[Acute respiratory insufficiency as initial manifestation of brain stem lesions]

We describe three patients with different brainstem lesions (bulbar angioma, bulbar infarct, and Arnold-Chiari malformation) who debuted with acute respiratory insufficiency. Other neurological manifestations had gone unobserved in all three cases. Respiratory insufficiency worsened notably during sleep to the point that mechanical ventilation was required or death occurred (Ondine s curse). The patient with a bulbar angioma is stable with only assisted ventilation by a nasal route at night, with good quality of life. Our conclusions are that: a) central nervous system anomalies need to be investigated as possible causes of respiratory insufficiency when lungs are normal; b) the respiratory control of patients with brainstem lesions should be studied, particularly at night (polysomnography), and c) even when awake-state ventilation is adequate, nighttime assisted ventilation may be required.