Loss of appetite in amyotrophic lateral sclerosis is associated with weight loss and decreased calorie consumption independent of dysphagia.

BACKGROUND: Loss of appetite has been reported to affect up to half of people with amyotrophic lateral sclerosis (ALS) and to be associated with weight loss. We wished to test whether loss of appetite correlates with reduced dietary intake independent of dysphagia. METHODS: Appetite was measured repeatedly using the Council on Nutrition Appetite Questionnaire (CNAQ) in participants in the Electronic health Application To Measure Outcomes REmotely study. Dietary intake and weight were compared to appetite, ALS Functional Rating Scale-Revised total and bulbar scores (dysphagia). RESULTS: The average baseline CNAQ score was 30.4 (n = 61; SD = 3.9) with 18.0% scoring <28 points (severe loss of appetite). Lower CNAQ scores correlated with greater weight loss since diagnosis (Pearson correlation coefficient, r = -0.34; P = 0.009) and lower baseline energy intake (P = 0.007), independent of dysphagia. CONCLUSIONS: Our results support an association between loss of appetite and decreased calorie intake and weight in ALS which is independent of dysphagia.

Nutritional counseling with or without mobile health technology: a randomized open-label standard-of-care-controlled trial in ALS.

BACKGROUND: Nutritional status is an important prognostic factor in Amyotrophic Lateral Sclerosis (ALS). We wished to study the safety, tolerability and efficacy of nutritional counseling with or without an mHealth application to maintain or increase body weight in ALS, compared to standard care. METHODS: In this randomized open-label, standard-of-care-controlled, single-center clinical trial, we randomly assigned adults with ALS to one of three nutritional interventions: counseling by their physician or nurse ("standard care"), counseling by a registered dietitian (RD) ("in-person"), or counseling supported by a mHealth app ("mHealth"). Both intervention arms received tailored nutrition recommendations and recorded dietary intake and weight biweekly (mHealth) or monthly (in-person). The primary outcome of weight and secondary and tertiary outcomes of calorie intake, ALSFRS-R, and quality of life (QOL) were recorded at each clinic visit and analyzed in an ITT mixed model analysis. RESULTS: A total of 88 participants were enrolled of whom 78 were included in this analysis. The three arms were well-balanced except for excess males in the mHealth arm and greater weight lost at baseline in the in-person arm. Participants in the mHealth arm increased their calorie intake at month 3 to an average of 94% (95% CI: 85, 103) of recommended calories, compared to 81% (95% CI: 72, 91, p = 0.06 vs. mHealth) in the standard care arm. After 6 months, calorie intake was not different among the three arms. Overall weight was stable across all three groups. QOL scores in the mHealth arm were stable over 3 months (0.3 points, 95% CI: - 1.7, 2.2) compared to worsening in standard care (- 2.1 points, 95% CI: - 4.0, - 0.2, p = 0.09 vs. mHealth), but all scores declined by 6 months. ALSFRS-R total scores declined by an average of - 2.6 points (95% CI: - 5.1, - 0.1) over 6 months in the mHealth arm (p = 0.13 vs. standard care) compared to - 5.8 points (95% CI: - 8.2, - 3.4, p = 0.74 vs. standard care) in the in-person and - 5.2 points (95% CI: - 7.6, - 2.9) in the standard care arm. CONCLUSIONS: Nutritional counseling by a registered dietitian (with or without support by an mHealth app) is safe but did not maintain weight significantly better than standard care in ALS patients. TRIAL REGISTRATION: https://clinicaltrials.gov/ identifier NCT02418546. Registered April 16, 2015.

Provisional best practices guidelines for the evaluation of bulbar dysfunction in amyotrophic lateral sclerosis.

INTRODUCTION: Universally established comprehensive clinical bulbar scales objectively assessing disease progression in amyotrophic lateral sclerosis (ALS) are currently lacking. The goal of this working group project is to design a best practice set of provisional bulbar ALS guidelines, available for immediate implementation within all ALS clinics. METHODS: ALS specialists across multiple related disciplines participated in a series of clinical bulbar symposia, intending to identify and summarize the currently accepted best practices for the assessment and management of bulbar dysfunction in ALS Results: Summary group recommendations for individual speech, Augmentative and Alternative Communication (AAC), and swallowing sections were achieved, focusing on the optimal proposed level of care within each domain. DISCUSSION: We have identified specific clinical recommendations for each of the 3 domains of bulbar functioning, available for incorporation within all ALS clinics. Future directions will be to establish a formal set of bulbar guidelines through a methodological and evidence-based approach. Muscle Nerve 59:531-531, 2019.

Best practices protocol for the evaluation of bulbar dysfunction: summary recommendations from the NEALS bulbar subcommittee symposium.

OBJECTIVE: The aim of this Symposium was to develop a consensus based, bulbar assessment protocol for implementation within NEALS clinics. METHODS: A one-day symposium, held in April 2017, was organized into Speech and Swallowing sections to establish summary recommendations for the assessment of bulbar dysfunction within each group. RESULTS: Summary recommendations included speech referrals and AAC evaluations at initial visit, CNS-BFS, maximum sustained phonation, and speaking rate. Dysarthria evaluation included the speech subsystem involvement of respiration, phonation, resonance, and articulation. Specific recommendations for swallowing were established for each of the following domains: dietary/oral intake, airway defense physiologic capacity, swallow safety screen, patient-reported swallow-related outcomes, oral sensorimotor exam, and pulmonary function. Practice parameters focused upon patient education and unresolved questions included the use of videofluoscopy, monitoring diet progression, and swallow safety screening. CONCLUSIONS: The working goal is to establish a clinical bulbar protocol, designed to be incorporated within ALS clinics and ultimately to formulate a best practice set of bulbar ALS guidelines, available for implementation throughout the international ALS community.