Neurology:男性抗NMDA受体脑炎常以癫痫发作起病

2014-01-23 佚名 不详

抗NMDA受体脑炎最早在伴卵巢畸胎瘤的年轻女性患者中发现。该研究的目的是描述 抗NMDA受体抗体脑炎的临床表现及特异性。来自法国的Aurélien Viaccoz等学者研究了男性抗NMDA受体脑炎的患者,发现其与女性患者表现有所差异。 该研究为观察性研究,纳入法国副肿瘤神经系统综合征研究中心的13例诊断为抗NMDA受体脑炎的男性患者。 研究发现成年男性患者多以癫

抗NMDA受体脑炎最早在伴卵巢畸胎瘤的年轻女性患者中发现。该研究的目的是描述 抗NMDA受体抗体脑炎的临床表现及特异性。来自法国的Aurélien Viaccoz等学者研究了男性抗NMDA受体脑炎的患者,发现其与女性患者表现有所差异。

该研究为观察性研究,纳入法国副肿瘤神经系统综合征研究中心的13例诊断为抗NMDA受体脑炎的男性患者。

研究发现成年男性患者多以癫痫发作(8/13,61.5%)起病。8例患者中5例为部分性发作,在平均12天后出现精神或认知方面的症状。相反,成年女性患者初始时较少有癫痫发作,且癫痫发作类型为全面性发作,很快(平均2天)后出现行为和精神方面的症状。

除此之外,男性患者很少与肿瘤相关,13例中只有1例有肿瘤,为会阴部神经鞘瘤,而女性患者中41%与肿瘤相关,多为卵巢畸胎瘤。两组患者的辅助检查,治疗方法,临床演变和预后方面表现相似。

男性抗NDA受体脑炎患者表现与女性患者不同,对于以部分性发作起病、MRI表现正常且没有明显病因的患者需要检测NMDA受体抗体以免延误初始治疗。诊断为抗NMDA受体脑炎的女性患者很少以癫痫发作作为首发症状,其临床表现与男性患者不同,且其癫痫发作类型多为全面性发作且很快出现行为和精神方面的症状。两者临床表现的不同或由男性与女性患者激素影响所致。

研究背景:

抗NMDA受体脑炎是一种与抗NMDA受体抗体相关的边缘叶脑炎(LE),其发病机制尚不清楚。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、头MRI一般无特异性表现,血及脑脊液抗NMDA受体抗体阳性。该病对包括肿瘤切除和免疫治疗在内的联合治疗敏感。随着对该病认识的增加,越来越多的男性患者被发现。

原文出处:

Viaccoz A, Desestret V, Ducray F, Picard G, Cavillon G, Rogemond V, Antoine JC, Delattre JY, Honnorat J.Clinical specificities of adult male patients with NMDA receptor antibodies encephalitis.Neurology. 2014 Jan 17. 

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    2014-11-02 yinhl1978
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    2014-06-09 chen111111

    我发现几例都是男性患者,而且住院期间没有发现伴随肿瘤,与文章所列非常符合。

    0

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    2014-11-07 wetgdt
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