Department of Neurology, Research Institute and Hospital of National Cancer Center, Korea.
Mult Scler. 2012 Sep;18(9):1340-3. doi: 10.1177/1352458512441271. Epub 2012 Jul 3.
Advances in the understanding of central nervous system aquarporin-4 autoimmunity have promoted the recognition of diverse clinical presentations beyond the traditional view of neuromyelitis optica. We describe a patient who developed hemiparesis caused by an extensive cerebral lesion as an initial manifestation of central nervous system aquarporin-4 autoimmunity. Although the patient had no history of optic neuritis or myelitis, not only was serum anti-aquarporin-4 antibody positive, but an imaging, treatment response and histopathological features also revealed characteristic findings suggestive of central nervous system aquarporin-4 autoimmunity. The present case highlights the importance of a comprehensive evaluation for anti-aquarporin-4 antibody even in patients presenting with isolated cerebral lesions.
对中枢神经系统水通道蛋白-4 自身免疫的认识不断提高，促使人们认识到除视神经脊髓炎这一传统观点之外的多种临床表现。我们描述了一位患者，其以广泛的脑病变引起的偏瘫为首发表现，从而引发中枢神经系统水通道蛋白-4 自身免疫。尽管患者无视神经炎或脊髓炎病史，但血清抗水通道蛋白-4 抗体不仅为阳性，而且影像学、治疗反应和组织病理学特征也显示出特征性发现，提示中枢神经系统水通道蛋白-4 自身免疫。本病例强调了即使患者仅表现为孤立性脑病变，也应对抗水通道蛋白-4 抗体进行全面评估的重要性。
