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Correlations among disability, anti-AQP4 antibody status and prognosis in the spinal cord involved patients with NMOSD

BACKGROUND: Neuromyelitis optica spectrum disorder (NMOSD) is a rare neuroinflammatory disorder of the central nervous system that typically involves the optic nerve, the spinal cord and other specific brain regions. In relapse of the disease, factors associated with clinical features and lesion sev...

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Autores principales: Hsu, Jung Lung, Liao, Ming-Feng, Chang, Kuo-Hsuan, Cheng, Mei-Yun, Ro, Long-Sun
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BioMed Central 2021
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8033738/
https://www.ncbi.nlm.nih.gov/pubmed/33836682
http://dx.doi.org/10.1186/s12883-021-02171-2
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author Hsu, Jung Lung
Liao, Ming-Feng
Chang, Kuo-Hsuan
Cheng, Mei-Yun
Ro, Long-Sun
author_facet Hsu, Jung Lung
Liao, Ming-Feng
Chang, Kuo-Hsuan
Cheng, Mei-Yun
Ro, Long-Sun
author_sort Hsu, Jung Lung
collection PubMed
description BACKGROUND: Neuromyelitis optica spectrum disorder (NMOSD) is a rare neuroinflammatory disorder of the central nervous system that typically involves the optic nerve, the spinal cord and other specific brain regions. In relapse of the disease, factors associated with clinical features and lesion severity are important for clinicians to predict disease-related disability. METHODS: We retrospectively analyzed 22 female patients with NMOSD who had spinal cord lesions. Detailed clinical features, onset symptoms, motor disability, relapse episodes, serum aquaporin-4 (AQP4) and myelin oligodendrocyte glycoprotein (MOG) autoantibodies and MRI characteristics were documented to correlate their associations with the nadir and three-month Expanded Disability Status Scale (EDSS) scores. Patients with three-month EDSS scores below four (< 4) were categorized as the good outcome group, while those with scores of four or more (> 4) were categorized as the poor outcome group. RESULTS: In patients with NMOSD, the mean age was 44.5 ± 12.8 years, and the mean three-month EDSS score was 4.3 ± 1.9. A significantly higher all-limb muscle power score was found in the good EDSS group than in the poor EDSS group (p = 0.01). A tendency toward longer follow-up periods and lower anti-AQP4 antibody levels was found in the good outcome group. Serum anti-AQP4 antibodies were present in 86% of patients with NMOSD, and MOG autoantibodies were found in one anti-AQP4 antibody-negative patient (33.3%). In patients with NMOSD, more than 40% of spinal cord lesions were distributed at the middle cervical and upper thoracic levels. CONCLUSIONS: Our findings suggest that EDSS scores and MRC scores at the nadir had significant associations with three-month EDSS scores. The topographic distributions of the spinal cord lesions might relate to different serum anti-AQP4 antibody status. However, further studies will be needed to corroborate this finding.
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spelling pubmed-80337382021-04-09 Correlations among disability, anti-AQP4 antibody status and prognosis in the spinal cord involved patients with NMOSD Hsu, Jung Lung Liao, Ming-Feng Chang, Kuo-Hsuan Cheng, Mei-Yun Ro, Long-Sun BMC Neurol Research Article BACKGROUND: Neuromyelitis optica spectrum disorder (NMOSD) is a rare neuroinflammatory disorder of the central nervous system that typically involves the optic nerve, the spinal cord and other specific brain regions. In relapse of the disease, factors associated with clinical features and lesion severity are important for clinicians to predict disease-related disability. METHODS: We retrospectively analyzed 22 female patients with NMOSD who had spinal cord lesions. Detailed clinical features, onset symptoms, motor disability, relapse episodes, serum aquaporin-4 (AQP4) and myelin oligodendrocyte glycoprotein (MOG) autoantibodies and MRI characteristics were documented to correlate their associations with the nadir and three-month Expanded Disability Status Scale (EDSS) scores. Patients with three-month EDSS scores below four (< 4) were categorized as the good outcome group, while those with scores of four or more (> 4) were categorized as the poor outcome group. RESULTS: In patients with NMOSD, the mean age was 44.5 ± 12.8 years, and the mean three-month EDSS score was 4.3 ± 1.9. A significantly higher all-limb muscle power score was found in the good EDSS group than in the poor EDSS group (p = 0.01). A tendency toward longer follow-up periods and lower anti-AQP4 antibody levels was found in the good outcome group. Serum anti-AQP4 antibodies were present in 86% of patients with NMOSD, and MOG autoantibodies were found in one anti-AQP4 antibody-negative patient (33.3%). In patients with NMOSD, more than 40% of spinal cord lesions were distributed at the middle cervical and upper thoracic levels. CONCLUSIONS: Our findings suggest that EDSS scores and MRC scores at the nadir had significant associations with three-month EDSS scores. The topographic distributions of the spinal cord lesions might relate to different serum anti-AQP4 antibody status. However, further studies will be needed to corroborate this finding. BioMed Central 2021-04-09 /pmc/articles/PMC8033738/ /pubmed/33836682 http://dx.doi.org/10.1186/s12883-021-02171-2 Text en © The Author(s) 2021 https://creativecommons.org/licenses/by/4.0/Open AccessThis article is licensed under a Creative Commons Attribution 4.0 International License, which permits use, sharing, adaptation, distribution and reproduction in any medium or format, as long as you give appropriate credit to the original author(s) and the source, provide a link to the Creative Commons licence, and indicate if changes were made. The images or other third party material in this article are included in the article's Creative Commons licence, unless indicated otherwise in a credit line to the material. If material is not included in the article's Creative Commons licence and your intended use is not permitted by statutory regulation or exceeds the permitted use, you will need to obtain permission directly from the copyright holder. To view a copy of this licence, visit http://creativecommons.org/licenses/by/4.0/ (https://creativecommons.org/licenses/by/4.0/) . The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/ (https://creativecommons.org/publicdomain/zero/1.0/) ) applies to the data made available in this article, unless otherwise stated in a credit line to the data.
spellingShingle Research Article
Hsu, Jung Lung
Liao, Ming-Feng
Chang, Kuo-Hsuan
Cheng, Mei-Yun
Ro, Long-Sun
Correlations among disability, anti-AQP4 antibody status and prognosis in the spinal cord involved patients with NMOSD
title Correlations among disability, anti-AQP4 antibody status and prognosis in the spinal cord involved patients with NMOSD
title_full Correlations among disability, anti-AQP4 antibody status and prognosis in the spinal cord involved patients with NMOSD
title_fullStr Correlations among disability, anti-AQP4 antibody status and prognosis in the spinal cord involved patients with NMOSD
title_full_unstemmed Correlations among disability, anti-AQP4 antibody status and prognosis in the spinal cord involved patients with NMOSD
title_short Correlations among disability, anti-AQP4 antibody status and prognosis in the spinal cord involved patients with NMOSD
title_sort correlations among disability, anti-aqp4 antibody status and prognosis in the spinal cord involved patients with nmosd
topic Research Article
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC8033738/
https://www.ncbi.nlm.nih.gov/pubmed/33836682
http://dx.doi.org/10.1186/s12883-021-02171-2
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