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表现为睡眠呼吸暂停低通气综合征的肌萎缩侧索硬化症

Amyotrophic lateral sclerosis presenting with sleep hypopnea syndrome.

作者信息

Carre P C, Didier A P, Tiberge Y M, Arbus L J, Leophonte P J

机构信息

Unit of Respiratory Diseases and Allergology, Hôpital Purpan, Toulouse, France.

出版信息

Chest. 1988 Jun;93(6):1309-12. doi: 10.1378/chest.93.6.1309.

DOI:10.1378/chest.93.6.1309
PMID:3371113
Abstract

Described is a 67-year-old man whose initial symptoms evoked an obesity-hypoventilation syndrome. Polysomnography showed hypopneas associated with O2 desaturation episodes, and no apnea; maximal changes were noted during REM sleep. A few months later, in spite of marked weight loss, acute alveolar hypoventilation occurred and necessitated mechanical ventilatory support. Tracheostomy was performed. The patient appeared to be dependent on nocturnal ventilatory assistance. Diaphragmatic paralysis was noted in addition to clinical and electrodiagnostic evidence of amyotrophic lateral sclerosis. While the patient was not ventilated, a nocturnal recording of SaO2 again revealed desaturation episodes partly corrected by O2 2 L/min administered through the tracheostomy tube. With volume-controlled ventilation, desaturations completely disappeared, although no oxygen enrichment of the air was provided. We speculate that sleep disorders with hypopneas and O2 desaturation episodes were the initial symptoms of amyotrophic lateral sclerosis. This leads us to suggest that nonspecific respiratory muscle fatigue frequently seen in COPD might be included in the hypothetic causes of nocturnal hypoxemia.

摘要

报告了一名67岁男性,其最初症状提示肥胖低通气综合征。多导睡眠图显示低通气与氧饱和度下降发作相关,无呼吸暂停;快速眼动睡眠期变化最为明显。几个月后,尽管体重显著减轻,但仍发生急性肺泡低通气,需要机械通气支持。进行了气管切开术。患者似乎依赖夜间通气辅助。除了肌萎缩侧索硬化的临床和电诊断证据外,还发现了膈肌麻痹。在患者未通气时,夜间记录的动脉血氧饱和度再次显示氧饱和度下降发作,通过气管切开套管给予2 L/min的氧气可部分纠正。采用容量控制通气时,尽管未提供富氧空气,但氧饱和度下降完全消失。我们推测,伴有低通气和氧饱和度下降发作的睡眠障碍是肌萎缩侧索硬化的初始症状。这使我们认为,慢性阻塞性肺疾病中常见的非特异性呼吸肌疲劳可能是夜间低氧血症的假设原因之一。

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引用本文的文献

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Amyotrophic lateral sclerosis presenting respiratory failure as the sole initial manifestation.以呼吸衰竭为唯一首发表现的肌萎缩侧索硬化症。
Case Rep Neurol. 2014 Aug 14;6(2):213-6. doi: 10.1159/000366191. eCollection 2014 May.