Seronegative neuromyelitis optica spectrum disorder with longitudinally extending transverse myelitis and optic neuritis: A case report

Khadka, Bibek; Bhattarai, Ayush Mohan; Dhakal, Bishal; Karki, Abinash; Acharya, Apshara; Poudel, Rajesh

doi:10.1016/j.amsu.2022.103757

Cited by 4 publications

(3 citation statements)

References 14 publications

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“…Prompt diagnosis and management is required to reduce the relapse of the disease. 6 Gibbons et al conducted a study on NMOSD in 725 patients out of which only 7% were seronegative NMOSD.…”

Section: Discussionmentioning

confidence: 99%

An interesting case of seronegative neuro myelitis optica spectrum disorder

Sriharsha

Kumar

et al. 2023

Int J Adv Med

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Neuromyelitis optica spectrum disorder (NMOSD) is an autoimmune disease that causes severe demyelination, especially in the optic nerve and spinal cord with typical clinical manifestations of acute optic neuritis and transverse myelitis. Seventy to ninety percent of the cases of NMOSD test positive for aquaporin 4 IgG Antibodies (AQP4 IgG Ab). Here we report a case of 22-year-old female who presented with history of diffuse headache since 1 week, history of double vision since1 week and excessive day time sleepiness since2 months. Patient had a past history of bilateral and simultaneous optic neuritis 9 months back. On further evaluation patient was diagnosed as seronegative NMOSD (AQP4 IgG Ab–negative).

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“…Prompt diagnosis and management is required to reduce the relapse of the disease. 6 Gibbons et al conducted a study on NMOSD in 725 patients out of which only 7% were seronegative NMOSD.…”

Section: Discussionmentioning

confidence: 99%

An interesting case of seronegative neuro myelitis optica spectrum disorder

Sriharsha

Kumar

et al. 2023

Int J Adv Med

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“…Another case report by Khadka et al involved a 35-year-old male presenting with weakness in the lower extremities diagnosed as double seronegative NMOSD. He developed optic neuritis and myelitis and was maintained on azathioprine and prednisone combination therapy [ 5 ].…”

Section: Introductionmentioning

confidence: 99%

Rituximab Therapy for Double Seronegative Neuromyelitis Optica Spectrum Disease

Saballegue,

Tiongson

2024

Cureus

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Neuromyelitis optica spectrum disorder (NMOSD) is a rare central nervous system disease presenting as optic neuritis, myelitis, and brainstem syndromes. It may be aquaporin-4 seropositive, anti-myelin oligodendrocyte glycoprotein (MOG) antibody seropositive, or double seronegative. Double-seronegative NMOSD can pose a diagnostic and therapeutic challenge. Treatment typically aims to decrease the incidence of relapse, for which high-dose intravenous methylprednisolone is the first-line agent. Non-steroid treatments include azathioprine, mycophenolate mofetil, and rituximab. This case describes a 45-year-old female presenting with left arm numbness and weakness for three months. She had been previously diagnosed with optic neuritis in 2013 but was lost to follow-up. Progression of weakness warranted admission to the neurology department. Diagnostic work and imaging were suggestive of neuromyelitis optica. Tests for aquaporin-4, anti-MOG, immunoglobulin G, and immunoglobulin M in the cerebrospinal fluid were all negative. Initial treatment comprised methylprednisolone; however, due to the progression of symptoms, she was given two cycles of rituximab. Rituximab targets the CD20 antigen in B cells and is thought to reduce the risk of relapse and the severity of NMOSD. The patient's Barthel index score, expanded disability status scale score, and motor examination improved after two cycles of rituximab.

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“…Case series support this hypothesis. 24 - 26 The only systematic analysis of DN-NMOSD so far described demographic, clinical, and imaging features of people with DN-NMOSD to cluster in a multiple sclerosis (MS)-like group, a NMOSD-like group, and a group with low brain lesion count—potentially suggesting multiple disease entities within the DN-NMOSD group. 18 Retinal involvement in DN-NMOSD has not been investigated in DN-NMOSD so far.…”

Section: Introductionmentioning

confidence: 99%

Retinal Changes in Double-Antibody Seronegative Neuromyelitis Optica Spectrum Disorders

Oertel,

Zimmermann,

Motamedi

et al. 2024

Neurol Neuroimmunol Neuroinflamm

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Background and ObjectivesTo systematically describe the clinical picture of double-antibody seronegative neuromyelitis optica spectrum disorders (DN-NMOSD) with specific emphasis on retinal involvement. MethodsCross-sectional data of 25 people with DN-NMOSD (48 eyes) with and without a history of optic neuritis (ON) were included in this study along with data from 25 people with aquaporin-4 antibody seropositive neuromyelitis optica spectrum disorder (AQP4-NMOSD, 46 eyes) and from 25 healthy controls (HCs, 49 eyes) for comparison. All groups were matched for age and sex and included from the collaborative retrospective study of retinal optical coherence tomography (OCT) in neuromyelitis optica (CROCTINO). Participants underwent OCT with central postprocessing and local neurologic examination and antibody testing. Retinal neurodegeneration was quantified as peripapillary retinal nerve fiber layer thickness (pRNFL) and combined ganglion cell and inner plexiform layer thickness (GCIPL). ResultsThis DN-NMOSD cohort had a history of [median (inter-quartile range)] 6 (5; 9) attacks within their 5 ± 4 years since onset. Myelitis and ON were the most common attack types. In DN-NMOSD eyes after ON, pRNFL (p < 0.001) and GCIPL (p = 0.023) were thinner compared with eyes of HCs. Even after only one ON episode, DN-NMOSD eyes already had considerable neuroaxonal loss compared with HCs. In DN-NMOSD eyes without a history of ON, pRNFL (p = 0.027) and GCIPL (p = 0.022) were also reduced compared with eyes of

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Seronegative neuromyelitis optica spectrum disorder with longitudinally extending transverse myelitis and optic neuritis: A case report

Cited by 4 publications

References 14 publications

An interesting case of seronegative neuro myelitis optica spectrum disorder

An interesting case of seronegative neuro myelitis optica spectrum disorder

Rituximab Therapy for Double Seronegative Neuromyelitis Optica Spectrum Disease

Retinal Changes in Double-Antibody Seronegative Neuromyelitis Optica Spectrum Disorders

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