Kubota Akihiro, Tajima Takashi, Narukawa Shinya, Yamazato Masamizu, Fukaura Hikoaki, Takahashi Yukitoshi, Tanaka Keiko, Shimizu Jun, Nomura Kyoichi
Department of Neurology, Saitama Medical Center, Saitama Medical University.
Rinsho Shinkeigaku. 2012;52(9):666-71. doi: 10.5692/clinicalneurol.52.666.
A 36-year-old man presented with cognitive impairment and disturbance of short-term memory functions with character change. Cerebrospinal fluid analysis revealed no abnormalities; however, brain MRI revealed high-signal intensity from bilateral hippocampus lesions on fluid attenuated inversion recovery (FLAIR) images and T(2) weighted images. The 18F-fluorodeoxyglucose PET demonstrated high glucose uptake in the bilateral hippocampus lesions. He was diagnosed as limbic encephalitis, and was administered high-dose intravenous methylprednisolone and immune adsorption plasma therapy followed by intravenous immunoglobulin therapy. MRI abnormalities improved after treatment but recent memory disturbance remained. Ma2 antibody, NMDA-receptor antibody, and GluRε2 antibody were positive. Eleven months atter the onset of disease, the tumor was identified in left testicle by ultrasound and removed the tumor. The pathological findings were seminoma. We experienced a case of paraneoplastic limbic encephalitis associated with seminoma with short-term memory disturbance. The occurrence of paraneoplastic limbic encephalitis with antibodies against cell membrane (NMDA-receptor antibody and GluRε2 antibody) and intracellular (Ma2 antibody) is rare even in the literature.
一名36岁男性出现认知障碍、短期记忆功能障碍及性格改变。脑脊液分析未见异常;然而,脑部MRI在液体衰减反转恢复(FLAIR)图像和T2加权图像上显示双侧海马病变呈高信号强度。18F-氟脱氧葡萄糖PET显示双侧海马病变有高葡萄糖摄取。他被诊断为边缘叶脑炎,接受了大剂量静脉注射甲泼尼龙和免疫吸附血浆治疗,随后进行静脉注射免疫球蛋白治疗。治疗后MRI异常有所改善,但近期记忆障碍仍存在。Ma2抗体、N-甲基-D-天冬氨酸受体抗体和GluRε2抗体呈阳性。发病11个月后,通过超声在左侧睾丸发现肿瘤并切除。病理结果为精原细胞瘤。我们遇到一例与精原细胞瘤相关的副肿瘤性边缘叶脑炎伴短期记忆障碍。即使在文献中,出现针对细胞膜(N-甲基-D-天冬氨酸受体抗体和GluRε2抗体)和细胞内(Ma2抗体)抗体的副肿瘤性边缘叶脑炎也很罕见。