First assessment at home of amyotrophic lateral sclerosis (ALS) patients by a nutrition network in the French region of Limousin

Abstract

Malnutrition is associated with poor survival among patients with amyotrophic lateral sclerosis (ALS). This study aimed to evaluate nutritional assessment by a network during first consultations in patients' homes. Patients identified by the regional ALS centre gave their informed consent. Assessment included functional, nutritional issues, evaluation of the need for help, whether personal or the use of aids, and noted any dietary supplementation and modification of the texture of food. Forty patients were seen a mean of 7.4 months after diagnosis; 52.5% had bulbar disease, 7.5% were malnourished; 29.4 ± 10.1 kcal/kg/day were consumed and protein intake was 1.3 ± 0.5 g/kg/day. Thirty-five percent of patients were anorexic, 43.8% reported taste disorders, and 70% had dysphagia, significantly associated with salivary stasis. Only 30% of dysphagic patients ate texture-modified food, and 90% of patients with problems drinking liquids did not use a thickener. In conclusion, assessment at home by a nutritional network can be conducted promptly. Malnutrition is rare in early disease, despite the fact that patients' diets are often low in energy and dysphagia is common. Unexpected taste disorders are detected. Dysphagia is very common but inadequately addressed. Consequently, home assessment by the network led several beneficial interventions.

Trends in Research Literature Describing Dysphagia in Motor Neuron Diseases (MND): A Scoping Review

“…Still, almost half (48%) of these recommendations were provided as secondary source review (e.g., [12,14,100,102,104,105]), which may include general techniques that have not been specifically evaluated for individuals with MND. Of the studies presenting original research findings to support their recommendations, many studies did not describe their efficacy [30,42,57,117,112,101], or concluded that swallowing function improved based solely on patient report [118]. Only four studies used instrumental assessment to confirm the effectiveness of a prescribed diet texture modification or compensatory strategy to prevent aspiration [39,45,56,61].…”

“…This has provided a rationale for using sensory tricks in dysphagia management – on the assumption that these strategies exploit unimpaired mechanisms to compensate for impaired motor function (e.g., [30,101]). However, we were surprised to find that 7 articles (i.e., 4.5% of all reviewed articles) reported observing silent aspiration [13,16,39,41,45,62,122], and an additional subset of articles reported sensory changes such as reduced laryngeal sensitivity, changes in taste, and odynophagia [32,37,46,112,123]. These observations support a more contemporary view of MND as a multi-system disorder [124].…”

ESPEN guideline clinical nutrition in neurology

“…With clinical assessment, 6% of isolated dysphagia and 15% of dysphagia and dysarthria were found (35% of bulbar form) [67]. The presence of dysphagia after patient interview was found in 85.7% of cases in bulbar form, 42.9% in upper limbs form and in 71.4% in lower limbs form (33,4%) [68]. With a same assessment 34.7% of dysphagia was found (21.4% of bulbar form) [69].…”

Metabolic Abnormalities, Dietary Risk Factors and Nutritional Management in Amyotrophic Lateral Sclerosis

“…On the other hand, stable weight at diagnosis was shown to be important, because patients who lost less than 5% of their usual body weight in the six months prior to ALS diagnosis had a higher median survival than those with higher body weight loss [ 61 ]. Out of the 40 ALS patients who were enrolled in a home network of surveillance, three patients were clinically malnourished and reported having unbalanced diets, with insufficient protein, high fat intake and not enough carbohydrate compared with French dietary recommendations [ 127 ]. In general, patients with ALS tend to assume an inadequate caloric intake, with about 15–16% fewer calories than recommended [ 99 ].…”