Fujimori Juichi, Endo Minoru, Irino Shigemi, Shiga Yusei, Shiraishi Hirokazu, Motomura Masakatsu, Tanno Takashi, Hisanaga Kinya, Itoyama Yasuto
Department of Neurology, Tohoku Employees' Pension Welfare Hospital, Miyagi, Japan.
Brain Nerve. 2010 Nov;62(11):1252-7.
We report the case of a 65-year-old man who had encephalitis with a high titer of voltage-gated potassium channel antibodies (VGKC-Abs). His initial symptoms included memory disturbance, confusion, and seizures. Laboratory tests revealed a low plasma sodium concentration and a strong positive result for VGKC-Abs. A diffusion-weighted magnetic resonance imaging (MRI) scan showed a high intensity lesion within the right basal ganglia, which later showed normal intensity. The patient's initial symptoms resolved without any treatment. During the first relapse, the patient experienced consciousness disturbance and an increased number of seizures than that observed initially. A diffusion weighted MRI scan showed a high intensity lesion within the right hippocampus, and a fluid attenuated inversion recovery (FLAIR) weighted MRI scan showed high intensity lesions within the right hippocampus, right thalamus, and pons. The patient's symptoms and the MRI abnormalities resolved with prednisolone therapy. During the second relapse, he again experienced consciousness disturbance and an increased number of seizures than that observed initially. Diffusion-and FLAIR weighted MRI scans showed high intensity lesions within the right thalamus. However, the array of immunosuppressive treatments used during the first relapse was not as effective during the second relapse. The serum VGKC-Ab titers before steroid therapy during the first relapse and after immunosuppressive treatment during the second relapse were 1,252 pmol/L and 22.4 pmol/L, respectively. Brain MRI revealed signal changes in the basal ganglia at the onset of disease, in the limbic area during the first relapse, and in the thalamus during the second relapse. VGKC-Ab-associated encephalopathy is usually considered a benign autoimmune disorder; however, in our case, the encephalitis gradually became intractable to various immunosuppressive treatments, and unique MRI abnormalities were observed.
我们报告了一例65岁男性，其患有高滴度电压门控钾通道抗体（VGKC-Abs）相关的脑炎。他的初始症状包括记忆障碍、意识模糊和癫痫发作。实验室检查显示血浆钠浓度降低，VGKC-Abs检测结果呈强阳性。弥散加权磁共振成像（MRI）扫描显示右侧基底节区有一个高强度病灶，随后显示为正常强度。患者的初始症状未经任何治疗即自行缓解。在首次复发时，患者出现意识障碍，癫痫发作次数比最初观察到的有所增加。弥散加权MRI扫描显示右侧海马区有一个高强度病灶，液体衰减反转恢复（FLAIR）加权MRI扫描显示右侧海马区、右侧丘脑和脑桥有高强度病灶。患者的症状及MRI异常经泼尼松龙治疗后缓解。在第二次复发时，他再次出现意识障碍，癫痫发作次数比最初观察到 的有所增加。弥散加权和FLAIR加权MRI扫描显示右侧丘脑有高强度病灶。然而，首次复发时使用的一系列免疫抑制治疗在第二次复发时效果不佳。首次复发时激素治疗前及第二次复发免疫抑制治疗后的血清VGKC-Ab滴度分别为1252 pmol/L和22.4 pmol/L。脑部MRI显示疾病发作时基底节区有信号改变，首次复发时边缘区有信号改变，第二次复发时丘脑有信号改变。VGKC-Ab相关的脑病通常被认为是一种良性自身免疫性疾病；然而，在我们的病例中，脑炎逐渐变得对各种免疫抑制治疗难以控制，并观察到独特的MRI异常。
