Analysis of relapses in anti-NMDAR encephalitis
Analysis of relapses in anti-NMDAR encephalitis
复制标题
DOI：
10.1212/wnl.0b013e31822cfc6b
复制
发表时间：
2011-09-01
期刊：
影响因子：
9.9
通讯作者：
Graus, F.
中科院分区：
文献类型：
--
作者：
Gabilondo, I.;Saiz, A.;Graus, F.
目的：复发性抗nmda受体脑炎（NMDAR）患者的临床特征不明确。在这项研究中，我们报告了一系列抗nmdar脑炎复发的临床概况和结果。方法：我们对25例抗nmdar脑炎复发患者进行回顾性分析。复发被定义为任何新的精神或神经系统综合症，不能由其他原因解释，在免疫治疗后改善，或较少情况下自发改善。结果：6例患者复发13次。其中四个人有过数次复发，从2次到4次。首次复发的中位延迟为2年（0.5至13年）。中位复发率为0.52次/患者-年。在首次发作时未接受免疫治疗的患者复发风险更高（p = 0.009）。多数病例（53%）表现为典型抗nmdar脑炎的部分症状。复发的主要症状为语言障碍（61%）、精神障碍（54%）、意识-注意力障碍（38%）和癫痫发作（31%）。3例复发（23%）表现为孤立的非典型症状，提示脑干-小脑受累。只有1例（17%）患者复发时发现卵巢畸胎瘤。复发并没有在首次发作的基础上增加残留缺陷。结论：抗nmdar脑炎复发是常见的（24%）。它们可能在最初发作多年后出现。复发可表现为部分症状或完全综合征的孤立症状。首发免疫治疗可降低复发风险。神经病学(R) 2011；77:996 - 999
Objective: The clinical characteristics of patients with relapsing anti-NMDA receptor (NMDAR) encephalitis are not well-defined. In this study, we report the clinical profile and outcome of relapses in a series of anti-NMDAR encephalitis.Methods: We did a retrospective review of relapses that occurred in 25 patients with anti-NMDAR encephalitis. Relapses were defined as any new psychiatric or neurologic syndrome, not explained by other causes, which improved after immunotherapy or, less frequently, spontaneously.Results: A total of 13 relapses were identified in 6 patients. Four of them had several, 2 to 4, relapses. There was a median delay of 2 years (range 0.5 to 13 years) for the first relapse. Median relapse rate was 0.52 relapses/patient-year. Relapse risk was higher in patients who did not receive immunotherapy in the first episode (p = 0.009). Most cases (53%) presented partial syndromes of the typical anti-NMDAR encephalitis. Main symptoms of relapses were speech dysfunction (61%), psychiatric (54%), consciousness-attention disturbance (38%), and seizures (31%). Three relapses (23%) presented with isolated atypical symptoms suggestive of brainstem-cerebellar involvement. An ovarian teratoma was detected at relapse in only 1 patient (17%). Relapses did not add residual deficit to that caused by the first episode.Conclusions: Relapses in anti-NMDAR encephalitis are common (24%). They may occur many years after the initial episode. Relapses may present with partial aspects or with isolated symptoms of the full-blown syndrome. Immunotherapy at first episode reduces the risk of relapses. Neurology (R) 2011;77:996-999