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Neurology:晚发型抗NMDA受体脑炎症状轻但预后更差

2013-08-28 sunight dxy

研究要点: 晚发型抗NMDA受体脑炎患者多为男性,肿瘤发病频率较低,往往延误诊断及治疗,临床预后更差。 利妥昔单抗和环磷酰胺治疗通常有效。 抗NMDA受体脑炎是近年来新发现的一类疾病,往往发生在伴随畸胎瘤的女性患者中,但是年龄超过45岁的成人晚发抗NMDA受体脑炎的临床特征和预后尚不明确。来自西班牙的Maarten J. Titulaer等学者对比了不



研究要点:

晚发型抗NMDA受体脑炎患者多为男性,肿瘤发病频率较低,往往延误诊断及治疗,临床预后更差。

利妥昔单抗和环磷酰胺治疗通常有效。

抗NMDA受体脑炎是近年来新发现的一类疾病,往往发生在伴随畸胎瘤的女性患者中,但是年龄超过45岁的成人晚发抗NMDA受体脑炎的临床特征和预后尚不明确。来自西班牙的Maarten J. Titulaer等学者对比了不同年龄组的抗NMDA受体脑炎情况,在线发表在2013年8月的Neurology杂志上,结果显示:晚发型抗NMDA受体脑炎症状轻但预后更差。【原文下载

该研究为观察性的队列研究,共纳入611例抗NMDA受体脑炎患者,对比不同年龄组的临床表现及预后。

在661例抗NMDA受体脑炎的患者队列中,有31例年龄超过45岁的患者。与年龄较轻,即18岁至44岁的患者相比,晚发型患者更多为男性,肿瘤的发病频率较低,诊断及治疗的平均时间更长,以改良Rankin评分评价,其临床预后更差。多因素分析显示,低年龄、早期治疗、不需重症监护治疗及更长的随访期与更好的临床预后相关。当一线免疫抑制治疗失败后,选用利妥昔单抗和环磷酰胺治疗通常有效。总的来说,超过60%的年龄超过45岁的患者在24个月随访期内基本痊愈。

研究结果显示,年龄超过45岁的抗NMDA受体脑炎患者相比年轻患者症状较轻,但是晚发型患者预后更差。在此年龄组中,延误诊断与治疗比年轻患者更为常见。晚发型患者伴发相关肿瘤的可能性更低,不过一旦发现其伴发有肿瘤,往往不是年轻患者中常见的畸胎瘤,而是以恶性肿瘤为主。早期及积极的免疫治疗往往能改善临床预后。

研究背景:

抗N-甲基-M-天冬氨酸受体脑炎,简称抗NMDA受体脑炎,是一种与抗NMDA受体抗体相关的边缘叶脑炎,其发病机制尚不明确。在年轻患者中该病常发生于伴有卵巢畸胎瘤的年轻女性患者,典型的临床表现为精神异常、痫性发作、意识障碍、中枢性通气不足、运动障碍以及自主神经功能紊乱,脑电图及头MRI一般无特异性表现,血及脑脊液抗NMDA受体抗体阳性,但是年龄超过45岁的晚发型抗NMDA受体脑炎的临床特征与预后有其独特的特点。

原始出处:

Titulaer MJ, McCracken L, Gabilondo I, Iizuka T, Kawachi I, Bataller L, Torrents A, Rosenfeld MR, Balice-Gordon R, Graus F, Dalmau J.Late-onset anti-NMDA receptor encephalitis.Neurology. 2013 Aug 14. 【原文下载

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    2013-12-21 wetgdt
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    2013-10-08 yinhl1978
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