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迟发性抗 NMDA 受体脑炎。

Late-onset anti-NMDA receptor encephalitis.

机构信息

From the Department of Neurology (M.J.T., I.G., M.R.R., F.G., J.D.), Hospital Clinic, Universitat de Barcelona/Institut d'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Spain; Department of Neurology and Neurosciences (M.J.T., L.M., M.R.R., R.B.-G., J.D.), Perelman School of Medicine, Hospital of the University of Pennsylvania, Philadelphia; Department of Neurology (T.I.), Kitasato University School of Medicine, Sagamihara; Department of Neurology (I.K.), Brain Research Institute, Niigata University, Japan; Department of Neurology (L.B.), Hospital Universitario La Fe, Valencia; Biostatistics and Data Management Platform (A.T.), IDIBAPS, Hospital Clinic; and Institució Catalana de Recerca i Estudis Avançats (ICREA) (J.D.), Barcelona, Spain.

出版信息

Neurology. 2013 Sep 17;81(12):1058-63. doi: 10.1212/WNL.0b013e3182a4a49c. Epub 2013 Aug 14.

Abstract

OBJECTIVE

To describe the clinical features and outcome of anti-NMDA receptor (NMDAR) encephalitis in patients ≥45 years old.

METHOD

Observational cohort study.

RESULTS

In a cohort of 661 patients with anti-NMDAR encephalitis, we identified 31 patients ≥45 years old. Compared with younger adults (18-44 years), older patients were more often male (45% vs. 12%, p < 0.0001), had lower frequency of tumors (23% vs. 51%, p = 0.002; rarely teratomas), had longer median time to diagnosis (8 vs 4 weeks, p = 0.009) and treatment (7 vs. 4 weeks, p = 0.039), and had less favorable outcome (modified Rankin Scale score 0-2 at 2 years, 60% vs. 80%, p < 0.026). In multivariable analysis, younger age (odds ratio [OR] 0.15, confidence interval [CI] 0.05-0.39, p = 0.0001), early treatment (OR 0.60, CI 0.47-0.78, p < 0.0001), no need for intensive care (OR 0.09, CI 0.04-0.22, p < 0.0001), and longer follow-up (p < 0.0001) were associated with good outcome. Rituximab and cyclophosphamide were effective when first-line immunotherapies failed (OR 2.93, CI 1.10-7.76, p = 0.031). Overall, 60% of patients older than 45 years had full or substantial recovery at 24 months follow-up.

CONCLUSIONS

Anti-NMDAR encephalitis is less severe in patients ≥45 years old than in young adults, but the outcome is poorer in older patients. In this age group, delays in diagnosis and treatment are more frequent than in younger patients. The frequency of underlying tumors is low, but if present they are usually carcinomas instead of teratomas in younger patients. Early and aggressive immunotherapy will likely improve the clinical outcome.

摘要

目的

描述年龄≥45 岁的抗 N-甲基-D-天冬氨酸受体(NMDAR)脑炎患者的临床特征和转归。

方法

观察性队列研究。

结果

在抗 NMDAR 脑炎的 661 例患者中,我们确定了 31 例年龄≥45 岁的患者。与年轻成人(18-44 岁)相比,老年患者更常为男性(45% vs. 12%,p<0.0001),肿瘤发生率较低(23% vs. 51%,p=0.002;很少为畸胎瘤),中位诊断时间(8 周 vs. 4 周,p=0.009)和治疗时间(7 周 vs. 4 周,p=0.039)更长,预后较差(2 年时改良 Rankin 量表评分 0-2,60% vs. 80%,p<0.026)。多变量分析显示,年龄较小(优势比 [OR] 0.15,置信区间 [CI] 0.05-0.39,p=0.0001)、早期治疗(OR 0.60,CI 0.47-0.78,p<0.0001)、无需重症监护(OR 0.09,CI 0.04-0.22,p<0.0001)和随访时间较长(p<0.0001)与良好结局相关。利妥昔单抗和环磷酰胺在一线免疫治疗失败时有效(OR 2.93,CI 1.10-7.76,p=0.031)。总体而言,60%的年龄大于 45 岁的患者在 24 个月随访时完全或基本恢复。

结论

与年轻成人相比,年龄≥45 岁的抗 NMDAR 脑炎患者的病情较轻,但老年患者的预后较差。在该年龄组中,诊断和治疗的延迟比年轻患者更为常见。潜在肿瘤的频率较低,但如果存在,通常为癌而不是年轻患者的畸胎瘤。早期和积极的免疫治疗可能会改善临床结局。

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