Noninvasive assessment of respiratory muscle strength and activity in Myotonic dystrophy

Evangelista, Morgana de Araújo; Dias, Fernando Augusto Lavezzo; Júnior, Mário Emílio Teixeira Dourado; Nascimento, George João Ferreira do; Sarmento, António; Gualdi, Lucien Peroni; Aliverti, Andréa; Resqueti, Vanessa; Fregonezi, Guilherme

doi:10.1371/journal.pone.0177318

Cited by 12 publications

(13 citation statements)

References 40 publications

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“…In our study, all parameters derived from the SNIP curve were significantly different between middle stage ALS and healthy subjects, being in agreement with two previous studies performed in subjects with neuromuscular disorders (47,49). Evangelista et al (49), observed that a reduced MRR was reliable in identifying the delayed relaxation of the respiratory muscles in myotonic dystrophy type 1 patients when compared to healthy controls; while Garcia-Rio et al (47), despite heterogeneity of the study population, found that the decreased MRR of neuromuscular disease patients was accompanied by the deterioration in the functional reserve of the diaphragm as well as of the inspiratory muscles. The rationale for measuring relaxation rates from pressure curves is based on the assumption that the decay portion of the curve, when expiration is totally passive, corresponds to the relaxation phase of inspiratory muscle contraction (18).…”

Section: Discussionsupporting

confidence: 93%

Multiparametric analysis of sniff nasal inspiratory pressure test in middle stage amyotrophic lateral sclerosis

Sarmento¹,

Aliverti²,

Marques³

et al. 2018

Clinical Respiratory Physiology, Exercise and Functional Imaging

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Section: Discussionsupporting

confidence: 93%

Multiparametric analysis of sniff nasal inspiratory pressure test in middle stage amyotrophic lateral sclerosis

Sarmento¹,

Aliverti²,

Marques³

et al. 2018

Clinical Respiratory Physiology, Exercise and Functional Imaging

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“…Moreover, measurements of nasal sniffs are simple, tolerated, and minimally invasive and can provide a quantitative response index to fatigue and therapeutic interventions in neuromuscular disease patients ( 47 , 48 ). In our study, all parameters derived from the SNIP curve were significantly different between middle stage ALS and healthy subjects, being in agreement with two previous studies performed in subjects with neuromuscular disorders ( 47 , 49 ). Evangelista et al ( 49 ), observed that a reduced MRR was reliable in identifying the delayed relaxation of the respiratory muscles in myotonic dystrophy type 1 patients when compared to healthy controls; while Garcia-Rio et al ( 47 ), despite heterogeneity of the study population, found that the decreased MRR of neuromuscular disease patients was accompanied by the deterioration in the functional reserve of the diaphragm as well as of the inspiratory muscles.…”

Section: Discussionsupporting

confidence: 93%

Multiparametric Analysis of Sniff Nasal Inspiratory Pressure Test in Middle Stage Amyotrophic Lateral Sclerosis

et al. 2018

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The relaxation rates and contractile properties of inspiratory muscles are altered with inspiratory muscle weakness and fatigue. This fact plays an important role in neuromuscular disorders patients and had never been extensively studied in amyotrophic lateral sclerosis (ALS). In this cross-sectional study, these parameters were investigated non-invasively through nasal inspiratory sniff pressure test (SNIP) in 39 middle stage spinal onset ALS subjects and compared with 39 healthy controls. ALS patients were also divided into three subgroups according to a decline in their percentage of predicted forced vital capacity (FVC%pred) as well as a decline in the ALS functional rating scale score and its respiratory subscore (R-subscore) in order to determine the best parameter linked to early respiratory muscle weakness. When compared with healthy subjects, middle stage ALS subjects exhibited a significantly lower (p < 0.0001) maximum relaxation rate and maximum rate of pressure development (MRPD), as well as a significantly higher (p < 0.0001) tau (τ), contraction time, and half-relaxation time. The results from receiver operating characteristic curves showed that MRPD (AUC 0.735, p < 0.001) and FVC%pred (AUC 0.749, p = 0.009) were the best discriminator parameters between ALS patients with ≤30 and >30 points in the ALS functional rating scale. In addition, 1/2RT (AUC 0.720, p = 0.01), FVC%pred (AUC 0.700, p = 0.03), τ (AUC 0.824, p < 0.0001), and MRPD (AUC 0.721, p = 0.01) were the parameters more sensitive in detecting a fall of three points in the R-subscore. On the other hand, MRPD (AUC 0.781, p < 0.001), τ (AUC 0.794, p = 0.0001), and percentage of predicted of SNIP (AUC 0.769, p = 0.002) were the parameters able to detect a fall in 30% of the FVC%pred in middle stage ALS patients. The contractile properties and relaxation rates of the diaphragm are altered in middle stage spinal onset ALS when compared with healthy subjects. These parameters are able to discriminate between those middle stage ALS subjects with early decline in inspiratory muscle function and those who not.

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“…Myotonic dystrophy type 1 (DM1) is an autosomal dominant neuromuscular disorder and is the most common form of muscular dystrophy in adults . While DM1 is a multi‐systemic disorder significantly affecting cardiac muscle, brain and other tissues, a primary cause of mortality is due to skeletal muscle weakness and wasting of respiratory muscles . Weakness typically begins in distal muscles, leading to impaired ambulation, and difficulty with fine motor tasks and is a top concern among individuals affected by DM1 .…”

Section: Introductionmentioning

confidence: 99%

Endurance exercise leads to beneficial molecular and physiological effects in a mouse model of myotonic dystrophy type 1

2019

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Introduction Myotonic dystrophy type 1 (DM1) is a multisystemic disease caused by expansion of a CTG repeat in the 3' UTR of the Dystrophia Myotonica‐Protein Kinase (DMPK) gene. While multiple organs are affected, more than half of mortality is due to muscle wasting. Methods It is unclear whether endurance exercise provides beneficial effects in DM1. Here, we show that a 10‐week treadmill endurance exercise program leads to beneficial effects in the HSALR mouse model of DM1. Results Animals that performed treadmill training displayed reduced CUGexp RNA levels, improved splicing abnormalities, an increase in skeletal muscle weight and improved endurance capacity. Discussion These results indicate that endurance exercise does not have adverse effects in HSALR animals and contributes to beneficial molecular and physiological outcomes.

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Noninvasive assessment of respiratory muscle strength and activity in Myotonic dystrophy

Cited by 12 publications

References 40 publications

Multiparametric analysis of sniff nasal inspiratory pressure test in middle stage amyotrophic lateral sclerosis

Multiparametric analysis of sniff nasal inspiratory pressure test in middle stage amyotrophic lateral sclerosis

Multiparametric Analysis of Sniff Nasal Inspiratory Pressure Test in Middle Stage Amyotrophic Lateral Sclerosis

Endurance exercise leads to beneficial molecular and physiological effects in a mouse model of myotonic dystrophy type 1

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