Clinical specificities of adult male patients with NMDA receptor antibodies encephalitis

被引:207
作者
Viaccoz, Aurelien [1 ,2 ,3 ]
Desestret, Virginie [1 ,2 ,3 ]
Ducray, Francois [1 ,2 ,3 ]
Picard, Geraldine [1 ]
Cavillon, Gaelle [1 ]
Rogemond, Veronique [1 ]
Antoine, Jean-Christophe [1 ,2 ,4 ]
Delattre, Jean-Yves [1 ,5 ]
Honnorat, Jerome [1 ,2 ,3 ]
机构
[1] Hop Neurol, Hosp Civils Lyon, French Reference Ctr Paraneoplast Neurol Syndrome, Bron, France
[2] CNRS, INSERM, U1028, UMR 5292,Lyon Neurosci Res Ctr, Lyon, France
[3] Univ Lyon 1, F-69365 Lyon, France
[4] CHU St Etienne, Serv Neurol, St Etienne, France
[5] Univ Paris 06, Grp Hosp Pitie Salpetriere, AP HP,UMR S975,CNRS,UMR 7225, Serv Neurol Mazarin,Ctr Rech,Inst Cerveau & Moell, Paris, France
关键词
LONG-TERM; ELECTROCONVULSIVE-THERAPY; LIMBIC ENCEPHALITIS; ESTROGEN; SEIZURES; TERATOMA; EPILEPSY; SERIES;
D O I
10.1212/WNL.0000000000000126
中图分类号
R74 [神经病学与精神病学];
学科分类号
摘要
Objective:The aim of this study was to describe the clinical features and specificities of adult male patients with NMDA receptor antibodies (NMDAr-Abs) encephalitis.Methods:Observational study of 13 adult male patients who were diagnosed with NMDAr-Abs encephalitis at the French Paraneoplastic Neurological Syndrome Reference Center.Results: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.Conclusion: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.
引用
收藏
页码:556 / 563
页数:8
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