Veale D, Cooper B G, Gilmartin J J, Walls T J, Griffith C J, Gibson G J
Dept of Respiratory Medicine, Freeman Hospital, Newcastle-upon-Tyne, UK.
Eur Respir J. 1995 May;8(5):815-8.
Patients with myotonic dystrophy often have an irregular pattern of breathing at rest, implying abnormality of breathing control. No central medullary defect has been found in such patients. We postulated that irregular breathing in myotonic dystrophy due to abnormal central respiratory output would persist during slow-wave sleep. We examined the patterns of breathing whilst awake and asleep in seven patients with myotonic dystrophy, seven similarly weak nonmyotonic subjects and seven normal controls. Polysomnography was performed, and the coefficients of variation (CoV) of the breath intervals were analysed during different stages of sleep. The myotonic group showed significantly greater variation in breath intervals than the other two groups whilst awake (median CoV 37 vs 18% for nonmyotonics) and during light sleep (31 vs 13%). This difference was not evident during slow-wave sleep (median CoV 12 vs 9% in nonmyotonic). We conclude that irregular breathing in patients with myotonic dystrophy whilst awake and during light sleep, does not persist during slow-wave sleep. These results suggest that "behavioural" influences play a role in the abnormal breathing pattern found in myotonic dystrophy. The source of the irregular breathing is unlikely to be found in the medulla, but may originate from forebrain influences.
强直性肌营养不良患者在静息状态下常出现不规则呼吸模式，这意味着呼吸控制存在异常。在这类患者中未发现中枢延髓缺陷。我们推测，由于中枢呼吸输出异常导致的强直性肌营养不良患者的不规则呼吸在慢波睡眠期间会持续存在。我们检查了7例强直性肌营养不良患者、7例同样虚弱的非强直性肌营养不良受试者和7例正常对照者在清醒和睡眠状态下的呼吸模式。进行了多导睡眠图检查，并分析了睡眠不同阶段呼吸间隔的变异系数（CoV）。强直性肌营养不良组在清醒时（非强直性肌营养不良组的CoV中位数为37%对18%）和浅睡眠期间（31%对13%），呼吸间隔的变异明显大于其他两组。在慢波睡眠期间这种差异不明显（非强直性肌营养不良组的CoV中位数为12%对9%）。我们得出结论，强直性肌营养不良患者在清醒和浅睡眠期间的不规则呼吸在慢波睡眠期间不会持续存在。这些结果表明，“行为”影响在强直性肌营养不良患者异常呼吸模式中起作用。不规则呼吸的根源不太可能在延髓找到，而可能源于前脑的影响。
