AQP4-IgG-seropositive neuromyelitis optica spectrum disorder (NMOSD) coexisting with anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis: A case report and literature review

Tao, Shuxin; Zhang, Yang; Ye, Hong; Guo, Dong

doi:10.1016/j.msard.2019.07.008

Cited by 18 publications

(7 citation statements)

References 15 publications

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“…The detection rate of positive antibodies has increased and will continue to increase due to improved autoimmune disease detection techniques. The specific mechanism of double-positive antibodies is not clear, and may be associated with autoimmune abnormalities, gene specificity, and tumor (Tao et al 2019 ). No tumor was found in our case, and the double-antibody positivity may be related to the special immune response of the body during pregnancy.…”

Section: Discussionmentioning

confidence: 99%

Recurrent anti-NMDAR encephalitis during pregnancy combined with two antibodies positive

Chen

2021

Arch Womens Ment Health

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Anti-N-methyl-d-aspartate receptor (NMDAR) encephalitis is an autoimmune synaptic encephalitis likely mediated by neuronal surface antibody. Clinically, it is characterized by a variety of neurological and psychiatric symptoms, predominantly affecting young women. Recurrent anti-NMDAR cases combined with double-antibody positive during pregnancy have not been reported. We report a 19-year-old pregnant woman with recurrent anti-NMDAR encephalitis and double-antibody positive. Through our case report and a review of the literature, we hope to heighten an awareness of anti-NMDAR encephalitis, particularly in a pregnant setting.

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Section: Discussionmentioning

confidence: 99%

Recurrent anti-NMDAR encephalitis during pregnancy combined with two antibodies positive

Chen

2021

Arch Womens Ment Health

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“…This strong association suggests a role of the tumor in the immunopathogenesis of the autoimmune disease. Furthermore, anti-NMDAR encephalitis has been reported in association with the central nervous system demyelinating diseases, such as ADEM (acute disseminated encephalomyelitis) [7], MOG-antibody related disease [8,9] and NMOSD [10][11][12]. Also, the occurrence of atypical symptoms for NMOSD (e.g.…”

Section: Discussionmentioning

confidence: 99%

Overlapping anti-N-methyl-D-aspartate receptor (NMDAR) Encephalitis With Neuromyelitis Optica Spectrum Disorders: A Case Report

Pan

Ovlyakulov

Zhou

2021

Preprint

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BackgroundAnti-N-methyl-D-aspartate receptor (NMDAR) encephalitis can coexist with neuromyelitis optica spectrum disorder (NMOSD). Patients with overlapping anti-NMDAR encephalitis with positive NMDAR antibodies and aquaporin 4 immunoglobulin G (AQP4-IgG)-seropositive NMOSD are rare but should not be ignored.Case presentationThis report describes a unique case of anti-NMDAR encephalitis coexisting with NMOSD is presented. A 27-year-old male presented with blurred vision, cognitive impairment, psychosis, dysphagia, gait instability and urinary incontinence. Brain magnetic resonance imaging (MRI) showed abnormal signals in the right cerebellar hemisphere, temporal lobe, and corpus callosum. NMDAR antibodies were positive in the CSF. AQP4-IgG antibodies were positive in the serum. The patient's condition was stable following intravenous gamma globulin, corticosteroids, immunosuppressants and symptomatic treatments. ConclusionsThis case provides further evidence for the occurrence of anti-NMDAR encephalitis overlapping NMOSD with AQP4-IgG-seropositive in a Chinese patient. However, the mechanisms underlying the occurrence of double positive antibodies remain elusive.

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“…For instance, the coexistence of NMDAR Ab and other autoantibodies associated with autoimmune encephalitis in MOGAD and AQP4 Ab-positive NMOSD patients has been observed. 183,184 AQP4 Ab-positive NMOSD has been found to occur in patients with AChR Abpositive myasthenia gravis with NMOSD onset most commonly occurring after diagnosis of MG. 185 This was further reflected in antibody titres, with AChR Ab decreasing as AQP4 Ab increased over the disease course.…”

Section: Standardised Serological Testingmentioning

confidence: 99%

“…Recent studies are also shedding light on the co‐occurrence of disease‐associated autoantibodies in CNS neurological disorders. For instance, the co‐existence of NMDAR Ab and other autoantibodies associated with autoimmune encephalitis in MOGAD and AQP4 Ab‐positive NMOSD patients has been observed 183,184 . AQP4 Ab‐positive NMOSD has been found to occur in patients with AChR Ab‐positive myasthenia gravis with NMOSD onset most commonly occurring after diagnosis of MG 185 .…”

Section: Current Trends and Future Directionsmentioning

confidence: 99%

Pathogenesis of autoimmune demyelination: from multiple sclerosis to neuromyelitis optica spectrum disorders and myelin oligodendrocyte glycoprotein antibody‐associated disease

et al. 2021

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Autoimmunity plays a significant role in the pathogenesis of demyelination. Multiple sclerosis (MS), neuromyelitis optica spectrum disorders (NMOSD) and myelin oligodendrocyte glycoprotein antibody-associated disease (MOGAD) are now recognised as separate disease entities under the amalgam of human central nervous system demyelinating disorders. While these disorders share inherent similarities, investigations into their distinct clinical presentations and lesion pathologies have aided in differential diagnoses and understanding of disease pathogenesis. An interplay of various genetic and environmental factors contributes to each disease, many of which implicate an autoimmune response. The pivotal role of the adaptive immune system has been highlighted by the diagnostic autoantibodies in NMOSD and MOGAD, and the presence of autoreactive lymphocytes in MS lesions. While a number of autoantigens have been proposed in MS, recent emphasis on the contribution of B cells has shed new light on the well-established understanding of T cell involvement in pathogenesis. This review aims to synthesise the clinical characteristics and pathological findings, discuss existing and emerging hypotheses regarding the aetiology of demyelination and evaluate recent pathogenicity studies involving T cells, B cells, and autoantibodies and their implications in human demyelination.

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AQP4-IgG-seropositive neuromyelitis optica spectrum disorder (NMOSD) coexisting with anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis: A case report and literature review

Cited by 18 publications

References 15 publications

Recurrent anti-NMDAR encephalitis during pregnancy combined with two antibodies positive

Recurrent anti-NMDAR encephalitis during pregnancy combined with two antibodies positive

Overlapping anti-N-methyl-D-aspartate receptor (NMDAR) Encephalitis With Neuromyelitis Optica Spectrum Disorders: A Case Report

Pathogenesis of autoimmune demyelination: from multiple sclerosis to neuromyelitis optica spectrum disorders and myelin oligodendrocyte glycoprotein antibody‐associated disease

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