Epidemiology of the amyotrophic lateral sclerosis Bahia: Brazil

The Amyotrophic Lateral Sclerosis is a neurological disorder, with the degeneration of the upper and lower motor neurons. The aim is investigate the start of the symptoms, describe the findings and study the survival period of patients with ALS. We analyzed 70 patients. The patients’ average age was 49.68 years old and we found 43 patients (61.4 percent) who were white, 22 who were grayish brown (31.4 percent) and 5 who were black (7.1 percent). Regarding the start of the symptoms, 51 patients (72.9 percent) showed a distal start, 31 a proximal one (44.3 percent) and 8 of them (11.4 percent) showed a bulbar start. The survival period, after de diagnosis, was of 64.11 months. The mean age, signs and symptoms and the patients’ survival period we found, are compatible with the ones found in the literature, except for the number of black patients, that was bigger in our survey.

A esclerose lateral amiotrófica (ELA) é uma desordem neurológica com degeneração dos neurônios motores superiores e inferiores. Objetivo: investigar o inicio dos sintomas, descrever a evolução e os achados neurológicos e estudar a sobrevida dos pacientes com ELA. Método: Analisamos 70 pacientes entre 1996 e 2007, que preencheram os critérios propostos no El Escorial, sendo 52 do sexo masculino e 18 do sexo feminino. A média de idade dos pacientes era de 49,6857 anos, encontramos 43 (61,4 por cento) brancos, 22 (31,4 por cento) pardos e 5 (7,1 por cento) negros. Quanto ao inicio dos sintomas, 51 (72,9 por cento) dos pacientes apresentaram inicio distal, 31 (44,3 por cento) de forma proximal e 8 (11,4 por cento) de forma bulbar. Os sintomas mais comuns foram dos fraqueza muscular, atrofia muscular e miofasciculações presente em 69 (98,6 por cento) pacientes. A sobrevida após diagnóstico foi de 64,116 meses. Conclusão: A idade média, os sinais e sintomas e a sobrevida dos pacientes analisados são compatíveis com os encontrados na literatura, exceto pela quantidade de pacientes da raça negra, que foi maior.

Evolution of HTLV-1 proviral load in patients from Salvador, Brazil

INTRODUCTION: Variations in human T cell lymphotropic virus type 1 (HTLV-1) proviral load (PVL) in infected individuals over time are not well understood. Objective: To evaluate the evolution of proviral load in asymptomatic individuals and HAM/TSP patients in order to help determine periodicity for measuring proviral load. METHODS: A group of 104 HTLV-1 infected patients, followed at the HTLV reference center in Salvador, Brazil, were included in the study (70 asymptomatic and 34 HTLV-1-associated myelopathy/tropical spastic paraparesis (HAM/TSP) patients). HTLV-1 PVL was measured using real-time polymerase chain reaction (PCR) at baseline and again at another point, either < 12 months, between 12-24 months, or > 24 months. RESULTS: HAM/TSP patients had higher PVL (ranging from 11,041 to 317,009 copies/10(6) PBMC) when compared to asymptomatic individuals (ranging from 0 to 68,228 copies/10(6) PBMC). No statistically significant differences were observed in the medians of PVL in HAM/TSP patients or asymptomatic individuals over time. However, in asymptomatic individuals with a PVL below 50,000 copies/10(6) PBMC, a statistically significant two-fold increase was observed over time. CONCLUSION: HTLV-1-PVL remained stable in both asymptomatic individuals and HAM/TSP patients over time. Frequent monitoring of asymptomatic individuals with low PVLs is recommended and further studies should be conducted to assess the course of PVL in these patients over extended periods of time.

Comparison between the spastic paraplegia rating scale, Kurtzke scale, and Osame scale in the tropical spastic paraparesis/myelopathy associated with HTLV / Comparação entre a escala para paraplegia espástica, escala de Kurtzke e escala de Osame, na paraparesia epástica tropical/mielopatia associada ao HTLV

INTRODUCTION:The objective of this study was to compare Osame's scale of motor incapacity and the expanded scale of the state of incapacity of Kurtzke with the spastic paraplegia rating scale for the clinical evaluation of patients with HTLV-I-associated myelopathy/tropical spastic paraparesis (HAM/TSP). METHODS: Patients with the diagnosis of infection by HTLV-I/HTLV-II and with the clinical suspicion of HAM/TSP were included in the study. RESULTS: There were 45 patients who were evaluated. When analyzing the results of the scales, the researchers found the following averages of 21.08 points for the spastic paraplegia rating scale, 4.35 points for Osame's scale, and 4.77 points for Kurtzke's scale. The relation between the scale of paraplegia with Osame's was very significant with p < 0.0001, and regarding Kurtzke's scale, there was a similar result of p < 0.0001. When comparing Osame's, Kurtze's, and the spastic paraplegia rating scale with the time of disease, the researchers found a significant result of p = 0.0004 for the scale of spastic paraplegia, p = 0.0018 for Osame's scale, and p < 0.0001 for Kurtzke's scale. CONCLUSION: The spastic paraplegia rating scale has a good relation with Osame's and Kurtzke's scales showing a p index that is very significant that indicates that, although the scale was not initially made to be applied to patients with HAM/TSP because of the infection by HLTV, it showed to be as efficient as Osame's and Kurtzke's scales in evaluating the patients' neurological conditions.

INTRODUÇÃO:O objetivo deste estudo foi comparar a escala de incapacidade motora de Osame e a escala expandida do estado de incapacidade de Kurtzke com a escala para avaliação da paraplegia espástica com o objetivo de avaliar a clinica dos pacientes com mielopatia associada a HTLV-I/paraparesia espastica tropical (PET/MAH). MÉTODOS: Foram incluídos pacientes com diagnóstico de infecção pelo HTLV-I/HTLV-II e a suspeita clinica de PET/MAH. RESULTADOS: Foram avaliados 45 pacientes. Ao analisar os resultados das escalas encontramos as seguintes médias de 21,08 pontos para a escala para paraplegia espástica, 4,35 pontos para a escala de Osame e 4,77 pontos para a de Kurtze. A relação entre a escala de paraplegia com a de Osame foi muito significativa com p < 0.0001, e com relação a escala de Kurtze obteve resultado semelhante com p < 0.0001, também significante. Comparando-se as escalas de Osame, Kurztze e escala para avaliação da paraplegia espástica com o tempo de doença obtivemos um resultado significante com p=0,0004, para a escala de paraplegia espastica, p = 0,0018 para a escala de Osame e p < 0,0001 para a escala de Kurtzke. CONCLUSÕES: A escala da paraplegia espástica possui boa relação com as escalas de Osame e Kurzte, mostrando um p muito significativo, indicando que apesar da escala não ter sido feita inicialmente para ser aplicada aos pacientes com PET/MAH devido à infecção pelo HLTV, ela se mostrou capaz de ser tão eficiente quanto às escalas de Osame e Kurtzke para avaliar o quadro neurológico dos pacientes.