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The Egyptian Journal of Neurology, Psychiatry and Neurosurgery

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Open Access 01-12-2021 | Neuromyelitis Optica Spectrum Disease | Research

Area postrema syndrome in neuromyelitis optica spectrum disorder: diagnostic challenges and descriptive patterns

Authors: Eman M. Khedr, Hassan M. Farweez, Noha Abo Elfetoh, Eman R. Badawy, Sara Hassanein, Doaa M. Mahmoud, Ahmed Nasreldein

Published in: The Egyptian Journal of Neurology, Psychiatry and Neurosurgery | Issue 1/2021

Abstract

Background

Although area postrema syndrome (APS) is one of the core clinical features of neuromyelitis optic spectrum disorder (NMOSD), it is frequently misdiagnosed as gastrointestinal or systemic disorders. In this study, we describe the diagnostic challenges in NMOSD patients with APS and their characteristic clinical and radiological features. All patients who attended our university hospitals during the period from March 2019 to August 2020 with a diagnosis of NMOSD according to the latest diagnostic criteria were admitted and evaluated clinically, radiologically with gadolinium-enhanced brain and spinal MRI, measures of serum Anti-Aquaporin 4 (Anti-AQP4) and clinical status using the Expanded Disability Status Scale (EDSS) scores. APS was diagnosed if there was a history of intractable nausea, vomiting, or hiccups (INVH) that had lasted longer than 1 week with the exclusion of other etiologies, or less than 48 h if associated with a lesion in the dorsal medulla on MRI scan.

Results

Twenty out of 90 (22.2%) identified patients with a diagnosis of NMOSD had a history of unexplained intractable nausea, vomiting or hiccoughs lasting an average of 20 days. Seventeen patients were anti-Aquaporin 4 seropositive. Seven patients (35%) presented initially with isolated clinical features of APS and were diagnosed only after subsequent relapse. Patients with APS preceding other core clinical presentations (13 cases, 65%) were diagnosed after development of motor manifestations. All patients developed acute myelitis during the course of illness. Brain and spinal MRI scans showed that 13 had a linear lesion in the dorsal tegmentum of the medulla oblongata adjacent to the fourth ventricle. Otherwise, longitudinally extensive transverse myelitis was found in 80%, while 35% showed extension of the cord lesion to the AP.

Conclusions

APS as a core clinical characteristic of NMOSD is not a rare presentation as was previously thought and can occur in both AQP4-seropositive and seronegative NMOSD.

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Title: Area postrema syndrome in neuromyelitis optica spectrum disorder: diagnostic challenges and descriptive patterns
Authors: Eman M. Khedr
Hassan M. Farweez
Noha Abo Elfetoh
Eman R. Badawy
Sara Hassanein
Doaa M. Mahmoud
Ahmed Nasreldein
Publication date: 01-12-2021
Publisher: Springer Berlin Heidelberg
Keywords: Neuromyelitis Optica Spectrum Disease
Vomiting
Magnetic Resonance Imaging
Magnetic Resonance Imaging
Published in: The Egyptian Journal of Neurology, Psychiatry and Neurosurgery / Issue 1/2021
Electronic ISSN: 1687-8329
DOI: https://doi.org/10.1186/s41983-021-00390-5

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Springer Medicine

Area postrema syndrome in neuromyelitis optica spectrum disorder: diagnostic challenges and descriptive patterns

Abstract

Background

Results

Conclusions

Keynote webinar | Spotlight on medication adherence

Springer Medicine

Abstract

Background

Results

Conclusions

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