Expert Clinical Perspectives: Evaluation and management of dyspnea as the dominant presenting feature in neuromuscular disorders-Article

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The article titled "Evaluation and management of dyspnea as the dominant presenting feature in neuromuscular disorders" covers the various aspects and strategies in diagnosing and treating dyspnea in patients with neuromuscular disorders (NMDs). Dyspnea, a common and often multifactorial symptom in NMDs, usually results from respiratory muscle involvement, musculoskeletal changes like scoliosis, or cardiomyopathy associated with some NMDs.<br /><br />A thorough clinical history and examination are crucial for distinguishing between neurologic and non-neurologic causes of dyspnea. The key historical elements include orthopnea, trepopnea, sleep disruption, dysphagia, weak cough, and secretion clearance difficulties. Physical examination should assess for muscle loss, accessory muscle use, thoraco-abdominal paradox, conversational dyspnea, and include a detailed neurological examination.<br /><br />Diagnostic evaluations include pulmonary function tests (PFTs) like spirometry, sitting and supine forced vital capacity (FVC), maximal inspiratory pressure (MIP), maximal expiratory pressure (MEP), and sniff nasal inspiratory pressure (SNIP). Additional tests like sniff fluoroscopy, diaphragm ultrasound, and electromyography (EMG) of the diaphragm may be used. Polysomnography is recommended for sleep-related symptoms.<br /><br />Noninvasive ventilation (NIV) has shown to alleviate dyspnea, improve the quality of life, and prolong survival in patients with NMDs. Respiratory muscle training (RMT) is beneficial in improving muscle endurance and strength. However, RMT should be avoided in patients with hypercapnia, FVC below 25%, or rapid progression of the disease.<br /><br />Management of respiratory complications also includes using mechanical insufflation-exsufflation devices and pharmacological agents to manage secretions. The review emphasizes that early diagnosis and intervention with the right therapeutic approach can significantly alter clinical outcomes and improve the quality of life for patients experiencing dyspnea due to neuromuscular disorders.

Keywords

dyspnea

neuromuscular disorders

respiratory muscle involvement

diagnostic evaluations

noninvasive ventilation

respiratory muscle training

pulmonary function tests

clinical history

mechanical insufflation-exsufflation

quality of life