Abstract

BackgroundNeuromyelitis 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.MethodsWe 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.ResultsIn 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.ConclusionsOur 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.

Highlights

  • Neuromyelitis optica spectrum disorder (NMOSD) is a group of chronic inflammatory and demyelinating disorders that are characterized by optic neuritis, transverse myelitis and extensive brain lesions in locations such as the brainstem, the area postrema and the diencephalic regions [1, 2]

  • NMOSD is different from multiple sclerosis (MS) in that the former causes greater disability due to severe optic nerve damage and longitudinally extensive transverse myelitis (LETM), fewer brain magnetic resonance imaging (MRI) lesions and the presence of anti-AQP4 antibody in the serum and cerebrospinal fluid (CSF) [3, 4]

  • We plotted the topographic distribution of LETM according to positive and negative serum anti-AQP4 antibody results, and we found that the serum anti-AQP4 antibody-positive group had more frequent lesions at the cervical levels (C1-C5), while the anti-AQP4 antibody-negative group had more frequent lesions at the upper thoracic levels (Fig. 3b)

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Summary

Introduction

Neuromyelitis optica spectrum disorder (NMOSD) is a group of chronic inflammatory and demyelinating disorders that are characterized by optic neuritis, transverse myelitis and extensive brain lesions in locations such as the brainstem, the area postrema and the diencephalic regions [1, 2]. NMOSD is different from multiple sclerosis (MS) in that the former causes greater disability due to severe optic nerve damage and longitudinally extensive transverse myelitis (LETM), fewer brain magnetic resonance imaging (MRI) lesions and the presence of anti-AQP4 antibody in the serum and cerebrospinal fluid (CSF) [3, 4]. 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

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