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Central Nervous System Idiopathic Inflammatory Demyelinating Disorders in South Americans: A Descriptive, Multicenter, Cross-Sectional Study.

Papais-Alvarenga RM, Vasconcelos CC, Carra A, de Castillo IS, Florentin S, Diaz de Bedoya FH, Mandler R, de Siervi LC, Pimentel ML, Alvarenga MP, Alvarenga MP, Grzesiuk AK, Gama Pereira AB, Gomes Neto AP, Velasquez C, Soublette C, Fleitas CV, Diniz DS, Armas E, Batista E, Hernandez F, Pereira FF, Siqueira HH, Cabeça H, Sanchez J, Brooks JB, Gonçalves MV, Barroso MC, Ravelo ME, Castillo MC, Ferreira ML, Rocha MS, Parolin MK, Molina O, Marinho PB, Christo PP, Brant de Souza R, Pessanha Neto S, Camargo SM, Machado SC, Neri VC, Fragoso YD, Alvarenga H, Thuler LC - PLoS ONE (2015)

Bottom Line: South America (SA) was colonized by Europeans from the Iberian Peninsula, and their miscegenation with natives and Africans slaves resulted in significant racial mixing.Despite the high degree of miscegenation 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.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology, Universidade Federal do Estado do Rio de Janeiro, Rio de Janeiro, Brazil; Department of Neurology, Hospital Federal da Lagoa, Ministério da Saúde, Rio de Janeiro, Brazil; Clinica de Neuroimunologia, Rio de Janeiro, Brazil.

ABSTRACT
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 prevalence. 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 heterogeneity of its population. A cross-sectional multicenter study was performed. Only individuals 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 categories. The white ethnicity also predominated, except in NMO. Except in ADEM, the disease 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 people 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 miscegenation 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 moderate 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.

No MeSH data available.


Related in: MedlinePlus

The IIDD spectrum in SA by ethnicity.IIDD = inflammatory idiopathic demyelinating disease, MS = multiple sclerosis; NMO = neuromyelitis optica; NMOSD = NMO syndrome; CIS = clinical isolated syndrome; ADEM = acute disseminated encephalomyelitis; acute IIDD with encephalopathy;
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pone.0127757.g001: The IIDD spectrum in SA by ethnicity.IIDD = inflammatory idiopathic demyelinating disease, MS = multiple sclerosis; NMO = neuromyelitis optica; NMOSD = NMO syndrome; CIS = clinical isolated syndrome; ADEM = acute disseminated encephalomyelitis; acute IIDD with encephalopathy;

Mentions: Fig 1 shows the distribution of the six major categories of IIDD by ethnicity.


Central Nervous System Idiopathic Inflammatory Demyelinating Disorders in South Americans: A Descriptive, Multicenter, Cross-Sectional Study.

Papais-Alvarenga RM, Vasconcelos CC, Carra A, de Castillo IS, Florentin S, Diaz de Bedoya FH, Mandler R, de Siervi LC, Pimentel ML, Alvarenga MP, Alvarenga MP, Grzesiuk AK, Gama Pereira AB, Gomes Neto AP, Velasquez C, Soublette C, Fleitas CV, Diniz DS, Armas E, Batista E, Hernandez F, Pereira FF, Siqueira HH, Cabeça H, Sanchez J, Brooks JB, Gonçalves MV, Barroso MC, Ravelo ME, Castillo MC, Ferreira ML, Rocha MS, Parolin MK, Molina O, Marinho PB, Christo PP, Brant de Souza R, Pessanha Neto S, Camargo SM, Machado SC, Neri VC, Fragoso YD, Alvarenga H, Thuler LC - PLoS ONE (2015)

The IIDD spectrum in SA by ethnicity.IIDD = inflammatory idiopathic demyelinating disease, MS = multiple sclerosis; NMO = neuromyelitis optica; NMOSD = NMO syndrome; CIS = clinical isolated syndrome; ADEM = acute disseminated encephalomyelitis; acute IIDD with encephalopathy;
© Copyright Policy
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=PMC4519274&req=5

pone.0127757.g001: The IIDD spectrum in SA by ethnicity.IIDD = inflammatory idiopathic demyelinating disease, MS = multiple sclerosis; NMO = neuromyelitis optica; NMOSD = NMO syndrome; CIS = clinical isolated syndrome; ADEM = acute disseminated encephalomyelitis; acute IIDD with encephalopathy;
Mentions: Fig 1 shows the distribution of the six major categories of IIDD by ethnicity.

Bottom Line: South America (SA) was colonized by Europeans from the Iberian Peninsula, and their miscegenation with natives and Africans slaves resulted in significant racial mixing.Despite the high degree of miscegenation 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.

View Article: PubMed Central - PubMed

Affiliation: Department of Neurology, Universidade Federal do Estado do Rio de Janeiro, Rio de Janeiro, Brazil; Department of Neurology, Hospital Federal da Lagoa, Ministério da Saúde, Rio de Janeiro, Brazil; Clinica de Neuroimunologia, Rio de Janeiro, Brazil.

ABSTRACT
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 prevalence. 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 heterogeneity of its population. A cross-sectional multicenter study was performed. Only individuals 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 categories. The white ethnicity also predominated, except in NMO. Except in ADEM, the disease 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 people 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 miscegenation 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 moderate 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.

No MeSH data available.


Related in: MedlinePlus