Diaphragmatic ultrasound: Prospects as a tool to assess respiratory muscle involvement in amyotrophic lateral sclerosis

2021 ◽  
Author(s):  
Rahul Reddy Rajula ◽  
Jitender Saini ◽  
Gopikrishnan Unnikrishnan ◽  
Seena Vengalil ◽  
Saraswati Nashi ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Respiratory Muscle ◽  
Muscle Involvement ◽  
Lateral Sclerosis

scholarly journals Assessment and monitoring of ventilatory function and cough efficacy in patients with amyotrophic lateral sclerosis

2016 ◽  
Vol 67 (1) ◽  
Author(s):  
A. Chetta ◽  
M. Aiello ◽  
P. Tzani ◽  
D. Olivieri
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Muscle Strength ◽  
Respiratory Muscle ◽  
Gas Analysis ◽  
Rational Approach ◽  
Strength Testing ◽  
Respiratory Muscle Strength ◽  
Ventilatory Function ◽  
Sleep Study ◽  
Lateral Sclerosis

Assessing and monitoring respiratory muscle function is crucial in patients with Amyotrophic Lateral Sclerosis, since impaired function can lead to either ventilatory failure or respiratory tract infection. Spirometry, diffusing capacity of the lung, breathing pattern, sleep study, blood gas analysis and respiratory muscle strength tests, as well as cough peak flow and cough expiratory volume measurements can provide relevant information on ventilatory function and cough efficacy. With regard to respiratory muscle strength testing, the rational approach consists in starting with volitional and non-invasive tests and later using invasive and non-volitional tests. This review focuses on both ventilatory and respiratory muscle strength testing, in order to undertake a timely treatment of respiratory failure and/or impaired cough efficacy. So far, the current literature has not highlighted any gold standard which stipulates when to commence ventilation and cough support in patients with Amyotrophic Lateral Sclerosis. A composite set of clinical and functional parameters is required for treatment scheduling to monitor lung involvement and follow-up in these patients.

scholarly journals Monitoring respiratory muscle strength assists in early diagnosis of respiratory dysfunction as opposed to the isolated use of pulmonary function evaluation in amyotrophic lateral sclerosis

2013 ◽  
Vol 71 (3) ◽  
pp. 146-152 ◽  
Author(s):  
Guilherme Fregonezi ◽  
Palomma Russelly Saldanha Araújo ◽  
Tathiana Lindemberg Ferreira Macêdo ◽  
Mario Emilio Dourado Junior ◽  
Vanessa Regiane Resqueti ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Muscle Strength ◽  
Early Diagnosis ◽  
Pulmonary Function ◽  
Healthy Subjects ◽  
Respiratory Muscle ◽  
Function Evaluation ◽  
Respiratory Muscle Strength ◽  
Respiratory Dysfunction ◽  
Lateral Sclerosis

ObjectiveIt was study the relationship between respiratory muscle strength and forced vital capacity (FVC) in patients with amyotrophic lateral sclerosis (ALS) versus healthy subjects.MethodsPulmonary function and respiratory muscle strength [maximal inspiratory (PImax), maximal expiratory (PEmax) and sniff nasal inspiratory pressure (SNIP)] were assessed in patients with ALS and healthy subjects, matched using cutoffs established in the literature for impaired pulmonary function and respiratory muscle weakness.ResultsTwenty-eight ALS patients and 28 healthy subjects were studied. We found sensitivity and specificity for PImax, PEmax and SNIP of 75/58%, 81/67% and 75/67%. The Receiver Operating Characteristic curve (ROC curve) indicated that the variables PImax, PEmax and SNIP can identify differences in respiratory muscle strength between ALS and healthy individuals at 0.89, 0.9 and 0.82, respectively. A positive correlation was recorded between FVC (%) versus SNIP, PImax and PEmax.ConclusionIn ALS, monitoring respiratory muscle strength assists in early diagnosis of respiratory dysfunction as opposed to the isolated use of FVC.

104. Distal vs. proximal muscle involvement pattern in patients with early-stage Amyotrophic Lateral Sclerosis

2008 ◽  
Vol 119 (9) ◽  
pp. e125 ◽  
Author(s):  
J. Gutierrez ◽  
H. Hernández ◽  
G. Lara ◽  
T. Zaldivar ◽  
R. Mustelier ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Early Stage ◽  
Proximal Muscle ◽  
Muscle Involvement ◽  
Lateral Sclerosis

scholarly journals Teaching an Old Dog New Tricks: Serum Troponin T as a Biomarker in Amyotrophic Lateral Sclerosis

2021 ◽  
Author(s):  
Sergio Castro-Gomez ◽  
Barbara Radermacher ◽  
Pawel Tacik ◽  
Sandra R. Mirandola ◽  
Michael T. Heneka ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Troponin I ◽  
Troponin T ◽  
Case Reports ◽  
Neuromuscular Disorders ◽  
University Hospital ◽  
Biological Origin ◽  
Muscle Involvement ◽  
Als Patients ◽  
Lateral Sclerosis

AbstractAmyotrophic lateral sclerosis (ALS) is a devastating neurodegenerative disease characterized by progressive loss of upper and lower motor neurons. Diagnosis, management and therapeutic trials are hampered by a lack of informative biomarkers. Troponins (Tn) are components of skeletal and cardiac muscles. Acute elevation of cardiac isoforms of troponin I (cTnI) and T (cTnT) in serum indicates myocardial injury. Case reports suggested that serum levels of cTnT, but not cTnI are chronically elevated in ALS and other neuromuscular disorders.Using standard clinical laboratory methodologies we studied serum troponin levels in a multicentric cross-sectional cohort of 75 ALS patients and sixty controls (DESCRIBE-ALS cohort) and in a real-world cohort of 179 consecutive patients from our ALS clinic at the University Hospital Bonn.We found that serum cTnT, is elevated in >60% of ALS patients while cTnI is always normal. Serum cTnT levels increase over time and correlate with disease severity as measured with the revised ALS FRS score. There was no correlation with the phosphorylated neurofilament heavy chain (pNfH) levels in the cerebrospinal fluid. We propose that cTnT elevations in ALS are of non-cardiac origin and may serve as a proxy of lower motor neuron or skeletal muscle involvement. They potentially help to stratify patients according to lower motoneuron involvement. Further research will determine the biological origin of the cTnT elevation and its validity as a diagnostic and/or prognostic marker. Our finding also serves as a reminder to interpret cTnT with caution elevations in patients with neuromuscular diseases.

Lung function and respiratory muscle strength in amyotrophic lateral sclerosis

2015 ◽  
Author(s):  
Astrid Sandnes ◽  
Tiina Andersen ◽  
Maria Vollsæter ◽  
Ove Fondenes ◽  
Ola Røksund ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Muscle Strength ◽  
Lung Function ◽  
Respiratory Muscle ◽  
Respiratory Muscle Strength ◽  
Lateral Sclerosis

scholarly journals Longitudinal Quantitative MRI Evaluation of Muscle Involvement in Amyotrophic Lateral Sclerosis

2021 ◽  
Vol 12 ◽  
Author(s):  
Matteo Paoletti ◽  
Luca Diamanti ◽  
Shaun I. Muzic ◽  
Elena Ballante ◽  
Francesca Solazzo ◽  
...  
Keyword(s):  
Amyotrophic Lateral Sclerosis ◽  
Disease Progression ◽  
Rating Scale ◽  
Quantitative Mri ◽  
Triceps Surae ◽  
Muscle Mri ◽  
Muscle Involvement ◽  
Leg Muscles ◽  
Lateral Sclerosis ◽  
Over Time

Background: Biomarkers of disease progression and outcome measures are still lacking for patients with amyotrophic lateral sclerosis (ALS). Muscle MRI can be a promising candidate to track longitudinal changes and to predict response to the therapy in clinical trials.Objective: Our aim is to apply quantitative muscle MRI in the evaluation of disease progression, focusing on thigh and leg muscles of patients with ALS, and to explore the correlation between radiological and clinical scores.Methods: We enrolled newly diagnosed patients with ALS, longitudinally scored using the ALS Functional Rating Scale-Revised (ALSFRS-R), who underwent a 3T muscle MRI protocol including a 6-point Dixon gradient-echo sequence and multi-echo turbo spin echo (TSE) T2-weighted sequence for quantification of fat fraction (FF), cross-sectional area (CSA), and water T2 (wT2). A total of 12 muscles of the thigh and six muscles of the leg were assessed by the manual drawing of 18 regions of interest (ROIs), for each side. A group of 11 age-matched healthy controls (HCs) was enrolled for comparison.Results: 15 patients (M/F 8/7; mean age 62.2 years old, range 29–79) diagnosed with possible (n = 2), probable (n = 12), or definite (n = 1) ALS were enrolled. Eleven patients presented spinal onset, whereas four of them had initial bulbar involvement. All patients performed MRI at T0, nine of them at T1, and seven of them at T2. At baseline, wT2 was significantly elevated in ALS subjects compared to HCs for several muscles of the thigh and mainly for leg muscles. By contrast, FF was elevated in few muscles, and mainly at the level of the thigh. The applied mixed effects model showed that FF increased significantly in the leg muscles over time (mainly in the triceps surae) and that wT2 decreased significantly in line with worsening in the leg subscore of ALSFRS-R, mainly at the leg level and in the anterior and medial compartment of the thigh.Conclusions: Quantitative MRI represents a non-invasive tool that is able to outline the trajectory of pathogenic modifications at the muscle level in ALS. In particular, wT2 was found to be increased early in the clinical history of ALS and also tended to decrease over time, also showing a positive correlation with leg subscore of ALSFRS-R.

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