Correlations among disability, anti-AQP4 antibody status and prognosis in the spinal cord involved patients with NMOSD
Abstract 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 le...
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| Format: | Article |
| Language: | English |
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BMC
2021-04-01
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| Series: | BMC Neurology |
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| Online Access: | https://doi.org/10.1186/s12883-021-02171-2 |
| _version_ | 1819142140053159936 |
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| author | Jung Lung Hsu Ming-Feng Liao Kuo-Hsuan Chang Mei-Yun Cheng Long-Sun Ro |
| author_facet | Jung Lung Hsu Ming-Feng Liao Kuo-Hsuan Chang Mei-Yun Cheng Long-Sun Ro |
| author_sort | Jung Lung Hsu |
| collection | DOAJ |
| description | Abstract 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. |
| first_indexed | 2024-12-22T12:05:36Z |
| format | Article |
| id | doaj.art-acc1ced02d14450f93199108905977b0 |
| institution | Directory Open Access Journal |
| issn | 1471-2377 |
| language | English |
| last_indexed | 2024-12-22T12:05:36Z |
| publishDate | 2021-04-01 |
| publisher | BMC |
| record_format | Article |
| series | BMC Neurology |
| spelling | doaj.art-acc1ced02d14450f93199108905977b02022-12-21T18:26:27ZengBMCBMC Neurology1471-23772021-04-012111910.1186/s12883-021-02171-2Correlations among disability, anti-AQP4 antibody status and prognosis in the spinal cord involved patients with NMOSDJung Lung Hsu0Ming-Feng Liao1Kuo-Hsuan Chang2Mei-Yun Cheng3Long-Sun Ro4Department of Neurology, New Taipei Municipal TuCheng Hospital, Chang Gung Memorial Hospital and Chang Gung UniversityDepartment of Neurology, Chang Gung Memorial Hospital Linkou Medical Center and College of Medicine, Chang-Gung UniversityDepartment of Neurology, Chang Gung Memorial Hospital Linkou Medical Center and College of Medicine, Chang-Gung UniversityDepartment of Neurology, Chang Gung Memorial Hospital Linkou Medical Center and College of Medicine, Chang-Gung UniversityDepartment of Neurology, Chang Gung Memorial Hospital Linkou Medical Center and College of Medicine, Chang-Gung UniversityAbstract 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.https://doi.org/10.1186/s12883-021-02171-2NMOSDEDSSAnti-AQP4MRI |
| spellingShingle | Jung Lung Hsu Ming-Feng Liao Kuo-Hsuan Chang Mei-Yun Cheng Long-Sun Ro Correlations among disability, anti-AQP4 antibody status and prognosis in the spinal cord involved patients with NMOSD BMC Neurology NMOSD EDSS Anti-AQP4 MRI |
| 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 | NMOSD EDSS Anti-AQP4 MRI |
| url | https://doi.org/10.1186/s12883-021-02171-2 |
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