The journey of the patient living with NMOSD with area postrema syndrome as the inaugural Attack, a single center retrospective study

Abstract

One of the typical manifestations of Neuromyelitis optica spectrum disorder (NMOSD) is the Area Postrema syndrome (APS), defined by nausea, vomiting or hiccups lasting longer than 48 h. This syndrome is usually misdiagnosed and associated with diagnosis delay. This study aims to understand whether APS as the initial attack is associated with a longer time to first neurology visit and diagnosis in a Brazilian population. Data from 74 patients was collected from patient records and patient interviews. The most common first manifestation was optic neuritis 29 (39 %) and isolated APS was the first clinical syndrome in 12 patients (16 %). Median time to first medical care was 7 days in the APS group and 4.5 days in the non-APS group (p = 0.87). Median time for first neurological assessment was 232.5 days in the APS group while it was 25 days in the non-APS group (p < 0.001). The median time to first disease modifying drug was 344 days in the APS group and 164 days in the non-APS group (p = 0.051). All 12 APS patients had another attack before diagnosis with a median attack number before diagnosis of 3 (range 2–5) and 2 (range 1–5) in the non-APS group (p = 0.001). Acute treatment was performed in 45 (72.5 %) of non-APS group while no patients on the APS group had received acute treatment (p = 0.001). The APS as the first NMOSD manifestation was associated with delay to neurology assessment, higher number of attacks before diagnosis and untreated attacks.