neuromuscular diseases that affect breathing

/10.1155/2019/2734054 respiratory muscle weakness is a major cause of morbidity and mortality in patients with neuromuscular diseases (nmds). the respiratory pump is responsible for moving the air in and out of the lungs. in patients with nmds the contractile function of the inspiratory and expiratory muscles is impaired. however, although vc is considered a robust index for the respiratory evaluation of patients with nmds, it can be fairly insensitive in early disease stages. moreover, a pecf less than 270 l/min is associated with higher risk for acute respiratory failure and higher respiratory morbidity during the course of a trivial respiratory tract infection [68–70]. in addition, when compared to pecf and pefrt, cva was the most reliable predictor of aspiration in patients with als [66].

snip has a strong correlation with pco2 in nonbulbar patients with als and a snip of less than −40 cm h2o was the most reliable noninvasive predictor of hypercapnic respiratory failure in nonbulbar als patients with a sensitivity of 85% and a specificity of 81% [63, 84]. the aim of respiratory management of patients with nmds is threefold: ventilatory support, cough augmentation, and lung volume recruitment. an improvement in sleep and respiratory outcomes which persisted for 15 months was also noted in a retrospective study of bulbar and nonbulbar als patients treated with niv [116]. moreover, in a study by tzeng and bach which included neuromuscular patients with pecf of less than 270 l/min or at least 1 episode of respiratory decompensation, the combination of nvs with cough augmentation techniques was associated with a significant reduction in total admissions and the duration in hospital stay due to respiratory exacerbations [68]. in the case of acute respiratory failure (arf) in patients with nmd, niv, and aggressive secretion management with mi-e should be introduced. early recognition of respiratory impairment is vital and a multidisciplinary approach should be adopted in order to provide the best optimal treatment.

disorders of neuromuscular transmission such as lambert-eaton syndrome, botulism, and myasthenia gravis often affect respiration. many muscle diseases also patients with neuromuscular disease may develop respiratory failure on account of weakness of respiratory muscles (diaphragm and accessory incidence — respiratory muscle weakness can be a serious problem among patients with neuromuscular disease. the incidence varies with the, neuromuscular weakness, neuromuscular weakness, neurological diseases that affect the cardiovascular and respiratory system, respiratory muscle fatigue signs and symptoms, treatment for weak lung muscles.

respiratory muscle weakness and sleepdisordered breathing are both common in neuromuscular diseases such as amyotrophic lateral sclerosis (als) 1u20135, muscular dystrophies (e.g. duchenne’s muscular dystrophy (dmd) 6u201310, and myotonic dystrophy (md) 11u201314, myopathies (e.g. nemaline myopathy 15), guillianbarrxe9 syndrome and the most common causes are diffuse muscle diseases or motor neuron disease such as als. in bilateral diaphragm paralysis orthopnea, dyspnea with spinal muscular atrophy (sma), which affects the spinal cord and nerves, resulting in muscle wasting and weakness muscular dystrophy (md), which causes the what is neuromuscular weakness? neuromuscular diseases affect the function of muscles in your body. these conditions are usually due to problems with the nerves, neuromuscular disorders, respiratory muscle weakness causes.

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