Central nervous system idiopathic inflammatory demyelinating disorders in south americans: a descriptive, multicenter, cross-sectional study
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2015-07
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The idiopathic inflammatory demyelinating disease (IIDD) spectrum has been investigated
among different populations, and the results have indicated a low relative frequency of neuromyelitis optica (NMO) among multiple sclerosis (MS) cases in whites (1.2%-1.5%),
increasing in Mestizos (8%) and Africans (15.4%-27.5%) living in areas of low MS preva-
lence. South America (SA) was colonized by Europeans from the Iberian Peninsula, and
their miscegenation with natives and Africans slaves resulted in significant racial mixing.
The current study analyzed the IIDD spectrum in SA after accounting for the ethnic hetero-
geneity of its population. A cross-sectional multicenter study was performed. Only individu-
als followed in 2011 with a confirmed diagnosis of IIDD using new diagnostic criteria were
considered eligible. Patients’ demographic, clinical and laboratory data were collected. In
all, 1,917 individuals from 22 MS centers were included (73.7% female, 63.0% white, 28.0%
African, 7.0% Mestizo, and 0.2% Asian). The main disease categories and their associated
frequencies were MS (76.9%), NMO (11.8%), other NMO syndromes (6.5%), CIS (3.5%),
ADEM (1.0%), and acute encephalopathy (0.4%). Females predominated in all main cate-
gories. The white ethnicity also predominated, except in NMO. Except in ADEM, the dis-
ease onset occurred between 20 and 39 years old, early onset in 8.2% of all cases, and late
onset occurred in 8.9%. The long-term morbidity after a mean disease time of 9.28±7.7
years was characterized by mild disability in all categories except in NMO, which was
scored as moderate. Disease time among those with MS was positively correlated with the
expanded disability status scale (EDSS) score (r=0.374; p=<0.001). This correlation was
not observed in people with NMO or those with other NMO spectrum disorders (NMOSDs).
Among patients with NMO, 83.2% showed a relapsing-remitting course, and 16.8% showed
a monophasic course. The NMO-IgG antibody tested using indirect immunofluorescence
(IIF) with a composite substrate of mouse tissues in 200 NMOSD cases was positive in peo-
ple with NMO (95/162; 58.6%), longitudinally extensive transverse myelitis (10/30; 33.3%)
and bilateral or recurrent optic neuritis (8/8; 100%). No association of NMO-IgG antibody
positivity was found with gender, age at onset, ethnicity, early or late onset forms, disease
course, or long-term severe disability. The relative frequency of NMO among relapsing-
remitting MS (RRMS) + NMO cases in SA was 14.0%. Despite the high degree of miscege-
nation found in SA, MS affects three quarters of all patients with IIDD, mainly white young
women who share similar clinical characteristics to those in Western populations in the
northern hemisphere, with the exception of ethnicity; approximately one-third of all cases
occur among non-white individuals. At the last assessment, the majority of RRMS patients
showed mild disability, and the risk for secondary progression was significantly superior
among those of African ethnicity. NMO comprises 11.8% of all IIDD cases in SA, affecting
mostly young African-Brazilian women, evolving with a recurrent course and causing mod-
erate or severe disability in both ethnic groups. The South-North gradient with increasing
NMO and non-white individuals from Argentina, Paraguay, Brazil and Venezuela confirmed
previous studies showing a higher frequency of NMO among non-white populations.
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PAPAIS-ALVARENGA, Regina Maria et al. Central nervous system idiopathic inflammatory demyelinating disorders in south americans: a descriptive, multicenter, cross-sectional study. Plos One, San Francisco, v. 10, n. 7, p. e0127757, 2015.