French Reference Center for Paraneoplastic Neurological Syndrome, Hospices Civils de Lyon, Hôpital Neurologique, 69677, Bron, France.
Institut NeuroMyoGene INSERM U1217/CNRS UMR 5310, Université de Lyon, Université Claude Bernard Lyon 1, 69372, Lyon, France.
J Neurol. 2018 Oct;265(10):2190-2200. doi: 10.1007/s00415-018-8970-0. Epub 2018 Jul 12.
The aim of this study was to describe specificities of patients with NMDA receptor antibody (NMDAR-Ab) encephalitis associated with a malignant tumor.
Retrospective observational study of 252 patients with NMDAR-Ab encephalitis of the French Paraneoplastic Neurological Syndrome Reference Center. Patients were classified in three groups: (1) non-malignant ovarian teratomas, (2) malignant ovarian teratomas (immature), and (3) other malignant tumors.
Sixty patients (23.8%) had an associated tumor and 15 (6%) were malignant. No particular neurological symptom was observed in these patients. Ovarian teratomas were the most frequent (51 cases) with 6 of them immature (11.8% of teratomas). Nine patients (3.6%) developed other malignant tumors (3 small cell lung carcinomas, 1 uterine adenocarcinoma, 1 prostate adenocarcinoma, 1 Hodgkin lymphoma, 1 pineal dysgerminoma, 1 neuroblastoma and 1 pancreatic neuroendocrine tumor). Among patients with a cancer other than teratoma, 6/9 were elderly patients (median age 65 years, representing 30% of elderly patients with such encephalitis) compared to a median age of 26 years in adult patients included herein. The clinical course was similar in the three groups, other than a higher death rate among patients with malignant tumors (86 versus 2%; p < 0.001) mainly due to tumor progression (5/7 deaths).
Immature ovarian teratomas represent 11.8% of all teratomas in patients with NDMAR-Ab encephalitis. The other malignant tumors are mainly observed in elderly patients. The presence of a malignant tumor does not impact the neurological presentation but is directly associated with a higher risk of death.
本研究旨在描述与 NMDA 受体抗体（NMDAR-Ab）脑炎相关的恶性肿瘤患者的特点。
回顾性观察法国副肿瘤神经综合征参考中心的 252 例 NMDAR-Ab 脑炎患者。患者分为三组：（1）非恶性卵巢畸胎瘤，（2）恶性卵巢畸胎瘤（不成熟），和（3）其他恶性肿瘤。
60 例（23.8%）患者合并肿瘤，15 例（6%）为恶性肿瘤。这些患者无特殊神经症状。卵巢畸胎瘤最常见（51 例），其中 6 例为不成熟型（占畸胎瘤的 11.8%）。9 例（3.6%）患者发生其他恶性肿瘤（3 例小细胞肺癌、1 例子宫腺癌、1 例前列腺腺癌、1 例霍奇金淋巴瘤、1 例松果体生殖细胞瘤、1 例神经母细胞瘤和 1 例胰腺神经内分泌肿瘤）。在非畸胎瘤癌症患者中，6/9 例为老年患者（中位年龄 65 岁，占此类脑炎老年患者的 30%），而纳入本研究的成年患者的中位年龄为 26 岁。三组患者的临床病程相似，但恶性肿瘤患者的死亡率较高（86%比 2%；p<0.001），主要原因是肿瘤进展（5/7 例死亡）。
不成熟卵巢畸胎瘤占 NDMAR-Ab 脑炎患者所有畸胎瘤的 11.8%。其他恶性肿瘤主要见于老年患者。恶性肿瘤的存在并不影响神经系统表现，但与更高的死亡风险直接相关。
