Differential diagnosis of neuromyelitis optica spectrum disorders

Neuromyelitis optica spectrum disorder (NMOSD) is an inflammatory disorder of the central nervous system (CNS) mostly manifesting as optic neuritis and/or myelitis, which are frequently recurrent/bilateral or longitudinally extensive, respectively. As the autoantibody to aquaporin-4 (AQP4-Ab) can me...

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Main Authors: Sung-Min Kim, Seong-Joon Kim, Haeng Jin Lee, Hiroshi Kuroda, Jacqueline Palace, Kazuo Fujihara
Format: Article
Language:English
Published: SAGE Publishing 2017-07-01
Series:Therapeutic Advances in Neurological Disorders
Online Access:https://doi.org/10.1177/1756285617709723
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author Sung-Min Kim
Seong-Joon Kim
Haeng Jin Lee
Hiroshi Kuroda
Jacqueline Palace
Kazuo Fujihara
author_facet Sung-Min Kim
Seong-Joon Kim
Haeng Jin Lee
Hiroshi Kuroda
Jacqueline Palace
Kazuo Fujihara
author_sort Sung-Min Kim
collection DOAJ
description Neuromyelitis optica spectrum disorder (NMOSD) is an inflammatory disorder of the central nervous system (CNS) mostly manifesting as optic neuritis and/or myelitis, which are frequently recurrent/bilateral or longitudinally extensive, respectively. As the autoantibody to aquaporin-4 (AQP4-Ab) can mediate the pathogenesis of NMOSD, testing for the AQP4-Ab in serum of patients can play a crucial role in diagnosing NMOSD. Nevertheless, the differential diagnosis of NMOSD in clinical practice is often challenging despite the phenotypical and serological characteristics of the disease because: (1) diverse diseases with autoimmune, vascular, infectious, or neoplastic etiologies can mimic these phenotypes of NMOSD; (2) patients with NMOSD may only have limited clinical manifestations, especially in their early disease stages; (3) test results for AQP4-Ab can be affected by several factors such as assay methods, serologic status, disease stages, or types of treatment; (4) some patients with NMOSD do not have AQP4-Ab; and (5) test results for the AQP4-Ab may not be readily available for the acute management of patients. Despite some similarity in their phenotypes, these NMOSD and NMOSD-mimics are distinct from each other in their pathogenesis, prognosis, and most importantly treatment. Understanding the detailed clinical, serological, radiological, and prognostic differences of these diseases will improve the proper management as well as diagnosis of patients.
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spelling doaj.art-52f707a5263142a2b857b8addb4e12d82022-12-22T00:16:00ZengSAGE PublishingTherapeutic Advances in Neurological Disorders1756-28561756-28642017-07-011010.1177/1756285617709723Differential diagnosis of neuromyelitis optica spectrum disordersSung-Min KimSeong-Joon KimHaeng Jin LeeHiroshi KurodaJacqueline PalaceKazuo FujiharaNeuromyelitis optica spectrum disorder (NMOSD) is an inflammatory disorder of the central nervous system (CNS) mostly manifesting as optic neuritis and/or myelitis, which are frequently recurrent/bilateral or longitudinally extensive, respectively. As the autoantibody to aquaporin-4 (AQP4-Ab) can mediate the pathogenesis of NMOSD, testing for the AQP4-Ab in serum of patients can play a crucial role in diagnosing NMOSD. Nevertheless, the differential diagnosis of NMOSD in clinical practice is often challenging despite the phenotypical and serological characteristics of the disease because: (1) diverse diseases with autoimmune, vascular, infectious, or neoplastic etiologies can mimic these phenotypes of NMOSD; (2) patients with NMOSD may only have limited clinical manifestations, especially in their early disease stages; (3) test results for AQP4-Ab can be affected by several factors such as assay methods, serologic status, disease stages, or types of treatment; (4) some patients with NMOSD do not have AQP4-Ab; and (5) test results for the AQP4-Ab may not be readily available for the acute management of patients. Despite some similarity in their phenotypes, these NMOSD and NMOSD-mimics are distinct from each other in their pathogenesis, prognosis, and most importantly treatment. Understanding the detailed clinical, serological, radiological, and prognostic differences of these diseases will improve the proper management as well as diagnosis of patients.https://doi.org/10.1177/1756285617709723
spellingShingle Sung-Min Kim
Seong-Joon Kim
Haeng Jin Lee
Hiroshi Kuroda
Jacqueline Palace
Kazuo Fujihara
Differential diagnosis of neuromyelitis optica spectrum disorders
Therapeutic Advances in Neurological Disorders
title Differential diagnosis of neuromyelitis optica spectrum disorders
title_full Differential diagnosis of neuromyelitis optica spectrum disorders
title_fullStr Differential diagnosis of neuromyelitis optica spectrum disorders
title_full_unstemmed Differential diagnosis of neuromyelitis optica spectrum disorders
title_short Differential diagnosis of neuromyelitis optica spectrum disorders
title_sort differential diagnosis of neuromyelitis optica spectrum disorders
url https://doi.org/10.1177/1756285617709723
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