Area Postrema Syndrome

Abstract

Abstract:Objectives:Area postrema syndrome (APS) is one of the core clinical feature of neuromyelitis optic spectrum disorder (NMOSD). APS is mostly associated with neuromyelitis optica (NMO) and rarely reported in myelin oligodendrocyte glycoprotein antibody disease (MOGAD). We herein report a case of area postrema syndrome as the initial presentation of double seropositive aquaporin-4 (AQP-4) and myelin oligodendrocyte glycoprotein (MOG)antibody.Methods:The patient fulfilled the NMOSD diagnostic criteria. MRI brain, CSF studies, electrophysiological test and serum NMO and MOG antibody testing were done.Results:An elderly female initially presented in Gastroenterology outpatient department with a history of nausea, vomiting and hiccups for 3 weeks. Detailed medical evaluation including upper gastrointestinal endoscopy was normal with no improvement with symptomatic therapy. Neurological examination showed bilateral nystagmus, postural imbalance and gait ataxia. MRI brain showed T2/FLAIR hyperintensity in dorsal medulla involving area postrema. Both anti NMO and 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. Early diagnosis and treatment results in resolution of APS- related symptoms and prevents further progression of disease.

The distinction of area postrema syndrome between MOGAD and NMOSD

“…No gastrointestinal disease was identified by the gastroenterologist for any patients. APS at onset especially when when it occurs independently might delay the diagnosis of NMOSD and cause further neurological deficits in the form of ON, transverse myelitis, or brainstem syndrome [ 10 ].…”

“…In the case report, the final diagnosis was MOGAD [ 9 ]. Abhay et al reported a case of APS as the initial presentation of double-seropositive aquaporin-4 and myelin oligodendrocyte glycoprotein (MOG) antibodies [ 10 ]. However, owing to its low frequency in MOGAD, data on APS involvement in MOGAD are limited to subsets of case series, and only a few studies have focused on APS in MOGAD [ 11 , 12 ].…”

A Narrative Review on the Clinical Relevance of Imaging the Circumventricular Brain Organs and Performing Their Anatomical and Histopathological Examination in Acute and Postacute COVID-19

“…Furthermore, the AP, SFO, and OVLT have fenestrated capillaries and are characterized by high blood-brain barrier permeability and thus are easily reachable by the SARS-CoV-2 virus via the bloodstream. Moreover, the AP has been associated clinically with the AP syndrome (characterized by intractable nausea, vomiting and hiccups for at least 48 hours) (eg, 47 ), which is also one of the core clinical features of neuromyelitis optica spectrum disorder (NMOSD). Neuromyelitis optica spectrum disorder is considered an autoimmune disease in which antibodies (ie, aquaporin-4-IgG or anti-AQP4 antibodies) are produced against the water channel protein aquaporin-4 (AQP4) present in perivascular astrocytes 48,49 .…”

“…3), to our knowledge, only the AP has been detected for clinical purposes to date. 65 There have been a few recent clinical reports showing alterations of the AP in patients affected by AP syndrome 47,65 and NMOSD including AP syndrome as the initial symptom. 65 Nevertheless, the latter investigation 65 did not address whether SFO or OVLT alterations were present as well.…”