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

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.

The impact of diagnostic criteria for neuromyelitis optica in patients with MS: a 10-year follow-up of the South Atlantic Project.

BACKGROUND: It is recognized that there is a particular geographic and ethnic distribution of neuromyelitis optica (NMO) among Caucasian and non-Caucasian populations. OBJECTIVE: To review the diagnoses of patients whom were enrolled in the South Atlantic Project, a Brazilian multiple sclerosis (MS) survey performed from 1995-1998, and to identify NMO and MS case frequencies. METHODS: We reviewed the data from a 10-year follow-up of MS patients. To apply the current diagnostic criteria, the neurologists were asked to collect clinical and laboratory data from the medical records of study patients treated from 1999-2009. RESULTS: The spectrum of inflammatory demyelinating disease in 322 patients (67% white; 33% African-Brazilian) was: 49 (15%) with NMO; 14 (4%) with NMO syndromes; 10 (3%) with acute disseminated encephalomyelitis (ADEM); one isolated tumefactive brain lesion; 249 (77%) with MS (151 with relapsing-remitting MS (RRMS), 70 with secondary progressive MS (SPMS) and 27 with primary progressive MS (PPMS)). Disability was more severe in NMO and PPMS. One-third of the NMO patients had died. CONCLUSIONS: The frequency of NMO was 6.8% in São Paulo and 20.5% in Rio de Janeiro, and mainly seen in persons of African descent, which strengthens the hypothesis of there being an ethnic association of this disease. We recommend that epidemiological studies on MS that were performed previously be reviewed again, to ensure more accurate diagnoses.

[Stroke prevalence among the elderly in Vassouras, Rio de Janeiro State, Brazil, according to data from the Family Health Program]. / Prevalência de acidente vascular cerebral em idosos no Município de Vassouras, Rio de Janeiro, Brasil, através do rastreamento de dados do Programa Saúde da Família.

This study estimated the prevalence of stroke among the elderly in Vassouras, Rio de Janeiro State, Brazil, based on data from the Family Health Program (FHP). The elderly population was chosen since it is growing as a proportion of the general population, and since stroke risk increases with age. Data were screened for all the elderly registered in the FHP in Vassouras, identifying those with a history of stroke and analyzing their socio-demographic profile. The study used data from the Information System on Primary Care, the population census conducted by the Brazilian Institute of Geography and Statistics (IBGE), and the Ministry of Health's standardized FHP patient form. Quality of stroke diagnoses in the FHP was analyzed. Data screening detected 122 elderly with a history of stroke diagnosis (prevalence = 2.9%; 3.2% in men, 2.7% in women) and a progressive increase with age. The prevalence rate was the same in the rural and urban area of the municipality (2.9%). Knowledge of stroke prevalence in the elderly population is essential to improve health planning.

Prevalência de acidente vascular cerebral em idosos no Município de Vassouras, Rio de Janeiro, Brasil, através do rastreamento de dados do Programa Saúde da Família / Stroke prevalence among the elderly in Vassouras, Rio de Janeiro State, Brazil, according to data from the Family Health Program

O estudo estimou a prevalência de acidente vascular cerebral (AVC) em idosos em Vassouras, Rio de Janeiro, Brasil, pelo rastreamento de dados do Programa Saúde da Família (PSF). A população idosa foi escolhida por seu aumento no Brasil e pelo risco do AVC aumentar com a idade. Foram rastreados todos os idosos de Vassouras cadastrados no PSF, identificando os acometidos por AVC e analisando o seu perfil sócio-demográfico. Foram utilizados os dados do Sistema de Informação e Atenção Básica, do censo populacional do Instituto Brasileiro de Geografia e Estatística e a ficha de atendimento padronizada pelo PSF, do Ministério da Saúde. Avaliou-se a qualidade dos diagnósticos de AVC do PSF. No rastreamento, foram encontrados 122 idosos com diagnóstico de AVC, com prevalência de 2,9 por cento, e aumento progressivo com o avançar da idade, sendo a prevalência nos homens (3,2 por cento) maior do que nas mulheres (2,7 por cento). A taxa de prevalência foi igual tanto na zona rural quanto na zona urbana (2,9 por cento). O conhecimento da magnitude da prevalência do AVC na população idosa é fundamental para melhor planejamento de saúde.

This study estimated the prevalence of stroke among the elderly in Vassouras, Rio de Janeiro State, Brazil, based on data from the Family Health Program (FHP). The elderly population was chosen since it is growing as a proportion of the general population, and since stroke risk increases with age. Data were screened for all the elderly registered in the FHP in Vassouras, identifying those with a history of stroke and analyzing their socio-demographic profile. The study used data from the Information System on Primary Care, the population census conducted by the Brazilian Institute of Geography and Statistics (IBGE), and the Ministry of Health's standardized FHP patient form. Quality of stroke diagnoses in the FHP was analyzed. Data screening detected 122 elderly with a history of stroke diagnosis (prevalence = 2.9 percent; 3.2 percent in men, 2.7 percent in women) and a progressive increase with age. The prevalence rate was the same in the rural and urban area of the municipality (2.9 percent). Knowledge of stroke prevalence in the elderly population is essential to improve health planning.

Potential risk factors for multiple sclerosis in Rio de Janeiro: a case-control study.

PURPOSE: To evaluate potential risk factors for the development of multiple sclerosis in Brazilian patients. METHOD: A case control study was carried out in 81 patients enrolled at the Department of Neurology of the Hospital da Lagoa in Rio de Janeiro, and 81 paired controls. A standardized questionnaire on demographic, social and cultural variables, and medical and family history was used. Statistical analysis was performed using descriptive statistics and conditional logistic regression models with the SPSS for Windows software program. RESULTS: Having standard vaccinations (vaccinations specified by the Brazilian government) (OR=16.2; 95% CI=2.3-115.2), smoking (OR=7.6; 95% CI=2.1-28.2), being single (OR=4.7; 95% CI=1.4-15.6) and eating animal brain (OR=3.4; 95% CI=1.2-9.8) increased the risk of developing MS. CONCLUSIONS: RESULTS of this study may contribute towards better awareness of the epidemiological characteristics of Brazilian patients with multiple sclerosis.

Potential risk factors for multiple sclerosis in Rio de Janeiro: a case-control study / Potenciais fatores de risco para o desenvolvimento de esclerose múltipla no Rio de Janeiro: um estudo caso controle

PURPOSE: To evaluate potential risk factors for the development of multiple sclerosis in Brazilian patients. METHOD: A case control study was carried out in 81 patients enrolled at the Department of Neurology of the Hospital da Lagoa in Rio de Janeiro, and 81 paired controls. A standardized questionnaire on demographic, social and cultural variables, and medical and family history was used. Statistical analysis was performed using descriptive statistics and conditional logistic regression models with the SPSS for Windows software program. RESULTS: Having standard vaccinations (vaccinations specified by the Brazilian government) (OR=16.2; 95 percent CI=2.3-115.2), smoking (OR=7.6; 95 percent CI=2.1-28.2), being single (OR=4.7; 95 percent CI=1.4-15.6) and eating animal brain (OR=3.4; 95 percent CI=1.2-9.8) increased the risk of developing MS. CONCLUSIONS: RESULTS of this study may contribute towards better awareness of the epidemiological characteristics of Brazilian patients with multiple sclerosis.

OBJETIVO: Avaliar os potenciais fatores de risco para o desenvolvimento de esclerose múltipla em pacientes brasileiros. MÉTODO: Um estudo caso-controle incluiu 81 pacientes atendidos no Departamento de Neurologia do Hospital da Lagoa, no Rio de Janeiro, e 81 controles. Um questionário padronizado incluiu variáveis demográficas, sociais e culturais, além da história familiar e clínica. A análise dos dados foi realizada por meio do programa SPSS para Windows e foi constituída de estatísticas descritivas e de um modelo de regressão logística condicional. RESULTADOS: Pacientes com história de imunização (OR=16,2; IC95 por cento=2,3-115,2), fumantes (OR=7,6; IC95 por cento=2,1-28,2), solteiros (OR=4,7; IC95 por cento=1,4-15,6) e que consumiam cérebro de animal (OR=3,4; IC95 por cento=1,2-9,8) tiveram risco mais elevado de desenvolver esclerose múltipla quando comparados aos controles. CONCLUSÃO: Os resultados deste estudo podem contribuir para um melhor entendimento das características epidemiológicas dos pacientes brasileiros com esclerose múltipla.

Modulaçäo autonômica cardíaca na doença de Parkinson / Cardiac autonomic modulation in Parkinson`s disease

O presente trabalho analisou as alteraçöes da variabilidade da frequência cardíaca (V-RR) nos pacientes com Doença de Parkinson Idiopática (DPI). Foram estudados 67 pacientes (Grupo I) comprovadamente normais e 32 com DPI (Grupo II). No Grupo I, a idade variou de 40 a 85 anos com média de 69,2 ñ 10,8 anos e 40 pertenciam ao sexo masculino. No Grupo II, a idade variou de 40 a 81 anos com média de 65,3 ñ 11,3 anos e 21 pertenciam ao sexo masculino (p=NS. Todos os pacientes do Grupo II tinham sinais e sintomas de DPI, 11 (34 por cento) tinham hipertensäo arterial (HA), leve a moderada, nenhum possuía doença coronária e diabetes mellitus. A DPI foi estadiada segundo a escala de Hoenh e Yahr, verificando-se o tempo de evoluçäo. Os sinais do ECG foram captados em período de 300 s, com o paciente em decúbito dorsal e respirando em sua frequência natural. Na análise da V-RR foram empregadas as variáveis do domínio do tempo (DT), representada pelo desvio-padräo (DPNN) de todos intervalos RR e pela diferença entre a bradi e a taquicardia máxima (D-BTmáx) durante a inspiraçäo sustentada. No domínio da frequência (DF) foram analisadas as bandas de alta frequência (AF) e as de baixa frequência (BF), do espectro de potência. A banda de AF foi também estudada após um período de um minuto de respiraçäo controlada (RC). Do ponto de vista estatístico o nível de significância foi fixado em 0,05. As variáveis do DF foram transformadas em seus logaritmos naturais antes da análise estatística. Tanto no DT quanto no DF os valores das variáveis analisadas foram significativamente menores no Grupo II que no Grupo I. Estas diferenças foram mais expressivas nos pacientes do Grupo II com idade maior ou igual a 65 anos. O estádio da doença, o tempo de evoluçäo e a presença de HA näo influenciaram, de forma significativa, na V-RR. A V-RR mostrou-se como importante meio para avaliar a açäo do sistema nervoso autonômico dos batimentos cardíacos nos pacientes com DPI e evidenciou que este sistema sofre perdas significativas, as quais säo mais intensas nos pacientes com idade maior ou igual a 65 anos. É provável que esta perda possa influenciar no prognóstico da DPI, em especial nos mais idosos

Genetics, drugs and environmental factors in Parkinson's disease: a case-control study

A case-control study of Parkinsonïs disease (PD) was conducted in the city of Rio de Janeiro based on the asumption that neurotoxins with secondary parkinsonian action may be related to the development of Parkinsonïs disease. Ninety-two subjects with PD and 110 controls were queried through a questionnaire in order to investigate possible risk factors for the disease. The following factors were studied: herbicides/pesticides, exposure to chemicals, ingestion of drugs with secondary PD effects, rural life, water well source, family history, cranial trauma and cigarette smoking. Study of mentioned factors was archieved through univariate, stratified and multivariate analyses. Univariate and multivariate analyses demonstrated that PD was positively associated with family history (OR=14.5; CI-2,98 - 91.38), with the use of drugs with secondary PD action (OR=11.01; CI=3.41 - 39.41) and with exposure to chemical agents (OR= 5.87; CI=1.48 - 27.23). PD was found to be inversely associated with cigarette smoking (OR=0.39; IC=0.16 - 0.95). Stratified analysis only confirmed family history and drug use, besides demonstrating that cigarette consumption could be a protection factor, when aforementioned factors were involved. This study might be a warning as to the cares that need to be taken regarding drug use and occupational exposure to chemical agents, as both types of substances present secondary PD action.

Esclerose múltipla: manifestaçöes clínicas, aspectos epidemiológicos e critérios diagnósticos / Multiple sclerosis: clinical manifestations, epidemiological aspects and diagnosis criteria

Os autores dissertam sobre as manifestaçöes clínicas da esclerose múltipla a partir da revisäo histórica dos trabalhos originais de Jean Charcot e seus discípulos que, na segunda metade do século passado, descreveram as bases de diagnóstico clínico desta enfermidade. Apresentam súmula das principais investigaçöes epidemiológicas que demonstram uma distribuiçäo geográfica única, com áreas de alta prevalência nos países frios no hemisfério norte e tem contribuído para o conhecimento da evoluçäo natural desta doença crônica do SNC. Finalmente apresentam a metodologia de análise clínica proposta por Kurtzke através do FC/EDSS e os protocolos de estudo criados com o objetivo de estabelecer procedimentos uniformes de diagnóstico clínico com especial ênfase ao de Poser, 1983, que vem sendo adotado nas pesquisas realizadas no Brasil

Avanços no entendimento da demência de Alzheimer / Development in Alzheimer's disease understanding

O autor detém em dois aspectos proeminentes da investigaçöes que tentam esclarecer a complexa patogenia desta enfermidade os achados neuropatológicos e as hipóteses etiológicas mais valorizadas no momento