Alamowitch S, Graus F, Uchuya M, Reñé R, Bescansa E, Delattre J Y
Department of Neurology, Hôpital de la Salpêtrière, Paris, France.
Brain. 1997 Jun;120 ( Pt 6):923-8. doi: 10.1093/brain/120.6.923.
Paraneoplastic limbic encephalitis (LE) is considered a particular manifestation of paraneoplastic encephalomyelitis (PEM), a remote effect of cancer almost always associated with anti-neuronal antibodies (anti-Hu; also called ANNA 1) and small cell lung carcinoma (SCLC). In order to define the frequency of anti-Hu antibodies in LE with SCLC and to analyse possible clinical differences between patients with and without anti-Hu antibodies, the charts of 16 patients with LE and SCLC were reviewed. Eight patients (50%) had anti-Hu antibodies (anti-Hu+) whereas eight patients (50%) had no detectable anti-neuronal antibodies (anti-Hu-). The clinical and laboratory features of LE and time to diagnosis of SCLC were similar in the anti-Hu+ and anti-Hu- groups. Involvement of other areas of the nervous system compatible with the diagnosis of PEM was observed in seven (87.5%) patients of the anti-Hu+ group but in only one (12.5%) of the anti-Hu- group (P = 0.012). Five patients, including four of the anti-Hu- group, had a partial improvement of the LE after treatment of the SCLC. Another anti-Hu- patient improved spontaneously. Six patients of the anti-Hu+ group died from the neurological disorder, whereas in the anti-Hu- group the cause of death was progression of the SCLC in the three patients who died. The results of this study indicate that the absence of anti-Hu antibodies does not rule out the presence of an underlying SCLC in patients with a clinical diagnosis of LE. Patients with LE and SCLC who are without anti-Hu antibodies are less likely to develop PEM and seem to improve more often after treatment of the cancer than those who present anti-Hu antibodies.
副肿瘤性边缘叶脑炎（LE）被认为是副肿瘤性脑脊髓炎（PEM）的一种特殊表现形式，PEM是癌症的一种远隔效应，几乎总是与抗神经元抗体（抗Hu；也称为ANNA 1）和小细胞肺癌（SCLC）相关。为了确定SCLC合并LE患者中抗Hu抗体的发生率，并分析抗Hu抗体阳性和阴性患者之间可能存在的临床差异，我们回顾了16例SCLC合并LE患者的病历。8例患者（50%）有抗Hu抗体（抗Hu阳性），而8例患者（50%）未检测到抗神经元抗体（抗Hu阴性）。抗Hu阳性和阴性组中LE的临床和实验室特征以及SCLC的诊断时间相似。抗Hu阳性组7例（87.5%）患者出现与PEM诊断相符的其他神经系统区域受累，而抗Hu阴性组仅1例（12.5%）出现（P = 0.012）。5例患者，包括抗Hu阴性组的4例，在SCLC治疗后LE有部分改善。另1例抗Hu阴性患者自发改善。抗Hu阳性组6例患者死于神经系统疾病，而抗Hu阴性组3例死亡患者的死因是SCLC进展。本研究结果表明，临床诊断为LE的患者中，抗Hu抗体阴性并不能排除潜在SCLC的存在。与抗Hu抗体阳性的患者相比，SCLC合并LE且抗Hu抗体阴性的患者发生PEM的可能性较小，且在癌症治疗后似乎更常改善。
