Clinical Analysis of NMOSD with Area Postrema Syndrome as Initial Symptom

Abstract

Objective: Patients with neuromyelitis optica spectrum disease (NMOSD) with the initial manifestation of area postrema syndrome (APS) often have unexplained nausea and vomiting and are easily misdiagnosed for the NMOSD. The purpose of this study was to report and discuss clinical analysis, including diagnosis and treatment of 4 cases of NMOSD with APS as the first symptom.Methods: Four patients with intractable nausea and vomiting were selected for the analysis and finally the cases were confirmed for the NMOSD. All of these patients started with misdiagnosis and mismanagement initially.Results: Among the 4 patients included in this study, 3 were admitted to the department of gastroenterology at the onset of the disease, and 2 of them were not correctly diagnosed and treated on time due to misdiagnosis. Therefore, their symptoms worsened, and they needed to be transferred to ICU for life support. No obvious early medulla lesions were found in one patient. One patient was treated with intravenous immunoglobulin, methylprednisolone, and plasma exchange but there was no significant clinical improvement, and then the disease was relapsed during the treatment with low-dose rituximab.Conclusion: The clinical manifestations of NMOSD are complex and diverse, and the initial symptoms, onset age of the patient, and MRI findings can all influence the clinicians' judgment of the disease. Early identification of the APS and timely therapy can prevent visual and physical disabilities, even respiratory failure and cardiac arrest. Therefore, it is necessary to identify specific and sensitive serum and imaging markers for predicting the prognosis and recurrence of the disease.