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抗电压门控钾通道复合物抗体相关边缘叶脑炎作为成人发作性内侧颞叶癫痫的病因

Limbic encephalitis associated with anti-voltage-gated potassium channel complex antibodies as a cause of adult-onset mesial temporal lobe epilepsy.

作者信息

Toyota Tomoko, Akamatsu Naoki, Tsuji Sadatoshi, Nishizawa Shigeru

机构信息

Department of Neurology, School of Medicine, University of Occupational and Environmental Health, Japan.

出版信息

J UOEH. 2014 Jun 1;36(2):129-33. doi: 10.7888/juoeh.36.129.

DOI:10.7888/juoeh.36.129
PMID:24930877
Abstract

Recently, some reports have indicated that limbic encephalitis associated with anti-voltage-gated potassium channel complex antibodies (VGKC-Ab) is a cause of adult-onset mesial temporal lobe epilepsy (MTLE). We report a 53-year-old woman who had her first epileptic seizure at the age of 50 years old. Examination by 3-Tesla brain MRI revealed left hippocampal high signal intensity and swelling on fluid-attenuated inversion recovery (FLAIR) and T2-weighted imaging at 2 months after her first seizure. The patient received intravenous methylprednisolone and carbamazepine 300 mg/day. One month later, MRI revealed improvement of her left hippocampal abnormalities. Thereafter, she had no seizures, however, three years after her first seizure, EEG revealed a seizure pattern in the left temporal region. Brain MRI revealed left hippocampal high signal intensity and brain fluorodeoxyglucose positron emission tomography revealed hypermetabolism. Her serum VGKC-Ab levels were 118 pM(normal < 100 pM). Intravenous methylprednisolone therapy was reinitiated. Two months later, her hippocampal abnormalities had improved and 3 months later her VGKC-Ab levels decreased to 4.4 pM. Remission of the epileptic seizures was also observed. This MTLE in the middle age was considered as limbic encephalitis associated with anti- VGKC-Ab. In cases of unexplained adult-onset MTLE, limbic encephalitis associated with anti-VGKC-Ab, which responds well to immunotherapy, should be considered in the differential diagnosis.

摘要

最近,一些报告表明,与抗电压门控钾通道复合物抗体(VGKC-Ab)相关的边缘叶脑炎是成人发作性内侧颞叶癫痫(MTLE)的一个病因。我们报告一名53岁女性,她在50岁时首次癫痫发作。首次发作2个月后,3特斯拉脑MRI检查显示,在液体衰减反转恢复(FLAIR)序列和T2加权成像上,左侧海马呈高信号强度且肿胀。患者接受了静脉注射甲泼尼龙和每天300毫克卡马西平治疗。1个月后,MRI显示其左侧海马异常有所改善。此后,她未再发作,但在首次发作3年后,脑电图显示左侧颞区有癫痫发作模式。脑MRI显示左侧海马高信号强度,脑氟脱氧葡萄糖正电子发射断层扫描显示代谢亢进。她的血清VGKC-Ab水平为118皮摩尔/升(正常<100皮摩尔/升)。再次开始静脉注射甲泼尼龙治疗。2个月后,她的海马异常有所改善,3个月后她的VGKC-Ab水平降至4.4皮摩尔/升。癫痫发作也得到缓解。这名中年MTLE患者被认为是与抗VGKC-Ab相关的边缘叶脑炎。在不明原因的成人发作性MTLE病例中,鉴别诊断时应考虑与抗VGKC-Ab相关的边缘叶脑炎,其对免疫治疗反应良好。

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