Recent studies have provided conflicting data regarding the role of dyslipidemia in amyotrophic lateral sclerosis (ALS). The aim of this study was to determine whether cholesterol level are an independent predictor of survival in ALS.
Cholesterol levels were measured in 427 ALS subjects from three clinical trial databases.
The LDL/HDL ratio did not decrease over time, despite significant declines in body mass index (BMI), forced vital capacity (FVC), and ALSFRS-R. After adjusting for BMI, FVC, and age, the lipid ratio was not associated with survival. There was a "U"-shaped association between BMI and mortality, with the highest survival at 30-35 kg/m(2). The adjusted hazard ratio for the linear association between BMI and survival was 0.860 (95% CI 0.80-0.93, P = 0.0001).
We found that dyslipidemia is not an independent predictor of survival in ALS. BMI is an independent prognostic factor for survival after adjusting for markers of disease severity.
"The impact of altered metabolic balance on the course of disease is demonstrated by the finding that correcting for hypermetabolism improves survival in mouse models of ALS  and that dietary supplementation of ALS patients provides some benefit, possibly by maintaining body weight . Indeed, ALS patients with a BMI between 30 and 35 have better survival outcome , and a faster rate of reduction in BMI throughout the course of ALS is highly correlated with accelerated disease progression . Whole body metabolism is regulated by a network of proteins and peptides. "
"As a matter of fact, cholesterol and phospholipids are essential for axonal membrane health and for peripheral nerve membrane injury repairs, in particular low-density lipoproteins . Interestingly, there are some epidemiological data demonstrating that hyperlipidaemia is a significant prognostic factor for survivals in patients with ASL , but these results were not confirmed by Paganoni et al.  who showed a “U-”shaped association between BMI and mortality, with the highest survival in subjects with higher BMI (30–35); in this study dyslipidemia is not an independent predictor of survival in ALS. Wills et al.  have recently showed that patients which received a high caloric/high carbohydrate enteral nutrition had a smaller total number of adverse events and deaths than those of the high fat/high calorie group or the control group. "
[Show abstract][Hide abstract] ABSTRACT: An increasing number of data demonstrate the utility of ketogenic diets in a variety of metabolic diseases as obesity, metabolic syndrome, and diabetes. In regard to neurological disorders, ketogenic diet is recognized as an effective treatment for pharmacoresistant epilepsy but emerging data suggests that ketogenic diet could be also useful in amyotrophic lateral sclerosis, Alzheimer, Parkinson's disease, and some mitochondriopathies. Although these diseases have different pathogenesis and features, there are some common mechanisms that could explain the effects of ketogenic diets. These mechanisms are to provide an efficient source of energy for the treatment of certain types of neurodegenerative diseases characterized by focal brain hypometabolism; to decrease the oxidative damage associated with various kinds of metabolic stress; to increase the mitochondrial biogenesis pathways; and to take advantage of the capacity of ketones to bypass the defect in complex I activity implicated in some neurological diseases. These mechanisms will be discussed in this review.
BioMed Research International 07/2014; 2014(5). DOI:10.1155/2014/474296 · 3.17 Impact Factor
"Dorst et al., 2011 N = 427 High BMI correlates to longer survival. Paganoni et al., 2011 N = 77 Fast reduction of BMI predicts faster decline. Shimizu et al., 2012 N = 150 High BMI correlate to slower ALSFRS score decline. "
[Show abstract][Hide abstract] ABSTRACT: Motor neuron diseases (MNDs) are characterized by selective death of motor neurons and include mainly adult-onset amyotrophic lateral sclerosis (ALS) and spinal muscular atrophy (SMA). Neurodegeneration is not the single pathogenic event occurring during disease progression. There are multiple lines of evidence for the existence of defects in lipid metabolism at peripheral level. For instance, hypermetabolism is well characterized in ALS, and dyslipidemia correlates with better prognosis in patients. Lipid metabolism plays also a role in other MNDs. In SMA, misuse of lipids as energetic nutrients is described in patients and in related animal models. The composition of structural lipids in the central nervous system is modified, with repercussion on membrane fluidity and on cell signaling mediated by bioactive lipids. Here, we review the main epidemiologic and mechanistic findings that link alterations of lipid metabolism and motor neuron degeneration, and we discuss the rationale of targeting these modifications for therapeutic management of MNDs.
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