Kawata A, Suga M, Miyamoto K, Hirose K, Tanabe H
Department of Neurology, Tokyo Metropolitan Neurological Hospital, Japan.
Intern Med. 1993 Aug;32(8):638-40. doi: 10.2169/internalmedicine.32.638.
A 17-year-old Japanese woman with rigid spine syndrome (RSS) presented with respiratory failure leading to CO2 narcosis. The clinical symptoms were drowsiness, asterixis and cardiac arrhythmias. Tracheostomy and temporary ventilatory support abolished these symptoms. However, polygraphic sleep studies without a ventilator revealed Cheyne-Stokes respiration and profound arterial oxygen desaturation during rapid eye movement sleep. Nocturnal ventilatory support improved not only nocturnal hypoxemia, but daytime blood gas values during spontaneous breathing. These findings indicate that the onset of respiratory failure is preceded by severe nocturnal hypoxemia and that the evaluation and control of nocturnal respiratory insufficiency is essential for RSS patients.
一名患有僵硬脊柱综合征（RSS）的17岁日本女性出现呼吸衰竭，导致二氧化碳麻醉。临床症状为嗜睡、扑翼样震颤和心律失常。气管切开术和临时通气支持消除了这些症状。然而，在没有呼吸机的情况下进行的多导睡眠研究显示，快速眼动睡眠期间出现潮式呼吸和严重的动脉血氧饱和度降低。夜间通气支持不仅改善了夜间低氧血症，还改善了自主呼吸时的日间血气值。这些发现表明，呼吸衰竭的发作之前存在严重的夜间低氧血症，并且评估和控制夜间呼吸功能不全对RSS患者至关重要。
