Should patients with ALS gain weight during their follow-up?

Abstract

a b s t r a c tIt was recently postulated that a nutritional intervention aiming at achieving weight gain might increase survival in ALS patients. This article discusses the effect of nutritional status and weight gain on survival, respiratory status and physical function. Based on the available literature, it remains unknown whether weight gain during the progression of the disease improves survival whatever the baseline body weight is. A high body mass index may impair respiratory muscle function and passive mobilization of paretic patients. Future research should evaluate the effect of changes in weight and body composition on clinical outcome while taking into account respiratory muscle strength and physical function.Ó 2015 Elsevier Inc. All rights reserved.Amyotrophic lateral sclerosis (ALS) is a neurodegenerative disease with a life expectancy of 2 to 5 y. Effective treatment for ALS is lacking. A multidisciplinary approach [1], non-invasive ventilation [2], riluzole, and enteral nutrition via percutaneous endoscopic gastrostomy have been shown to improve survival and quality of life to some extent [1]. In a recently published article, it was postulated that a nutritional intervention, aiming at achieving weight gain, might be a therapeutic option for patients with ALS [3]. These assumptions relied on the increased survival with a high-calorie diet in an ALS mouse model [4] and on the lower risk for ALS in overweight or obese individuals [5].Here we summarize the effect of initial nutritional status and weight gain during follow-up on survival and respiratory and physical function to explore whether weight gain is beneficial for patients with ALS.Weight and body mass index at diagnosis and survival A study of 92 patients with ALS demonstrated that a history of weight loss at diagnosis was significantly associated with survival [7]. After adjustment for age, sex, bulbar versus non-bulbar onset, ALS functional rating scale (ALSFRS), manual muscular testing, forced vital capacity, and diagnostic delay, the study found a 30% increase in risk for death for each 5% weight loss and a 20% increase in risk for death for each BMI unit loss.In the same study population, undernutrition at diagnosis, defined as a BMI <18.5 kg/m 2 if <70 y or <21 kg/m 2 if 70 y (n ¼ 8), was not associated with survival, after the aforementioned adjustments [7]. Effect of obesity was not assessed. A larger study stratified 285 patients with ALS according to BMI categories at baseline [8]. BMI categories were not predictive of survival or rate of progression. However, only 3 patients were undernourished and 65 were obese. A shorter survival in patients with ALS and a baseline BMI <18.5 kg/m 2 (n ¼ 9) than in those with a higher BMI was reported in univariate Cox regressions [9]. One group of researchers studied 427 patients with ALS [10]. A decreased survival was seen in patients with a BMI >35 or <18.5 kg/m 2 at diagnosis; thus a U-shaped relationship between BMI and survival was established. This finding was confirmed in the PRO-ACT (Po...

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“…Control of symptoms related to respiratory muscle weakness and/or sleep-related hypoventilation or SRBD are per se an indication for NIV (e.g., morning headaches and fatigue, orthopnea, resting dyspnea, severe recruitment of accessory muscles, unexplained weight loss), even in the absence of daytime hypercapnia [181-183] .…”

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“…However, despite the notion that increased calorie intake compensates for increased energy demands and improves outcome in ALS, the exact composition of dietary intervention and when to initiate dietary changes have yet to be determined. This is particularly important as significant weight gain in some ALS patients may impair respiratory muscle function and mobilization [154]. Other dietary modifications which aim to alter energy substrate supply and/or promote anaplerosis (replenishing depleted metabolic intermediates), including the ketogenic diet [155], the ‘Deanna protocol' [156,157], and triheptanoin, could potentially be useful in treating the energy deficit in ALS but need further formal testing.…”

Energy metabolism in amyotrophic lateral sclerosis: assessment of body composition and energy expenditure

“…In most populations, excessive weight gain is detrimental to cardiovascular health (Poirier et al, 2006). Furthermore, in ALS patients, excessive weight gain could impair respiratory muscle function or increase carer burden during passive mobilization of paralyzed patients (Héritier et al, 2015).…”

“…This is particularly important as significant weight gain in some ALS patients may impair respiratory muscle function and mobilization (Héritier et al, 2015). The best method for monitoring the response to nutritional interventions has yet to be determined, and it is currently unknown whether anthropometric predictors of FM are accurate in ALS and can be used to guide nutritional advice.…”