Rosenfeld Myrna R, Titulaer Maarten J, Dalmau Josep
IDIBAPS and Service of Neurology (MRR, MJT, JD), Hospital Clinic, University of Barcelona, Barcelona, Spain; Department of Neurology (MRR, MJT, JD), University of Pennsylvania, Philadelphia; and Institució Catalana de Recerca i Estudis Avançats (ICREA) (JD), Barcelona, Spain.
Neurol Clin Pract. 2012 Sep;2(3):215-223. doi: 10.1212/CPJ.0b013e31826af23e.
We review novel findings in paraneoplastic syndromes including the Lambert-Eaton myasthenic syndrome, and then focus on the novel disorders associated with antibodies against cell surface antigens, discussing the importance and caveats of antibody testing, and providing an algorithm for interpretation of results. In anti-NMDAR encephalitis 2 novel findings include the recognition of a characteristic EEG pattern ("extreme delta brush") in 30% of patients and the demonstration of a fronto-temporo-occipital gradient of glucose metabolism that correlates with disease activity. In limbic encephalitis, antibodies to GABA(B) receptor are the most frequently detected in patients with small-cell lung cancer who are anti-Hu negative, and antibodies to mGluR5 distinctively associate with Hodgkin lymphoma (Ophelia syndrome). We also address the syndromes associated with "VGKC-complex antibodies," a problematic term that groups well-characterized immune-mediated disorders (LGI1, Caspr2) with others that lack syndrome specificity, are less responsive to treatment, and for which the target antigens are unknown.
我们回顾了副肿瘤综合征的新发现，包括兰伯特-伊顿肌无力综合征，然后重点关注与抗细胞表面抗原抗体相关的新疾病，讨论抗体检测的重要性和注意事项，并提供结果解读算法。在抗NMDAR脑炎中，两项新发现包括：30%的患者可识别出特征性脑电图模式（“极端δ刷”），以及葡萄糖代谢的额颞枕梯度与疾病活动相关。在边缘叶脑炎中，抗GABA(B)受体抗体在抗Hu阴性的小细胞肺癌患者中最常被检测到，而抗mGluR5抗体则与霍奇金淋巴瘤（奥菲莉亚综合征）显著相关。我们还讨论了与“VGKC复合物抗体”相关的综合征，这是一个有问题的术语，它将特征明确的免疫介导疾病（LGI1、Caspr2）与其他缺乏综合征特异性、对治疗反应较差且靶抗原未知的疾病归为一类。
