ObjectiveTo determine the prognostic factors associated with survival in amyotrophic lateral sclerosis at diagnosis.MethodsThis retrospective population-based study evaluated 218 patients treated with riluzole between 2005 and 2014 and described their clinical and demographic profiles after the analysis of clinical data and records from the mortality information system in the Federal District, Brazil. Cox multivariate regression analysis was conducted for the parameters found.ResultsThe study sample consisted of 132 men and 86 women with a mean age at disease onset of 57.2±12.3 years; 77.6% of them were Caucasian. The mean periods between disease onset and diagnosis were 22.7 months among men and 23.5 months among women, and the mean survival periods were 45.7±47.0 months among men and 39.3±29.8 months among women. In addition, 80.3% patients presented non-bulbar-onset amyotrophic lateral sclerosis, and 19.7% presented bulbar-onset. Cox regression analysis indicated worse prognosis for body mass index (BMI) <25 kg/m2 (relative risk [RR]: 3.56, 95% confidence interval [CI]: 1.44–8.86), age >75 years (RR: 12.47, 95% CI: 3.51–44.26), and bulbar-onset (RR: 4.56, 95% CI: 2.06–10.12). Electromyography did not confirm the diagnosis in 55.6% of the suspected cases and in 27.9% of the bulbar-onset cases.ConclusionsThe factors associated with lower survival in amyotrophic lateral sclerosis were age >75 years, BMI <25 kg/m2, and bulbar-onset.

BackgroundPublished studies seeking to improve survival in amyotrophic lateral sclerosis (ALS) have poor results in humans, although there are several studies in animal models with positive results.MethodsWe conducted a systematic review and meta-analysis of studies that were published between March 2009 and March 2015 on stem cell therapy and survival in animal models and patients with ALS. A total of 714 articles were identified, and from these, we selected preclinical in vivo studies and retrospective clinical studies.Results and conclusionsA meta-analysis confirmed the efficacy of stem cell therapy in improving survival in preclinical trials, where a mean difference of 9.79 days (95% confidence interval: 4.45 - 15.14) in lifespan favored stem cell therapy. In contrast, the number of clinical studies is still insufficient to assess their effectiveness, and these studies only demonstrate the absence of serious adverse events. However, even this conclusion should be interpreted with caution because clinical studies are retrospective and heterogeneous and have an unsatisfactory quality.

The results of published studies with various neuroprotectors seeking to preserve motor neuron function and improve survival in amyotrophic lateral sclerosis patients have poor evidence in humans, although there are several studies in animal models with positive results. A systematic review and meta-analysis of studies on drug treatment options and survival times in animal models and patients with amyotrophic lateral sclerosis from March, 2009 to March, 2015 was conducted. Four hundred eighty-nine (489) articles were found, and from these, we selected 30 preclinical 'in vivo' studies, 18 randomized controlled trials, and four systematic reviews. A meta-analysis confirmed the effectiveness of various drugs in improving the life span in preclinical trials, in particular, Resveratrol, which had a mean difference of 10.8 days (95% CI: 9.57 to 12.02), whereas no drug showed efficacy in clinical trials. The positive results of preclinical studies should be interpreted with caution because there is a mismatch between those results and the negative results in clinical trials.

There is evidence that multidisciplinary care improves quality of life and there is a growing appreciation of public policies in Brazil that favor home care with a multidisciplinary team in chronic diseases. This study aimed to determine the epidemiological profile of amyotrophic lateral sclerosis in the Federal District and the impact of tertiary reference center creation on patient care. A descriptive, crosssectional study analyzing secondary data regarding mortality and hospitalizations for amyotrophic lateral sclerosis patients over 10 years and clinical and epidemiological profiles of patients evaluated at the Center of Reference for Neuromuscular Disease over three years was used. An incidence rate of 1.3/100,000 person-years over 20 years and an age at onset of 49.3 ± 15.1 years (Hospital Information System) and 57.2 ± 12.3 years (at the Center admission) was observed. The risk of death was greater in patients older than 75 years (RR = 4.05, p = 0.0018) and in bulbar-onset patients (RR = 2.53, p = 0.0027). Multidisciplinary care reduced 75% of hospitalization frequency and length of stay (p = 0.03) and 80 to 90% of the reimbursement value of hospitalization (p = 0.05). The adoption of multidisciplinary care has improved the efficiency of patient care for amyotrophic lateral sclerosis in the Brazilian public health system.