Respiratory therapies for amyotrophic lateral sclerosis: A primer

Respiratory complications are a common cause of morbidity and mortality in amyotrophic lateral sclerosis (ALS). Treatment of respiratory insufficiency with noninvasive ventilation (NIV) improves ALS patients' quality of life and survival. Evidence‐based practice guidelines for the management of ALS patients recommend treatment of respiratory insufficiency with NIV as well as consideration of insufflation/exsufflation to improve clearance of airway secretions. Despite these recommendations respiratory therapies remain underused. In this review we provide a practical guide for the clinician to prescribe and manage respiratory therapies for the patient with ALS. Muscle Nerve 46: 313–331, 2012Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/93524/1/23282_ftp.pd

Validation of the Tranquillity Rating Prediction Tool (TRAPT): comparative studies in UK and Hong Kong

YesThe Tranquillity Rating Prediction Tool (TRAPT) has been used to make predictions of the quality of tranquility in outdoor urban areas using two significant factors i.e. the average level of anthropogenic noise and the percentage of natural features in view. The method has a number of applications including producing tranquillity contours that can inform decisions regarding the impact of new anthropogenic noise sources or developments causing visual intrusion. The methodwas intended for use in mainly outdoor areas and yet was developed using responses from UK volunteers to video clips indoors. Because the volunteers for this study were all UK residents it was important to calibrate responses for other ethnic groups who may respond differently depending on cultural background. To address these issues further studies were performed in Hong Kong using the same video recording played back under the same conditions as the study in the UK. The HK study involved recruiting three groups i.e. residents fromHong Kong, Mainland China and a diverse group from 16 different nations. There was good agreement between all these groups with average tranquillity ratings for the different locations differing by less than one scale point in most cases.The studywas supported by the Bradford Centre for Sustainable Environments at the University of Bradford and by the Research Grants Council of the Hong Kong Special Administrative Region, China (RGC/GRF. CUHK 449612

Mechanical cough augmentation techniques in amyotrophic lateral sclerosis/motor neuron disease

© 2016 The Cochrane Collaboration.This is a protocol for a Cochrane Review (Intervention). The objectives are as follows: To assess the effects of mechanical insufflator/exsufflator (MI-E) and the breath-stacking technique for reducing morbidity and mortality and enhancing quality of life in people with amyotrophic lateral sclerosis (ALS)/motor neuron disease (MND)

Respiratory Measures in Amyotrophic Lateral Sclerosis

Objective: Amyotrophic lateral sclerosis (ALS) is a progressive neuromuscular disease that causes skeletal muscle weakness, including muscles involved with respiration. Death often results from respiratory failure within 3€“5 years. Monitoring respiratory status is therefore critical to ALS management, as respiratory/pulmonary function tests (PFTs) are used to make decisions including when to initiate noninvasive ventilation. Understanding the different respiratory and PFTs as they relate to disease progression and survival may help determine which tests are most suitable. Methods: This review describes the tests used to assess respiratory muscle and pulmonary function in patients with ALS and the correlations between different respiratory measures and clinical outcomes measures. Results: The most commonly used measurement, forced vital capacity (VC), has been shown to correlate with clinical milestones including survival, but also requires good motor coordination and facial strength to form a tight seal around a mouthpiece. Other tests such as slow VC, sniff inspiratory pressure, or transdiaphragmatic pressure with magnetic stimulation are also associated with distinct advantages and disadvantages. Conclusions: Therefore, how and when to use different tests remains unclear. Understanding how each test relates to disease progression and survival may help determine which is best suited for specific clinical decisions

MiToS and King\u27s staging as clinical outcome measures in ALS: A retrospective analysis of the FORTITUDE-ALS trial

OBJECTIVE: To evaluate the Milano-Torino staging (MiToS) and King\u27s staging systems as potential outcome measures for clinical trials in amyotrophic lateral sclerosis (ALS) by assessing these outcomes in FORTITUDE-ALS. METHODS: This was a RESULTS: The full analysis set consisted of 456 patients randomized 3:1 ( CONCLUSION: This exploratory analysis showed the feasibility of MiToS and King\u27s staging as potential outcome measures in ALS. Additional studies of these staging systems are needed to further explore their utility in ALS clinical trials

How Respiratory Muscle Strength Correlates with Cough Capacity in Patients with Respiratory Muscle Weakness

PURPOSE: The purpose of this study is to investigate how respiratory muscle strength correlates to cough capacity in patients with respiratory muscle weakness. MATERIALS AND METHODS: Forty-five patients with amyotrophic lateral sclerosis (ALS), 43 with cervical spinal cord injury (SCI), and 42 with Duchenne muscular dystrophy (DMD) were recruited. Pulmonary function tests including forced vital capacity (FVC) and respiratory muscle strength (maximal expiratory pressure, MEP; maximal inspiratory pressure, MIP) were performed. The correlation between respiratory muscle strength and cough capacity was analyzed. RESULTS: In the SCI group, FVC in a supine position (2,597 +/- 648 mL) was significantly higher than FVC in a sitting position (2,304 +/- 564 mL, p < 0.01). Conversely, in the ALS group, FVC sitting (1,370 +/- 604 mL) was significantly higher than in supine (1,168 +/- 599 mL, p < 0.01). In the DMD group, there was no statistically significant difference between FVC while sitting (1,342 +/- 506 mL) and FVC while supine (1,304 +/- 500 mL). In addition, the MEP and MIP of all three groups showed a significant correlation with peak cough flow (PCF) (p < 0.01, Pearson's correlation analysis). In the SCI group, MIP was more closely correlated with PCF, while in the ALS and DMD groups, MEP was more closely correlated with PCF (p < 0.01, multiple regression analysis). CONCLUSION: To generate cough flow, inspiratory muscle strength is significantly more important for SCI patients, while expiratory muscle function is significantly more important for ALS and DMD patients.ope