Neuromyelitis optica spectrum disorders with recurrent syncope due to area postrema syndrome: a case report and literature review.

Abstract

Background: Area postrema syndrome is one of the core clinical features of neuromyelitis optica spectrum disorders (NMOSD), characterized by intractable hiccups, nausea, and vomiting. Brain MRI often reveals lesions in the dorsal medulla oblongata. However, recurrent syncope has rarely been described in NMOSD. In a few reported cases, patients have been diagnosed with bradycardia or orthostatic hypotension due to medullary lesions, often requiring pacemaker implantation or cardioneuroablation.

Case presentation: We reported a case of a 70-year-old female who presented with intractable nausea for 3 weeks and recurrent syncope for 2 days, while no remarkable lesions were found on brain MRI. A 24-h electrocardiogram (ECG) confirmed sick sinus syndrome, revealing multiple sinus pauses longer than 8 s. Meanwhile, high titer anti-aquaporin-4 immunoglobulin G was detected in her serum, leading to a diagnosis of NMOSD. Following treatment with one course of an intravenous methylprednisolone pulse and four courses of eculizumab, her symptoms of nausea and syncope resolved completely, her ECG normalized to sinus rhythm, and pacemaker implantation was successfully averted.

Conclusions: NMOSD should be considered a differential diagnosis in patients with recurrent syncope accompanied by intractable hiccups, nausea, and vomiting. Prompt immunotherapy may be sufficient for rhythm recovery, potentially eliminating the need for invasive procedures.