Toyota Tomoko, Akamatsu Naoki, Tsuji Sadatoshi, Nishizawa Shigeru
Department of Neurology, School of Medicine, University of Occupational and Environmental Health, Japan.
J UOEH. 2014 Jun 1;36(2):129-33. doi: 10.7888/juoeh.36.129.
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.
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。