The natural history of ALS is changing: Improved survival

Abstract

In the past two decades new practice parameters for clinical care in ALS were developed and several clinical trials were performed. We sought to review information in these prospective datasets and assess whether natural history of ALS has changed over time. Survival and the rate of functional decline were compared across the placebo arms of efficacy trials conducted from 1999 to 2005 by the Northeast ALS Consortium (NEALS). Similar data from the placebo arms of 12 other published efficacy ALS trials conducted between 1990 and 2008 were compared descriptively. In the three NEALS clinical trials, survival improved over time in the placebo cohort (p=0.05) while the rate of change in maximum voluntary isometric contraction (MVIC) (p=0.15), ALS Functional Rating Scale (ALSFRS) (p=0.6) and vital capacity (%VC) (p=0.5) was unchanged. No differences were observed in the mean rates of decline of these outcome measures in the published clinical trials. However, survival improved in the more recently conducted trials. Survival improved over time in placebo controlled participants enrolled in clinical trials in ALS since 1990. However, the decline in measures of function appears unchanged since then. These changes in natural history reflect improvements in symptomatic care of ALS.

Riluzole, Neuroprotection and Amyotrophic Lateral Sclerosis

“…The use of such data, however, has received mixed reviews amongst the scientific community [152], given the evolving standards of ALS patient care, which have resulted in improved patient survival [153], as well as regional differences in clinical practice.…”

Meta-analysis of the relationship between amyotrophic lateral sclerosis and susceptibility to serum ferritin level elevation

“…There is a lack of research exploring the relationship between decreasing ALSFRS and %FVC and serum ferritin level elevation. Nemba et al22 showed us that lower BMI and serum insulin-like growth factor 1 (IGF-1) could increase serum ferritin levels in malnourished adolescents with eating disorders 1. Only one report13 picked up the BMI value, we found that the changes of BMI value have relationship with serum ferritin levels.…”

“…The elevated heterogeneity and publication bias may be explained by the study design, type of measure of association, geographic area, ferritin assay technique, %Bulbar onset, age at sample, ALSFRS, BMI, %FVC, non-invasive positive-pressure ventilation, oral riluzole, and enteral nutrition 1. Because serum ferritin is an acute-phase reactive protein that can be raised in the presence of acute or chronic inflammation, it changes according to the inflammation levels of ALS.…”

“…A myotrophic lateral sclerosis (ALS) is a neurodegenerative disorder caused by the loss of motor neurons leading to progressive bulbar, limb, and respiratory muscle weakness 1. Death generally occurs within 2-5 years after symptom onset, mostly due to respiratory failure 2.…”

The prognosis of motor neurone disease

“…It has recently been argued that people with motor neurone disease (MND) are living longer because of improvements in symptomatic care 1. However, this conclusion is based on an analysis of selected patients recruited into the placebo arm of trials.…”