106, Freiburg, Germany.
BMC Neurol. 2017 Jan 10;17(1):5. doi: 10.1186/s12883-016-0779-9.
Some so-called "non-classical" paraneoplastic neurological syndromes (PNS), namely optic neuritis and myelitis, clinically overlap with neuromyelitis optica spectrum disorders (NMOSD), and conversely, in cancer-associated NMOSD, a paraneoplastic etiology has been suggested in rare cases. Therefore, we retrospectively investigated the prevalence of onconeural antibodies, which are highly predictive for a paraneoplastic etiology, and the prevalence of malignancies in NMOSD patients.
We retrospectively screened 23 consecutive patients from our clinic with NMOSD (13 were anti-aquaporin-4 [AQP4] antibody positive, 10 were AQP4 negative) for onconeural antibodies using an immunoblot.
All patients were negative for a broad spectrum of antibodies targeting intracellular onconeural antigens (Hu, Yo, Ri, CV2/CRMP5, Ma1, Ma2, Zic4, SOX1, Tr, and amphiphysin). Notably, only two patients had a malignancy. However, neoplastic entities (astrocytic brain tumor and acute myeloid leukemia) were not typical for PNS.
Our data suggest that there is no need to routinely screen anti-AQP4 antibody positive NMOSD patients with a typical presentation for onconeural antibodies. Furthermore, absence of these antibodies in NMOSD, which is typically non-paraneoplastic, confirms their high specificity for PNS.
一些所谓的“非经典”副肿瘤性神经系统综合征（PNS），即视神经炎和脊髓炎，在临床上与视神经脊髓炎谱系障碍（NMOSD）重叠，反之，在癌症相关的NMOSD中，罕见情况下提示有副肿瘤病因。因此，我们回顾性研究了对副肿瘤病因具有高度预测性的肿瘤神经抗体的患病率以及NMOSD患者中恶性肿瘤的患病率。
我们使用免疫印迹法对来自我们诊所的23例连续的NMOSD患者（13例抗水通道蛋白4 [AQP4]抗体阳性，10例AQP4阴性）进行肿瘤神经抗体的回顾性筛查。
所有患者针对细胞内肿瘤神经抗原（Hu、Yo、Ri、CV2/CRMP5、Ma1、Ma2、Zic4、SOX1、Tr和 amphiphysin）的广谱抗体均为阴性。值得注意的是，只有两名患者患有恶性肿瘤。然而，肿瘤实体（星形细胞瘤和急性髓系白血病）并非PNS的典型表现。
我们的数据表明，对于具有典型表现的抗AQP4抗体阳性的NMOSD患者，无需常规筛查肿瘤神经抗体。此外，在通常非副肿瘤性的NMOSD中缺乏这些抗体，证实了它们对PNS具有高度特异性。
