Highlights
- •A total of 180 Portuguese patients met the 2015 Wingerchuk NMOSD criteria, 77 were AQP4-antibody positive (Abs+), 67 MOG-Abs+, and 36 seronegative.
- •Point prevalence on December 31, 2018 was 1.71/100,000 for NMOSD, 0.71/100,000 for AQP4-Abs+, 0.65/100,000 for MOG-Abs+, and 0.35/100,000 for seronegative NMOSD.
- •A total of 44 new NMOSD cases were identified during the two-year study period (11 AQP4-Abs+, 27 MOG-Abs+, and 6 seronegative). The annual incidence rate in that period was 0.21/100,000 person-years for NMOSD, 0.05/100,000 for AQP4-Abs+, 0.13/100,000 for MOG-Abs+, and 0.03/100,000 for seronegative NMOSD.
- •Epidemiological/clinical NMOSD profiles in the Portuguese population are similar to other European countries.
- •Although it is a rare disorder, the significant number of patients we found justifies the existence of reference centers, favouring clinical assessment and treatment by teams with growing and accumulated experience.
Abstract
Introduction
Neuromyelitis optica spectrum disorder (NMOSD) is a rare disorder in which astrocyte
damage and/or demyelination often cause severe neurological deficits.
Objective
To identify Portuguese patients with NMOSD and assess their epidemiological/clinical
characteristics.
Methods
This was a nationwide multicenter study. Twenty-four Portuguese adult and 3 neuropediatric
centers following NMOSD patients were included.
Results
A total of 180 patients met the 2015 Wingerchuk NMOSD criteria, 77 were AQP4-antibody
positive (Abs+), 67 MOG-Abs+, and 36 seronegative. Point prevalence on December 31,
2018 was 1.71/100,000 for NMOSD, 0.71/100,000 for AQP4-Abs+, 0.65/100,000 for MOG-Abs+,
and 0.35/100,000 for seronegative NMOSD. A total of 44 new NMOSD cases were identified
during the two-year study period (11 AQP4-Abs+, 27 MOG-Abs+, and 6 seronegative).
The annual incidence rate in that period was 0.21/100,000 person-years for NMOSD,
0.05/100,000 for AQP4-Abs+, 0.13/100,000 for MOG-Abs+, and 0.03/100,000 for seronegative
NMOSD.
AQP4-Abs+ predominated in females and was associated with autoimmune disorders. Frequently
presented with myelitis. Area postrema syndrome was exclusive of this subtype, and
associated with higher morbidity/mortality than other forms of NMOSD. MOG-Ab+ more
often presented with optic neuritis, required less immunosuppression, and had better
outcome.
Conclusion
Epidemiological/clinical NMOSD profiles in the Portuguese population are similar to
other European countries
Keywords
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Article info
Publication history
Published online: September 07, 2021
Accepted:
September 5,
2021
Received in revised form:
August 14,
2021
Received:
July 5,
2021
Identification
Copyright
© 2021 Elsevier B.V. All rights reserved.
