Yamamoto Masanari, Kokubun Norito, Watanabe Yuka, Okabe Ryuta, Nakamura Toshiki, Hirata Koichi
Department of Neurology, Dokkyo Medical University.
Rinsho Shinkeigaku. 2013;53(5):345-50. doi: 10.5692/clinicalneurol.53.345.
We present the case of a 31-year-old woman who developed N-methyl-d-aspartate (NMDA) receptor encephalitis during the course of relapsing and remitting multiple brain lesions. The patient developed a tingling sensation in the left upper and lower extremities, and was first admitted to our hospital at age 27. She was tentatively diagnosed with multiple sclerosis on the basis of multiple lesions with Gd-enhancement in the brainstem, and 2 separate clinical relapses by age 28. At age 31, she developed a headache and pyrexia, followed by confusion and abnormal behavior. Her symptoms acutely progressed to stupor, and subsequently, she developed oral dyskinesia and athetosis-like involuntary movement of the left arm. The stupor state continued over 2 months. However, she had completely recovered by 3 months after the onset of psychiatric symptoms. Her serum and CSF samples tested positive for anti-NMDA receptor antibodies, and she was diagnosed with NMDA receptor encephalitis. Her serum was negative for anti-AQP4 antibody, but showed weak positivity for antinuclear antibody. Between ages 32 and 34, she experienced 2 clinical relapses, including right-hand clumsiness, confusion, aphasia, and dysphagia. FLAIR images showed a high-intensity area in the brain stem, thalamus, and subcortical white matter. No tumors were found throughout the course. A clinical entity of NMDA receptor encephalitis can include various neurologic disorders, such as the development of recurrent demyelinating brain lesions. Further investigation is required to clarify the pathophysiological role of anti-NMDA receptor antibody in our patient.
我们报告一例31岁女性病例，该患者在复发缓解型多发性脑损伤病程中发生了N-甲基-D-天冬氨酸（NMDA）受体脑炎。患者左上肢和下肢出现刺痛感，27岁时首次入住我院。基于脑干多发Gd强化病灶以及28岁前出现2次独立临床复发，她被初步诊断为多发性硬化。31岁时，她出现头痛和发热，随后出现意识模糊和异常行为。她的症状迅速进展为昏迷，随后出现口部运动障碍和左臂手足徐动样不自主运动。昏迷状态持续了2个多月。然而，在精神症状出现后3个月，她已完全康复。她的血清和脑脊液样本抗NMDA受体抗体检测呈阳性，她被诊断为NMDA受体脑炎。她的血清抗水通道蛋白4抗体阴性，但抗核抗体呈弱阳性。在32岁至34岁之间，她经历了2次临床复发，包括右手笨拙、意识模糊、失语和吞咽困难。液体衰减反转恢复（FLAIR）图像显示脑干、丘脑和皮质下白质有高强度区域。整个病程中未发现肿瘤。NMDA受体脑炎这一临床实体可包括各种神经系统疾病，如复发性脱髓鞘性脑损伤的发生。需要进一步研究以阐明抗NMDA受体抗体在我们患者中的病理生理作用。
