From the French Reference Center on Paraneoplastic Neurological Syndrome (A.V, V.D., F.D., G.P., G.C., V.R., J.-C.A., J.-Y.D., J.H.), Hospices Civils de Lyon, Hôpital Neurologique, Neuro-Oncology, Bron; Lyon Neuroscience Research Center (A.V., V.D., F.D., J.-C.A., J.H.), INSERM U1028/CNRS UMR 5292, Lyon; Université de Lyon-Université Claude Bernard Lyon 1 (A.V., V.D., F.D., J.H.), Lyon; Service de Neurologie (J.-C.A.), CHU de Saint-Etienne, Saint-Etienne; and Service de Neurologie Mazarin (J.-Y.D.), Groupe Hospitalier Pitié-Salpêtrière, APHP, Université Pierre et Marie Curie-Paris 6, Centre de Recherche de l'Institut du Cerveau et de la Moelle épinière, UMR S975, CNRS, UMR 7225, Paris, France.
Neurology. 2014 Feb 18;82(7):556-63. doi: 10.1212/WNL.0000000000000126. Epub 2014 Jan 17.
The aim of this study was to describe the clinical features and specificities of adult male patients with NMDA receptor antibodies (NMDAr-Abs) encephalitis.
Observational study of 13 adult male patients who were diagnosed with NMDAr-Abs encephalitis at the French Paraneoplastic Neurological Syndrome Reference Center.
Adult male patients frequently presented initially with a seizure (8/13, 61.5%). Seizures were partial in 5/8 patients and were followed only a few days later (median 12 days; range 2-17 days) by psychiatric or cognitive symptoms. Conversely, adult female patients rarely presented with a seizure initially (8/58, 14%, p < 0.001), and most of their seizures were generalized and were rapidly followed (median 2 days; range 1-7 days) by behavioral and psychiatric features. Additionally, in male patients the disease was rarely associated with a tumor (1/13 or 8%, a perineal schwannoma); in contrast, 41% of female patients had an associated tumor (95% of which were ovarian teratomas; p = 0.02 male vs female association with tumor). The incidences of abnormalities in ancillary tests, treatment modalities, clinical evolution, and outcome were equal for both subgroups.
Adult male patients who have partial seizures, normal MRI results, and no clear etiology should be tested for NMDAr-Abs to avoid any delays in treatment initiation. Adult female patients who had a seizure as the first symptom are infrequent when NMDAr-Abs encephalitis is diagnosed; additionally, their clinical pattern is different from male patients, with more generalized seizures and rapid development of behavioral and psychiatric symptoms. The differences in hormonal influence could contribute to this difference in clinical pattern.
本研究旨在描述 NMDA 受体抗体（NMDAr-Abs）脑炎成年男性患者的临床特征和特点。
对法国副肿瘤神经综合征参考中心诊断为 NMDAr-Abs 脑炎的 13 名成年男性患者进行观察性研究。
成年男性患者常以癫痫发作（8/13，61.5%）为首发症状。8/5 例癫痫发作为部分性，随后几天（中位数 12 天；范围 2-17 天）出现精神或认知症状。相比之下，成年女性患者最初很少以癫痫发作为首发症状（8/58，14%，p < 0.001），且大多数癫痫发作为全身性，随后迅速出现（中位数 2 天；范围 1-7 天）行为和精神症状。此外，男性患者的疾病很少与肿瘤相关（1/13 或 8%，为会阴神经鞘瘤）；相比之下，41%的女性患者有相关肿瘤（95%为卵巢畸胎瘤；p = 0.02 男性与女性肿瘤相关）。辅助检查、治疗方式、临床转归和预后的发生率在两个亚组之间相等。
出现部分性癫痫发作、MRI 结果正常且无明确病因的成年男性患者应进行 NMDAr-Abs 检测，以避免治疗延误。诊断为 NMDAr-Abs 脑炎的成年女性患者中，以癫痫发作为首发症状的情况较为罕见；此外，她们的临床模式与男性患者不同，表现为全身性癫痫发作，且行为和精神症状迅速发展。激素影响的差异可能导致这种临床模式的差异。
