Objectives: To report the clinical features and outcome of 24 Brazilian patients with optic neuromyelitis syndrome (ONM); discuss the underlying pathological events associated with the ONM syndrome; review the nosological situation of ONM in the group of inflammatory and demyelinating diseases of the central nervous system. Patients and Methods: Patients with ONM treated at the Hospital da Lagoa, Rio de Janeiro were studied. Demographic, clinical, magnetic resonance imaging, cerebrospinal fluid, and pathological data were analysed. Results: The study consisted of 20 women, four men of whom 10 were white and 14 Afro-Brazilians. Clinical course was recurrent in 22 cases and monophasic in two. Neurological manifestations at inclusion were: sensory impairment (66%), bilateral (41.6%) or unilateral blindness (20.8%), paraplegia or quadriplegia (37.5%). The EDSS was moderate/severe in 70.8%. The underlying pathological events were respectively pulmonary tuberculosis and upper respiratory infection in the two monophasic cases; in the 22 recurrent ONM patients: pulmonary tuberculosis (3), neurocysticercosis (1), polyarteritis nodosa (1), antinuclear antibody and rheumatoid factor (1), antiphospholipid antibody primary syndrome (1), diabetes mellitus (1), hypothyroidism (1), and amenorrhea-galactorrhea (4). Normal cerebrospinal fluid was found in 52% and an inflammatory profile in 48%. Only four recurrent ONM white patients had brain and spinal cord magnetic resonance imaging and cerebrospinal fluid findings compatible with the diagnosis of multiple sclerosis. Large lesions were seen in 62% of spinal magnetic resonance images. Six of 12 recurrent ONM Afro-Brazilian died. There were no statistical differences in the demographic data of the two ethnic groups. Afro-Brazilians were significantly more severely impaired and had a higher mortality rate than the white patients. Conclusion: These cases were classified as follows: two monophasic acute disseminated encephalomyelitis; one recurrent disseminated encephalomyelitis; three recurrent ONM associated with Hughes syndrome, autoantibodies and polyarteritis nodosa; six recurrent ONM with endocrinopathies; and finally, four muliple sclerosis cases. The remaining cases were not associated with any other condition. It would seem clear that ONM is a syndrome rather than a single disease.

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.

-A case-control study of Parkinson's disease (PD) was conducted in the city of Rio de Janeiro based on the assumption that neurotoxins with secondary parkinsonian action may be related to the development of Parkinson's 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 achieved 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.KEY WORDS: Parkinson's disease, genetics, chemical agents, drugs, environmental factors. Genética, medicamentos e fatores do meio ambiente na doença de ParkinsonRESUMO -Um estudo caso-controle foi realizado na cidade do Rio de Janeiro, partindo-se do pressuposto de que neurotoxinas com ação parkinsoniana secundária poderiam facilitar a ocorrência da doença de Parkinson. Noventa e dois pacientes com doença de Parkinson e 110 controles foram avaliados através de questionário para a investigação de possíveis fatores de risco para a doença. Foram pesquisados os seguintes fatores: herbicidas e pesticidas, exposição a agentes químicos, uso de medicamentos com ação parkinsoniana secundária, vida rural, ingestão de água de poço, história familiar, passado de trauma central e consumo de cigarro. O estudo destes fatores foi realizado através das análises univariada, estratificada e multivariada. As análises univariada e multivariada demonstraram que a história familiar (RC = 14,5; IC = 2,38), o uso de medicamentos (RC = 11,01; IC = 3,41) e a exposição a agentes químicos (RC = 5,87; IC = 1,23) eram positivos como fatores de risco para a doença. Papel inverso foi ocupado pelo consumo de cigarros (RC = 0,39; IC = 0,16 -0,95). A análise estratificada confirmou apenas história familiar e medicamentos como fatores de risco, além de demonstrar que o consumo de cigarros poderia ser um fator de proteção, quando houve alguma participação destes mesmos fatores. Este estudo talvez possa alertar quanto aos cuidados que devem ser tomados na utilização de drogas e na atividade ocupacional com agentes químicos, quando estes...

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...

-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.KEY WORDS: multiple sclerosis, risk factors, immunization, tobacco, single person, consumption of animal brain, Brazil.Potenciais fatores de risco para o desenvolvimento de esclerose múltipla no Rio de Janeiro: um estudo caso controle Resumo -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%=2,3-115,2), fumantes (OR=7,6; IC95%=2,2), solteiros (OR=4,7; IC95%=1,(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)6) e que consumiam cérebro de animal (OR=3,4; IC95%=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. PALAVRAS-CHAVE: esclerose múltipla, fatores de risco, epidemiologia, Brasil.