Ondine's curse--never too late.

a normal life span. It has recently been shown that CCHS confers risk for adverse neurocognitive outcome [3]. Visuoperceptual reasoning and clerical/ visuographic speed appear particularly vulnerable. In this issue of IMAJ, Cohen-Cymberknoh and colleagues [4] present an interesting and unusual case of a 12 year old girl with late-onset CCHS that was suspected and diagnosed only after she failed extubation from assisted ventilation that was initiated due to respiratory failure caused by pneumonia. Extubation failure resulted from central hypoventilation. Pneumonia is a common disease in children. However, it uncommonly results in respiratory failure necessitating assisted ventilatory support in healthy school-aged children. Hence, it is possible that central hypoventilation contributed to impaired gas exchange and respiratory failure before the intubation and precipitated intubation similarly to its contribution to extubation failure. Indeed, the patient had high CO2 levels before intubation. We cannot conclude that the pneumonia changed the respiratory drive of the patient and initiated the expression of CCHS. Hence, the hypoventilation during sleep was probably present before the ventilatory assist. A significant difference in arterial blood gas and oxygen saturation between sleep and awake states during an acute respiratory disease (pneumonia in this case) could be an early clue. The present case is also interesting in that hypoxemia during sleep was not continuous as expected in central hypoventilation, but episodic – described as "several events of hypoxemia with "You swore faithfulness to me with every waking breath, and I accepted your oath. So be it. As long as you are awake, you shall have your breath, but should you ever fall asleep, then that breath will be taken from you and you will die"