Koda Aya, Kaneko Satoshi, Asayama Shinya, Fujita Kengo, Kusaka Hirofumi
Department of Neurology, Kansai Medical University.
Rinsho Shinkeigaku. 2015;55(1):41-4. doi: 10.5692/clinicalneurol.55.41.
A 39-year-old woman initially developed vomiting and intractable hiccup, followed by progressive dysphagia, dysarthria and hypoglossal nerve palsy. She was admitted to our department on the 30th day of illness. MRI-FLAIR images of the brain revealed a hyperintense lesion in the dorsal medulla. A diagnosis of neuromyelitis optica spectrum disorder (NMOSD) was entertained according to the clinical course and the MRI images. The dysphagia was intractable to methylprednisolone pulse therapy, and so a course of plasma exchange therapy was initiated on the 32nd day of illness. After the third plasma exchange, the symptoms began to improve. Thereafter the patient's serum on admission was reported as positive for anti-aquaporin-4 antibody. Considering the irreversible nature of NMOSD pathology, early initiation of plasma exchange therapy is recommended to minimize the lesion in the case of steroid-refractory NMOSD patients.
一名39岁女性最初出现呕吐和顽固性呃逆，随后逐渐出现吞咽困难、构音障碍和舌下神经麻痹。她在发病第30天入住我科。脑部MRI-FLAIR图像显示延髓背侧有一个高信号病变。根据临床病程和MRI图像，考虑诊断为视神经脊髓炎谱系障碍（NMOSD）。吞咽困难对甲泼尼龙冲击治疗无效，因此在发病第32天开始进行一个疗程的血浆置换治疗。第三次血浆置换后，症状开始改善。此后，患者入院时的血清抗水通道蛋白4抗体检测呈阳性。考虑到NMOSD病理的不可逆性，对于激素难治性NMOSD患者，建议早期开始血浆置换治疗，以尽量减少损伤。
