Osanai S, Ohmiya H, Akashi K, Inaba S, Nagai T, Yoshikawa T, Terai T, Ishimaru O, Yahara O, Onodera S
First Department of Medicine, Obihiro-Kohsei Hospital, Japan.
Nihon Kyobu Shikkan Gakkai Zasshi. 1991 Oct;29(10):1334-8.
We reported a case of Kearns-Sayre syndrome with impaired respiratory regulation. A 55-year-old male was admitted to our hospital complaining of chronic progressive external ophthalmoplegia, limb muscle weakness and dyspnea. On admission, because arterial blood gas analysis showed marked alveolar hypoventilation, ventilatory response was measured and diminished chemosensitivity to both hypoxia and hypercapnia was found. His vital capacity and forced expiratory volume in 1 second were slightly decreased, and a chest X-ray film revealed a moderate degree of elevation of the bilateral diaphragm. Therefore, we considered that the diminished response to hypoxia and hypercapnia in this case was caused by an impairment of the respiratory center, as well as chemoreceptors and also the presence of respiratory muscular weakness.
我们报告了一例伴有呼吸调节受损的卡恩斯-塞尔综合征病例。一名55岁男性因慢性进行性眼外肌麻痹、肢体肌肉无力和呼吸困难入院。入院时，由于动脉血气分析显示明显的肺泡通气不足，我们测量了通气反应，发现对低氧和高碳酸血症的化学敏感性均降低。他的肺活量和一秒用力呼气量略有下降，胸部X光片显示双侧膈肌中度抬高。因此，我们认为该病例中对低氧和高碳酸血症反应减弱是由呼吸中枢、化学感受器受损以及呼吸肌无力所致。
