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Progression to mechanical ventilation was highly likely to occur in those patients with rapid disease progression, bulbar dysfunction, bilateral facial weakness, or dysautonomia. Factors associated with progression to respiratory failure in cluded vital capacity of less than 20 mL/kg, maximal inspiratory pressure less than 30 cm H2O, maximal expiratory pressure less than 40 cm H2O or a reduction of more than 30% in vital capacity, maximal inspiratory pressure, or maximal expiratory pressure. No clinical features predicted the pattern of respiratory decline; however, serial measurements of pulmonary function tests allowed detection of those at risk for respiratory failure. While inherently unpredictable, the course of patients with severe G BS can, to some extent, be predicted on the basis of clinical information and simple bedside tests of respiratory function. These data may be used in the decisions regarding admission to the intensive care unit and preparation for elective intubation. |
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