Overlapping demyelinating syndrome (Neuromyelitis optica spectrum disorders NMOSD with anti-NMDA receptor encephalitis); A case report

Sinani, Ahmed Al; Maawali, Said Al; Alshekaili, Jalila; Kindi, Mahmood Al; Ramadhani, Khalsa Al; Khabouri, Jaber Al; Nadeem, Ahmed Sameer; Salti, Abdullah Al

doi:10.1016/j.msard.2020.102153

Cited by 9 publications

(8 citation statements)

References 23 publications

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“…At present, as a new method for the diagnosis of pathogen infection, metagenomic next-generation sequencing can help improve the detection rate of encephalitis caused by pathogen infection (Wilson et al, 2019). Although our study did not include cortical encephalitis caused by other pathogens, during the screening of the literature we found that MOG-Ab can coexist with other antibodies, especially anti-NMDAR-Ab (Zhou et al, 2017;Sinani et al, 2020;Nan et al, 2021). The new data show that NMDARs exist on the myelin sheath formed by oligodendrocytes (Lipton, 2006); therefore, NMDAR may be positive and can cause demyelination when an immune response occurs.…”

Section: Discussionmentioning

confidence: 86%

“…Although our study did not include cortical encephalitis caused by other pathogens, during the screening of the literature we found that MOG-Ab can coexist with other antibodies, especially anti-NMDAR-Ab ( Zhou et al, 2017 ; Sinani et al, 2020 ; Nan et al, 2021 ). The new data show that NMDARs exist on the myelin sheath formed by oligodendrocytes ( Lipton, 2006 ); therefore, NMDAR may be positive and can cause demyelination when an immune response occurs.…”

Section: Discussionmentioning

confidence: 97%

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Myelin Oligodendrocyte Glycoprotein Antibody Associated Cerebral Cortical Encephalitis: Case Reports and Review of Literature

Shu

Ding

Shang

et al. 2022

Front. Hum. Neurosci.

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Myelin oligodendrocyte glycoprotein antibody-associated disease is an immune-mediated demyelinating disease of the central nervous system that is present in both adults and children. The most common clinical manifestations are optic neuritis, myelitis, acute disseminated encephalomyelitis, and brainstem syndrome. Cerebral cortical encephalitis (CCE) is a rare clinical phenotype of myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease (MOGAD), which usually begins with seizures, headaches, and fever, and may be misdiagnosed as viral encephalitis in the early stages. Herein, we report two typical MOG antibody (MOG-Ab)-positive patients presenting with CCE, both of whom presented with headache, fever, seizures, and who recovered completely after immunotherapy. In addition, we performed a systematic review of the present literature from the perspectives of population characteristics, clinical symptoms, MRI abnormalities, treatments, and prognosis. Among the patients reported in 25 articles, 33 met our inclusion criteria, with the age of onset ranging from 4 to 52 years. Most of the patients had seizures, headache, fever, and unilateral cortical lesions on brain MRI. For acute CCE, 30 patients were treated with high-dose intravenous methylprednisolone, and the symptoms of most patients were completely relieved after immunotherapy. This study reported our experience and lessons learned in the diagnosis and treatment of MOG-Ab-positive CCE and provides a systematic review of the literature to analyse this rare clinical phenotype.

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Section: Discussionmentioning

confidence: 86%

Section: Discussionmentioning

confidence: 97%

Myelin Oligodendrocyte Glycoprotein Antibody Associated Cerebral Cortical Encephalitis: Case Reports and Review of Literature

Shu

Ding

Shang

et al. 2022

Front. Hum. Neurosci.

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“…Common localization for cognitive and psychiatric relapses in AQP4+ NMOSD have included lesions in the cerebral hemispheres (4), diencephalon (5), and corpus callosum (7), and both of our cases demonstrated prominent involvement of the latter. There have been reported cases of autoimmune encephalitis, in particular NMDA receptor antibody positive encephalitis, co-existing with AQP4+ NMOSD (8,9). This was considered in our second case because of the patient's psychiatric symptoms and confusion, but autoimmune encephalitis panel was negative.…”

Section: Discussionmentioning

confidence: 90%

Chronic Cognitive Impairment in AQP4+ NMOSD With Improvement in Cognition on Eculizumab: A Report of Two Cases

2022

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Cognitive impairment may be associated with aquaporin-4 antibody positive (AQP4+) NMOSD, particularly where there is prominent cerebral, corpus callosum, or thalamic involvement. It is unclear to what extent this phenomenon may be treatable after months to years. We describe two cases of AQP4+ NMOSD with cognitive impairment persisting over more than 6 months, where cognition improved after eculizumab was initiated. In the first case, a 51-year-old woman presented with a 2-month history of cognitive decline and ataxia, and diffuse involvement of the corpus callosum on MRI. AQP4 antibody testing returned positive. Cognitive impairment persisted on therapy with mycophenolate, then rituximab. She was switched to eculizumab from rituximab 18 months after disease onset because of breakthrough optic neuritis; memory and cognitive function improved on eculizumab. In the second case, a 26-year-old woman initially presented with visual, auditory and tactile hallucinations, and impairment in activities of daily living, and was given a diagnosis of schizophrenia. Nine months later she was hospitalized for increasing confusion. MRI showed leukoencephalopathy and diffuse involvement of the corpus callosum with multiple enhancing callosal lesions. AQP4 antibody testing was positive and CSF testing for other antibodies of autoimmune encephalitis was negative. She had some improvement in cognition with high dose corticosteroids but remained significantly impaired. On follow-up, her repeat MRI showed a small new right inferomedial frontal enhancing lesion although she did not complain of any new cognitive issues, her MOCA score was 21/30, and she was started on eculizumab. Two months after eculizumab initiation she and her family reported cognitive improvement and MOCA score was 25/30. Common features of these two cases included extensive callosal involvement and an element of ongoing gadolinium enhancement on MRI. Our experience suggests the possibility that cognitive impairment may be amenable to immunotherapy in certain cases of NMOSD.

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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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Overlapping demyelinating syndrome (Neuromyelitis optica spectrum disorders NMOSD with anti-NMDA receptor encephalitis); A case report

Cited by 9 publications

References 23 publications

Myelin Oligodendrocyte Glycoprotein Antibody Associated Cerebral Cortical Encephalitis: Case Reports and Review of Literature

Myelin Oligodendrocyte Glycoprotein Antibody Associated Cerebral Cortical Encephalitis: Case Reports and Review of Literature

Chronic Cognitive Impairment in AQP4+ NMOSD With Improvement in Cognition on Eculizumab: A Report of Two Cases

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

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