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Conus involvement and leptomeningeal enhancement in aquaporin-4 antibody-positive neuromyelitis optica spectrum disorder: A case report.

MedStar author(s):

Citation: Multiple Sclerosis and Related Disorders. 52:103011, 2021 May 07.PMID: 34015641Institution: MedStar Washington Hospital CenterDepartment: Neurology | Neurology ResidencyForm of publication: Journal ArticleMedline article type(s): LetterSubject headings: IN PROCESS -- NOT YET INDEXEDYear: 2021 ISSN:

2211-0348

Name of journal: Multiple sclerosis and related disordersAbstract: There are a variety of clinical phenotypes and radiological features that continue to make a diagnosis of neuromyelitis optica spectrum disorder (NMOSD) challenging. We present an atypical case of an adult woman who presented with flaccid paralysis of all extremities with unusual neuroimaging features, including extensive enhancing lesions in the upper cervical cord and conus medullaris with associated leptomeningeal enhancement. She was ultimately found to have AQP4 antibody-positive NMOSD. We discuss the factors that complicated a timely diagnosis, including her atypical radiographic features and an initially negative cell-based assay for myelin oligodendrocyte glycoprotein (MOG) and aquaporin-4 (AQP4) antibodies. Despite the rarity of conus medullaris involvement or leptomeningeal enhancement in AQP4 antibody-positive NMOSD, it is important to maintain a high level of clinical suspicion to avoid diagnostic and therapeutic delays. Though cell-based assays have high sensitivities, testing should be repeated on negative values in these scenarios. Copyright (c) 2021 Elsevier B.V. All rights reserved.All authors: Hu NN, Myers CJ, Osborne B, Safadi ALFiscal year: FY2021 Digital Object Identifier:

https://dx.doi.org/10.1016/j.msard.2021.103011

Date added to catalog: 2021-06-28

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Item type	Current library	Collection	Call number	Status	Date due	Barcode
Journal Article	MedStar Authors Catalog	Article	34015641	Available		34015641

There are a variety of clinical phenotypes and radiological features that continue to make a diagnosis of neuromyelitis optica spectrum disorder (NMOSD) challenging. We present an atypical case of an adult woman who presented with flaccid paralysis of all extremities with unusual neuroimaging features, including extensive enhancing lesions in the upper cervical cord and conus medullaris with associated leptomeningeal enhancement. She was ultimately found to have AQP4 antibody-positive NMOSD. We discuss the factors that complicated a timely diagnosis, including her atypical radiographic features and an initially negative cell-based assay for myelin oligodendrocyte glycoprotein (MOG) and aquaporin-4 (AQP4) antibodies. Despite the rarity of conus medullaris involvement or leptomeningeal enhancement in AQP4 antibody-positive NMOSD, it is important to maintain a high level of clinical suspicion to avoid diagnostic and therapeutic delays. Though cell-based assays have high sensitivities, testing should be repeated on negative values in these scenarios. Copyright (c) 2021 Elsevier B.V. All rights reserved.

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