Sleep disorders and diaphragmatic function patients with amyotrophic lateral sclerosis
Sleep disorders and diaphragmatic function patients with amyotrophic lateral sclerosis
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DOI：
10.1164/ajrccm.161.3.9805008
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发表时间：
2000-03-01
影响因子：
24.7
通讯作者：
Derenne, JP
中科院分区：
文献类型：
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作者：
Arnulf, I;Similowski, T;Derenne, JP
在肌萎缩侧索硬化症(ALS)中，上下运动神经元的进行性丢失会导致呼吸衰竭，通常以横隔膜功能障碍为主，甚至死亡。由于横隔膜是快速眼动(REM)睡眠中唯一活跃的吸气肌，理论上ALS患者在快速眼动(REM)睡眠期间呼吸障碍的风险很高。为了评估这一假设，我们研究了21名ALS患者的睡眠特征(多导睡眠图)，根据是否存在横隔膜功能障碍进行分层。隔膜功能障碍定义为对颈部或皮质磁刺激、腹部反常或呼吸脉搏的不存在或延迟的隔膜反应(第1组，13例患者)。这些患者在年龄、临床病程或形式(球或脊柱)上与其他8名没有横隔肌功能障碍的患者没有区别(第2组)。第1组快速眼动睡眠减少(占总睡眠时间的7+/-7%；平均+/-SD)，第2组正常(18+/-6%，p=0.004)。呼吸暂停或低呼吸在两组中都很少见。在第1组中，5例患者REM睡眠缺失或极少(<3min)。在其他6名横隔膜功能障碍的患者中，在REM期间不寻常且显著地保留了吸气相的胸锁乳突肌激活与较长的REM睡眠时间有关。第1组的中位生存期(217d)明显短于第2组(619d，p=0.015)。
In amyotrophic lateral sclerosis (ALS), the progressive loss of upper and lower motor neurons leads to respiratory failure, often with predominant diaphragm dysfunction, and death. Because the diaphragm is the only active inspiratory muscle during rapid eye movement (REM) sleep, there is a high theoretical risk of respiratory disorders during REM sleep in patients with ALS. To assess this hypothesis, we studied sleep characteristics (polysomnography) in 21 patients with ALS, stratified according to the presence or absence of diaphragmatic dysfunction. Diaphragmatic dysfunction was defined as an absent or delayed diaphragm response to cervical or cortical magnetic stimulation, abdominal paradox, or respiratory pulse (Group 1, 13 patients). These patients did not differ in age, clinical course, or form (bulbar or spinal) from the eight others, who did not have diaphragmatic dysfunction (Group 2). REM sleep was reduced in Group 1 (7 +/- 7% of total sleep time; mean +/- SD) and normal in Group 2 (18 +/- 6%, p = 0.004). Apneas or hypopneas were rare in both groups. In Group 1, REM sleep was absent or minimal (less than 3 min) in five patients. An unusual and remarkable preservation of phasic inspiratory sternomastoid activation during REM was associated with longer REM sleep duration in six of the other patients with diaphragmatic dysfunction. Median survival time was dramatically shorter (217 d) in Group 1 than in Group 2 (619 d, p = 0.015).