Spirometry

The most obvious life-threatening complication of weakness is the involvement of muscle associated with coughing and swallowing. It is important to note that arterial blood gas analysis is practically useless in the assessment of impending ventilatory failure. Patients with respiratory muscle weakness will characteristically develop abnormal blood gases shortly after respiratory arrest. In the early stages of respiratory muscle weakness the clinical signs are subtle, but there may be dyspnea of effort and the use of accessory muscles of respiration. Paradoxical abdominal movement may suggest diaphragmatic weakness. Difficulty in swallowing, coughing, and speaking suggests impending respiratory failure and may also expose the patient to the risk of aspiration pneumonia. Patients with respiratory muscle weakness and a consequent inability to increase tidal volumes compensate by increasing the respiratory rate. Tachypnea with a respiratory rate of more than 30 breaths/min is an ominous sign. Therefore the most useful bedside test is measurement of tidal volume and vital capacity. A vital capacity of less than 1 liter or a fall of 50 per cent from normal should lead to intensive care referral. Vital capacity should be measured at least twice daily, and more often if the disease is progressing rapidly. If the patient develops severe difficulty in swallowing or coughing or develops an aspiration pneumonia, the airway should be secured and mechanical ventilatory support provided.

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