Berger Benjamin, Bischler Patricia, Dersch Rick, Hottenrott Tilman, Rauer Sebastian, Stich Oliver
University Hospital Freiburg, Department of Neurology and Neurophysiology, Breisacher Strasse 64, D-79106 Freiburg, Germany.
University Hospital Freiburg, Department of Neurology and Neurophysiology, Breisacher Strasse 64, D-79106 Freiburg, Germany.
J Neurol Sci. 2015 May 15;352(1-2):58-61. doi: 10.1016/j.jns.2015.03.027. Epub 2015 Mar 23.
Paraneoplastic neurological syndromes (PNSs) are rare disorders in association with cancer and sub-divided into "classical" and "non-classical" syndromes according to a 2004 consensus paper proposed by a panel of PNS experts. "Classical" PNSs are regarded to account for the vast majority of cases. However, systematic reports on clinical PNS manifestations are rare. Therefore, we analyzed the spectrum of PNS in our clinic.
We retrospectively investigated medical records from consecutive patients diagnosed with definite PNS and serological evidence of well-characterized onconeural antibodies (anti-Hu, Yo, Ri, CV2/CRMP5, Ma1, Ma2, and amphiphysin) analyzed between 1991 and 2014 in our clinic.
Of the 50 patients identified with onconeural antibody-positive PNS, 28 patients (56.0%) had "classical" PNS, and 22 (44.0%) "non-classical" PNS. Subacute cerebellar degeneration was the most frequent "classical" syndrome, brainstem encephalitis and subacute sensorimotor neuronopathy the most frequent "non-classical" syndromes. Anti-Hu antibodies were most frequent in both groups. 86.1% of patients developed neurological symptoms before the cancer was known. No differences between "classical" and "non-classical" syndromes were detected with respect to age, tumor entities and median time to diagnosis. However, whereas most patients with "classical" syndromes were females, there was no gender predominance in patients with "non-classical" PNS and the latter had significantly more frequent peripheral neurological syndromes.
The so-called "non-classical" PNSs in association with well-characterized onconeural antibodies were more common in our patient population than expected. Therefore, in neurological disorders of unclear etiology with a subacute onset and atypical presentation further diagnostic work-up including investigation of onconeural antibodies is necessary.
副肿瘤性神经系统综合征（PNSs）是与癌症相关的罕见疾病，根据PNS专家小组在2004年提出的一份共识文件，可分为“经典”和“非经典”综合征。“经典”PNSs被认为占绝大多数病例。然而，关于临床PNS表现的系统性报告很少。因此，我们分析了我们诊所中PNS的谱系。
我们回顾性研究了1991年至2014年间在我们诊所诊断为明确PNS且有特征明确的肿瘤神经抗体（抗Hu、Yo、Ri、CV2/CRMP5、Ma1、Ma2和抗 amphiphysin）血清学证据的连续患者的病历。
在50例确诊为肿瘤神经抗体阳性的PNS患者中，28例（56.0%）患有“经典”PNS，22例（44.0%）患有“非经典”PNS。亚急性小脑变性是最常见的“经典”综合征，脑干脑炎和亚急性感觉运动神经元病是最常见的“非经典”综合征。抗Hu抗体在两组中最为常见。86.1%的患者在癌症确诊之前出现神经症状。在年龄、肿瘤类型和诊断中位时间方面，“经典”和“非经典”综合征之间未检测到差异。然而，虽然大多数“经典”综合征患者为女性，但“非经典”PNS患者中没有性别优势，且后者外周神经综合征更为常见。
在我们的患者群体中，与特征明确的肿瘤神经抗体相关的所谓“非经典”PNSs比预期更为常见。因此，对于病因不明、亚急性起病且表现不典型的神经系统疾病，有必要进行进一步的诊断检查，包括检测肿瘤神经抗体。
