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Epidemiology, diagnosis, and assessment of neuromuscular syndromes 

Epidemiology, diagnosis, and assessment of neuromuscular syndromes
Epidemiology, diagnosis, and assessment of neuromuscular syndromes

David Orlikowski

and Tarek Sharshar

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date: 16 May 2022

Admission to ICU with severe limb weakness, or the occurrence of a respiratory or motor deficit, and failure to wean from mechanical ventilation while in the intensive care unit are common presentations of a neuromuscular disease. Neuromuscular diseases include neuronopathies, neuropathies, myasthenic syndromes, and myopathies. An accurate neurological examination and complementary investigations are necessary for both diagnosis and for evaluating the severity of the disease. Assessment of respiratory muscle function is a key step in deciding the need for mechanical ventilation and subsequently its weaning. Hypercapnia often indicates an impending respiratory arrest, but normocapnia, which can be seen in a patient with severe reduction in vital capacity is not reassuring. Hypoxaemia can be due to hypercapnia, pulmonary injury (atelectasis or pneumonia), or pulmonary embolism. Cardiac evaluation is important as cardiomyopathies are frequent in myopathies.

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