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Published in: BMC Neurology 1/2021

Open Access 01-12-2021 | Magnetic Resonance Imaging | Research article

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

Authors: Jung Lung Hsu, Ming-Feng Liao, Kuo-Hsuan Chang, Mei-Yun Cheng, Long-Sun Ro

Published in: BMC Neurology | Issue 1/2021

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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.
Literature
3.
go back to reference Jarius S, Franciotta D, Paul F, Ruprecht K, Bergamaschi R, Rommer PS, et al. Cerebrospinal fluid antibodies to aquaporin-4 in neuromyelitis optica and related disorders: frequency, origin, and diagnostic relevance. J Neuroinflammation. 2010;7(1):52. https://doi.org/10.1186/1742-2094-7-52. Jarius S, Franciotta D, Paul F, Ruprecht K, Bergamaschi R, Rommer PS, et al. Cerebrospinal fluid antibodies to aquaporin-4 in neuromyelitis optica and related disorders: frequency, origin, and diagnostic relevance. J Neuroinflammation. 2010;7(1):52. https://​doi.​org/​10.​1186/​1742-2094-7-52.
14.
go back to reference Kitley J, Leite MI, Nakashima I, Waters P, McNeillis B, Brown R, et al. Prognostic factors and disease course in aquaporin-4 antibody-positive patients with neuromyelitis optica spectrum disorder from the United Kingdom and Japan. Brain. 2012;135(Pt 6):1834–49. https://doi.org/10.1093/brain/aws109. Kitley J, Leite MI, Nakashima I, Waters P, McNeillis B, Brown R, et al. Prognostic factors and disease course in aquaporin-4 antibody-positive patients with neuromyelitis optica spectrum disorder from the United Kingdom and Japan. Brain. 2012;135(Pt 6):1834–49. https://​doi.​org/​10.​1093/​brain/​aws109.
22.
go back to reference Pekcevik Y, Mitchell CH, Mealy MA, Orman G, Lee IH, Newsome SD, et al. Differentiating neuromyelitis optica from other causes of longitudinally extensive transverse myelitis on spinal magnetic resonance imaging. Mult Scler. 2016;22(3):302–11. https://doi.org/10.1177/1352458515591069. Pekcevik Y, Mitchell CH, Mealy MA, Orman G, Lee IH, Newsome SD, et al. Differentiating neuromyelitis optica from other causes of longitudinally extensive transverse myelitis on spinal magnetic resonance imaging. Mult Scler. 2016;22(3):302–11. https://​doi.​org/​10.​1177/​1352458515591069​.
27.
29.
go back to reference Chee CG, Park KS, Lee JW, Ahn HW, Lee E, Kang Y, et al. MRI features of Aquaporin-4 antibody-positive longitudinally extensive transverse myelitis: insights into the diagnosis of Neuromyelitis Optica Spectrum disorders. AJNR Am J Neuroradiol. 2018;39(4):782–7. https://doi.org/10.3174/ajnr.A5551. Chee CG, Park KS, Lee JW, Ahn HW, Lee E, Kang Y, et al. MRI features of Aquaporin-4 antibody-positive longitudinally extensive transverse myelitis: insights into the diagnosis of Neuromyelitis Optica Spectrum disorders. AJNR Am J Neuroradiol. 2018;39(4):782–7. https://​doi.​org/​10.​3174/​ajnr.​A5551.
33.
go back to reference Dommisse GF. The blood supply of the spinal cord. A critical vascular zone in spinal surgery. J Bone Joint Surg Br. 1974;56(2):225–35.CrossRef Dommisse GF. The blood supply of the spinal cord. A critical vascular zone in spinal surgery. J Bone Joint Surg Br. 1974;56(2):225–35.CrossRef
37.
go back to reference Jarius S, Ruprecht K, Wildemann B, Kuempfel T, Ringelstein M, Geis C, et al. Contrasting disease patterns in seropositive and seronegative neuromyelitis optica: a multicentre study of 175 patients. J Neuroinflammation. 2012;9(1):14. https://doi.org/10.1186/1742-2094-9-14. Jarius S, Ruprecht K, Wildemann B, Kuempfel T, Ringelstein M, Geis C, et al. Contrasting disease patterns in seropositive and seronegative neuromyelitis optica: a multicentre study of 175 patients. J Neuroinflammation. 2012;9(1):14. https://​doi.​org/​10.​1186/​1742-2094-9-14.
47.
Metadata
Title
Correlations among disability, anti-AQP4 antibody status and prognosis in the spinal cord involved patients with NMOSD
Authors
Jung Lung Hsu
Ming-Feng Liao
Kuo-Hsuan Chang
Mei-Yun Cheng
Long-Sun Ro
Publication date
01-12-2021
Publisher
BioMed Central
Published in
BMC Neurology / Issue 1/2021
Electronic ISSN: 1471-2377
DOI
https://doi.org/10.1186/s12883-021-02171-2

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