Successful treatment of neuromyelitis optica spectrum disorder by early initiation of plasma exchange

: 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.