Area Postrema Syndrome

Objectives Area postrema syndrome (APS) is one of the core clinical features of neuromyelitis optic spectrum disorder (NMOSD). APS is mostly associated with neuromyelitis optica (NMO) and rarely reported in myelin oligodendrocyte glycoprotein antibody disease. We herein report a case of APS as an initial presentation of double-seropositive aquaporin-4 and myelin oligodendrocyte glycoprotein (MOG) antibodies.

Methods The patient fulfilled the NMOSD diagnostic criteria. Brain MRI, CSF studies, electrophysiologic test, and serum NMO and MOG antibody testing were performed.

Results An elderly woman initially presented to a gastroenterology outpatient department with a history of nausea, vomiting, and hiccups for 3 weeks. A detailed medical evaluation, including upper gastrointestinal endoscopy, was performed, which showed normal findings with no improvement with symptomatic therapy. A neurologic examination showed bilateral nystagmus, postural imbalance, and gait ataxia. An MRI examination of the brain showed T2/fluid attenuated inversion recovery hyperintensity in the dorsal medulla involving area postrema. Both anti-NMO and anti-MOG antibodies were found to be positive in serum. She was treated with intravenous methyl prednisolone with complete symptomatic resolution.

Discussion Double-seropositive APS-onset NMOSD has not been previously reported in literature. An early diagnosis and treatment result in the resolution of APS-related symptoms and prevent further progression of the disease.