RIGID SPINE SYNDROME AND NOCTURNAL ALVEOLAR HYPOVENTILATION

A 17-year-old Japanese woman with rigid spine syndrome (RSS) presented with respiratory failure leading to CO2 narcosis. The clinical sympto ms mere drowsiness, asterixis and cardiac arrhythmias. Tracheostomy an d temporary ventilatory support abolished these symptoms. However, pol ygraphic sleep studies without a ventilator revealed Cheyne-Stokes res piration and profound arterial oxygen desaturation during rapid eye mo vement sleep. Nocturnal ventilatory support improved not only nocturna l hypoxemia, but daytime blood gas values during spontaneous breathing . These findings indicate that the onset of respiratory failure is pre ceded by severe nocturnal hypoxemia and that the evaluation and contro l of nocturnal respiratory insufficiency is essential for RSS patients .