This subproject is one of many research subprojects utilizing the resources provided by a Center grant funded by NIH/NCRR. The subproject and investigator (PI) may have received primary funding from another NIH source, and thus could be represented in other CRISP entries. The institution listed is for the Center, which is not necessarily the institution for the investigator. Malnutrition due to caloric deficiency is very prevalent in amyotrophic lateral sclerosis (ALS) during the course of the disease. Profound weight loss, muscle weakness and muscle atrophy are prominent features which produce progressive disability and early death. Nutritional compromise necessitates gastrostomy and carries with it an adverse influence on survival. Since the precise cause(s) of malnutrition in ALS has not been adequately studied using modern technology, it is unknown whether caloric deficiency is due solely to inadequate intake, or could be due to increased expenditure of calories, or both of these mechanisms. Further, although gastrostomy is frequently recommended to ALS patients who are believed to be malnourished, guidelines on neither the degree of caloric deficiency requiring gastrostomy nor the amount of caloric replacement to correct the malnutrition after gastrostomy are forthcoming. The overall goal of this proposal is to examine caloric requirements in patients with ALS to identify caloric deficiency early in the course of their disease, plan for timely nutritional intervention, and predict the need for gastrostomy by objective nutritional criteria rather than simply by the presence of dysphagia. The hypothesis in this proposal is that relative caloric deficiency in ALS is almost universal in the symptomatic stages of the disease, is due to a combination of decreased daily intake and increased daily expenditure of calories, and will be evident by comparing data from patients and matched normal controls. To test this hypothesis we propose to undertake the following Specific Aims: 1) Estimate daily caloric intake and expenditure in ALS patients and compare values with those in age- and gender-matched healthy controls from our database (cross-sectional analysis), and 2) Measure daily caloric intake and expenditure in ALS patients periodically over 12 months to examine the effects of progressively increasing disease severity and changes in body composition and physical activity level on caloric requirements (longitudinal analysis).
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