dxy logo
首页丁香园病例库全部版块
搜索
登录

【资讯翻译】Ann Neurol: 脱髓鞘疾病与抗NMDAR脑炎常合并发生

医疗行业从业者 · 最后编辑于 2014-04-21 · IP 新加坡新加坡
2971 浏览
这个帖子发布于 11 年零 85 天前,其中的信息可能已发生改变或有所发展。
Overlapping demyelinating syndromes and anti–N-methyl-D-aspartate receptor encephalitis
脱髓鞘综合征与抗N-甲基-D-天门冬氨酸受体脑炎合并发生
Objective: To report the clinical, radiological, and immunological association of demyelinating disorders with anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis.
目的:报告脱髓鞘综合征与抗N-甲基-D-天门冬氨酸受体(NMDAR)脑炎之间临床,放射学以及免疫学相关性。
Methods: Clinical and radiological analysis of a cohort of 691 patients with anti-NMDAR encephalitis. Determination of antibodies to NMDAR, aquaporin-4 (AQP4) and myelin oligodendrocyte glycoprotein (MOG) was performed using brain immunohistochemistry and cell-based assays.
方法:入选了691例抗NMDAR脑炎的患者,对其进行临床和放射学的分析。采用脑免疫组织化学法以及基于细胞的分析,明确抗NMDAR,水通道蛋白-4(AQP4)以及髓鞘少突胶质细胞糖蛋白(MOG)抗体。

Results: Twenty-three of 691 patients with anti-NMDAR encephalitis had prominent MRI and/or clinical features of demyelination. Group 1 included 12 patients in whom anti-NMDAR encephalitis was preceded or followed by independent episodes of NMO-spectrum disorder (5 cases, 4 anti-AQP4-positive), or brainstem or multifocal demyelinating syndromes (7 cases, all anti-MOG-positive). Group 2 included 11 patients in whom anti-NMDAR encephalitis occurred simultaneously with MRI and symptoms compatible with demyelination (5 AQP4-positive, 2 MOG-positive). Group 3 (136 controls) included 50 randomly selected patients with typical anti-NMDAR encephalitis, 56 with NMO, and 30 with multiple sclerosis: NMDAR-antibodies were detected only in the 50 anti-NMDAR patients, MOG-antibodies in 3/50 anti-NMDAR and 1/56 NMO patients, and AQP4-antibodies in 48/56 NMO and 1/50 anti-NMDAR patients (p<0.0001 for all comparisons with Groups 1 and 2). Most patients improved with immunotherapy, but compared with anti-NMDAR encephalitis the demyelinating episodes required more intensive therapy and resulted in more residual deficits. Only 1/23 NMDAR patients with signs of demyelination had ovarian teratoma compared with 18/50 anti-NMDAR controls (p=0.011)
结果:691例抗NMDAR脑炎的患者中有23例存在显著的脱髓鞘疾病的磁共振成像(MRI)特征和/或临床特征。第一组有12例抗NMDAR脑炎的患者,他们在发病之前或之后均有独立发生的NMO谱系疾病(5 例患者, 4 例抗AQP4 抗体阳性),或者脑干/多灶性脱髓鞘综合征(7 例患者, 所有患者抗MOG抗体阳性)。第2组包含11例抗NMDAR脑炎患者,这些患者同时出现脱髓鞘的MRI以及症状表现(5 例AQP4 抗体阳性, 2 例抗MOG 抗体阳性)。第3组患者(136例对照者)包括50例随机选择的典型的抗NMDAR脑炎,56例NMO患者,30例多发性硬化患者:仅在50例抗NMDAR脑炎的患者中检测到NMDAR抗体,3例抗NMDAR脑炎(共50例)以及1例NMO患者(共56例)出现抗MOG抗体;48例NMO患者以及1例NMDAR患者出现AQP-4抗体阳性(与第1,2组相比,p<0.0001)。大部分患者在免疫治疗后症状改善,但与抗NMDAR脑炎相比,脱髓鞘疾病发作的患者需要更强化的治疗以及导致更多的残留损害。伴有脱髓鞘体征的23例NMDAR患者中,只有1例患者出现卵巢畸胎瘤,而与之相比,50例对照组中抗NMDAR患者中,有18例患者出现卵巢畸胎瘤(p=0.011)。
Interpretation: Patients with anti-NMDAR encephalitis may develop concurrent or separate episodes of demyelinating disorders, and conversely patients with NMO or demyelinating disorders with atypical symptoms (e.g., dyskinesias, psychosis) may have anti-NMDAR encephalitis.
解释:抗NMDAR脑炎的患者可能同时伴发或单独发生脱髓鞘疾病的发作;而与之相反,伴有不典型症状(如运动障碍,精神症状)的NMO或脱髓鞘疾病的患者可能发生抗NMDAR脑炎。









全部讨论(0)

默认最新
avatar
3
分享帖子
share-weibo分享到微博
share-weibo分享到微信
认证
返回顶部