Takahashi Teruyuki, Kamei Satoshi, Miki Kenji, Ogawa Katsuhiko, Mizutani Tomohiko
Division of Neurology, Department of Internal Medicine, Nihon University School of Medicine.
Rinsho Shinkeigaku. 2003 Apr;43(4):162-9.
We report two cases of non-herpetic acute limbic encephalitis (NHALE) which showed elevation of interleukin (IL)-6 in the cerebrospinal fluid (CSF). [Case 1] The patient was a 25-year-old woman who was admitted to another hospital because of fever and severe headache, following common cold. After the admission, she developed severe disturbance of consciousness and suffered from generalized convulsions, and was then transferred to our hospital. The CSF examination revealed neither pleocytosis nor elevation of total protein. Her consciousness improved by intravenous administration of high-dose methylprednisolone, but mild retrograde amnesia and symptomatic epilepsy remained as sequelae. [Case 2] The patient was a 58-year-old man who was admitted to our hospital because of fever, severe headache, and mild disturbance of consciousness, following common cold. After the admission, he exhibited marked psychiatric symptoms and severe amnestic syndrome. The CSF examination revealed mild lymphocytic pleocytosis and mild elevation of total protein. His clinical symptoms improved markedly by intravenous administration of high-dose methylprednisolone, but mild retrograde amnesia and personality changes remained. Cranial MRI showed reversible high signal intensity lesions in bilateral hippocampi and amygdaloid bodies on diffusion weighted images (DWI) in both cases. No laboratory findings suggesting herpes simplex virus infection or malignancy were detected in either case. In the CSF analysis of cytokines including IL-1 beta, IL-2, IL-6, IL-10, tumor necrosis factor alpha, and interferony gamma, only IL-6 was elevated in both cases. We recognized four clinical features in both cases as follows: 1. the episode of preceding infection such as common cold, 2. appearance of reversible high signal intensity lesions in bilateral hippocampi and amygdaloid bodies on DWI, 3. elevation of only IL-6 in CSF, and 4. marked neurological improvement by intravenous administration of high-dose methylprednisolone. We speculate that the immune reaction of the host might play some significant roles in the pathogenesis of NHALE, based on these four clinical features.
我们报告了两例非疱疹性急性边缘叶脑炎(NHALE)病例,其脑脊液(CSF)中的白细胞介素(IL)-6水平升高。[病例1] 患者为一名25岁女性,因感冒后发热和严重头痛入住另一家医院。入院后,她出现了严重的意识障碍并伴有全身性惊厥,随后被转至我院。脑脊液检查显示既无细胞增多也无总蛋白升高。通过静脉注射大剂量甲基强的松龙,她的意识有所改善,但仍遗留轻度逆行性遗忘和症状性癫痫等后遗症。[病例2] 患者为一名58岁男性,因感冒后发热、严重头痛和轻度意识障碍入住我院。入院后,他出现了明显的精神症状和严重的遗忘综合征。脑脊液检查显示轻度淋巴细胞增多和总蛋白轻度升高。通过静脉注射大剂量甲基强的松龙,他的临床症状明显改善,但仍遗留轻度逆行性遗忘和人格改变。两例患者的头颅磁共振成像(MRI)在扩散加权成像(DWI)上均显示双侧海马和杏仁核有可逆性高信号病变。两例患者均未检测到提示单纯疱疹病毒感染或恶性肿瘤的实验室检查结果。在对包括IL-1β、IL-2、IL-6、IL-10、肿瘤坏死因子α和干扰素γ在内的细胞因子进行脑脊液分析时,两例患者仅IL-6升高。我们在两例患者中均认识到以下四个临床特征:1. 前驱感染如感冒发作;2. DWI上双侧海马和杏仁核出现可逆性高信号病变;3. 脑脊液中仅IL-6升高;4. 静脉注射大剂量甲基强的松龙后神经功能明显改善。基于这四个临床特征,我们推测宿主的免疫反应可能在NHALE的发病机制中起重要作用。