Highlights
- •Disability in NMOSD results from relapsing attacks.
- •Long-standing NMOSD is usually associated with severe disability.
- •A small proportion of patients remained fully functional in all systems 15 years after disease onset.
- •ARR 0.4 was the boundary line above which severe disability occurred.
- •Good prognosis associated with Caucasians, low ARR, and absent myelitis at disease onset.
Abstract
Background
Neuromyelitis optica spectrum disorders (NMOSD) most commonly cause severe disability
which is related to disease attacks. However, some patients retain good neurological
function for a long time after disease onset.
Objectives
To determine the frequency, demographic and the clinical features of good outcome
NMOSD, and analyze their predictive factors.
Methods
We selected patients who met the 2015 International Panel for NMOSD diagnostic criteria
from seven MS Centers. Assessed data included age at disease onset, sex, race, number
of attacks within the first and three years from onset, annualized relapsing rate
(ARR), total number of attacks, aquaporin-IgG serum status, presence of cerebrospinal
fluid (CSF)-specific oligoclonal bands (OCB) and the Expanded Disability Status Scale
(EDSS) score at the last follow-up visit. NMOSD was classified as non-benign if patients
developed sustained EDSS score >3.0 during the disease course, or benign if patients
had EDSS score ≤3.0 after ≥15 years from disease onset. Patients with EDSS <3.0 and
disease duration shorter than 15 years were not qualified for classification. We compared
the demographic and clinical characteristics of benign and non-benign NMOSD. Logistic
regression analysis identified predictive factors of outcome.
Results
There were 16 patients with benign NMOSD (3% of the entire cohort; 4.2% of those qualified
for classification; and 4.1% of those who tested positive for aquaporin 4-IgG), and
362 (67.7%) with non-benign NMOSD, whereas 157 (29.3%) did not qualify for classification.
All patients with benign NMOSD were female, 75% were Caucasian, 75% tested positive
for AQP4-IgG, and 28.6% had CSF-specific OCB. Regression analysis showed that female
sex, pediatric onset, and optic neuritis, area postrema syndrome, and brainstem symptoms
at disease onset, as well as fewer relapses in the first year and three years from
onset, and CSF-specific OCB were more commonly found in benign NMOSD, but the difference
did not reach statistical significance. Conversely, non-Caucasian race (OR: 0.29,
95% CI: 0.07–0.99; p = 0.038), myelitis at disease presentation (OR: 0.07, 95% CI: 0.01–0.52; p <0.001), and high ARR (OR: 0.07, 95% CI: 0.01–0.67; p = 0.011) were negative risk factors for benign NMOSD.
Conclusion
Benign NMOSD is very rare and occurs more frequently in Caucasians, patients with
low ARR, and those who do not have myelitis at disease onset.
Keywords
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Article info
Publication history
Published online: April 24, 2023
Accepted:
April 23,
2023
Received in revised form:
March 16,
2023
Received:
February 2,
2023
Identification
Copyright
© 2023 Elsevier B.V. All rights reserved.
