Highlights
- •Asian type MS has two subtypes nowadays: opticospinal MS (OSMS) and Neuromyelitis optica (NMO). Both subtypes of the Asian type MS occur in the Brazilian population.
- •OSMS and NMO differ by demographic, clinical and laboratory data.
- •OSMS predominate in white women and has a milder clinical course.
- •OSMS negative for AQP4 and MOG antibodies is an MS phenotype.
Abstract
Background
A specific particularity of neurological diseases in Asia is the relative commonality
of neuromyelitis optica (NMO) and Asian type MS (OSMS). Both conditions also occur
in South American patients. The Brazilian population differs from the European and
the Asian populations due to the mixture of ancestralities between European colonizers
and African slaves. To better know the clinical characteristics of Brazilian patients
with Asian type MS this study aimed to analyze the clinical, radiological and serological
data that would help to distinguish between OSMS and NMO and clarify, in a Non-Asian
population, if OSMS is an MS phenotype, an NMO spectrum disorder by 2015 classification,
or a complement activating antibody to myelin oligodendrocyte glycoprotein (MOG-IgG)
antibody-related disease.
Methods
We selected cases retrospectively with NMO and OSMS in the medical registry of patients
with idiopathic inflammatory demyelinating diseases under follow-up since 1997 in
Federal Hospital da Lagoa, the principal reference center for MS treatment in Rio
de Janeiro, Brazil. OSMS has selective involvement of the optic nerve and spinal cord
with no cerebral or cerebellar symptoms associated with small spinal cord lesions
and negativity for the aquaporin-4 antibody (AQP4-IgG). NMO full-filled the revised
criteria (2006) associated with longitudinally extensive transverse myelitis (LETM).
We recorded the following data: ethnicity/skin color, neurologic impairment “at nadir”
and “at recovery” of the index events (optic neuritis and transverse myelitis), long
term disability, mortality, health quality of life scores by the SF-36 questionnaire,
CSF IgG oligoclonal bands and serological AQP4-IgG and MOG-IgG antibodies tested by
Cell-based assay. The last brain MRIs were classified as either satisfying or not
satisfying MAGNIMS radiologic criteria for MS or typical or not typical for NMOSD.
The new classification of NMO spectrum disorders (2015) was applied.
Results
Forty-one OSMS and 122 NMO cases were analyzed. OSMS affected mainly young white women,
causing unilateral optic neuritis and partial myelitis with excellent recovery. After
a mean disease duration of 20 years, 90% of the patients had free ambulation, and
70% had a mild disability or no disability. Only 7.2% presented a secondary progressive
course, and no deaths occurred. All cases had negativity to AQP4-IgG and MOG-IgG biomarkers.
95% had resonance criteria for MS. OSMS differed from NMO by ethnicity, morbidity,
and mortality: most were African descendants, with severe motor and visual dysfunction,
and one third died. Only NMO cases full-filled the new NMOSD classification (52 AQP4-IgG
positive, 29 AQP4-IgG negative, and 41 AQP4-IgG unknown).
Conclusion
In Brazilian patients, OSMS and NMO are different immune-mediated diseases. OSMS is
a milder MS phenotype.
Keywords
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Article info
Publication history
Published online: April 13, 2020
Accepted:
March 29,
2020
Received in revised form:
February 19,
2020
Received:
November 26,
2019
Identification
Copyright
© 2020 Elsevier B.V. All rights reserved.
