Cirignotta F, Mondini S, Zucconi M, Barrot-Cortes E, Sturani C, Schiavina M, Coccagna G, Lugaresi E
Institute of Neurology, University of Bologna, Italy.
J Neurol. 1987 Dec;235(2):80-5. doi: 10.1007/BF00718014.
Respiratory failure has been described in myotonic dystrophy; it worsens during sleep but its central or peripheral origin has yet to be determined. Moreover, patients may present severely disturbed sleep and daytime somnolence. Eight patients with mild to moderate myotonic dystrophy were studied to assess breathing function while awake and during sleep by means of the pulmonary function tests, nocturnal polysomnographic examination and the multiple sleep latency test (MSLT). Three patients had restrictive respiratory defects; none had signs of airway obstruction. All patients had very disrupted nocturnal sleep. Of six patients who underwent the MSLT only two showed a mild tendency to sleep during the day. Six patients had pathological apnoea plus hypopnoea index [(A+H)I] and there was a prevalence of central apnoeas. The apnoeas occurred while resting but awake and throughout all sleep stages. Only two patients (the ones with the least vital capacity) had episodes of progressive oxygen desaturation during rapid eye movement sleep, similar to those found in other restrictive disorders and in chronic obstructive pulmonary disease. It is concluded that the breathing pattern characteristic of our myotonic dystrophy patients was the occurrence of central apnoeas both at rest while awake and during sleep.
呼吸衰竭在强直性肌营养不良中已有描述；它在睡眠期间会恶化，但其起源是中枢性还是外周性尚未确定。此外，患者可能会出现严重的睡眠障碍和日间嗜睡。对8例轻至中度强直性肌营养不良患者进行了研究，通过肺功能测试、夜间多导睡眠图检查和多次睡眠潜伏期试验（MSLT）来评估其清醒和睡眠期间的呼吸功能。3例患者存在限制性呼吸缺陷；无一例有气道阻塞的迹象。所有患者夜间睡眠均严重紊乱。在接受MSLT的6例患者中，只有2例表现出轻度的日间嗜睡倾向。6例患者的病理性呼吸暂停加呼吸不足指数[（A+H）I]升高，且以中枢性呼吸暂停为主。呼吸暂停在静息但清醒时以及整个睡眠阶段均会出现。只有2例患者（肺活量最小的患者）在快速眼动睡眠期间出现进行性氧饱和度下降，类似于在其他限制性疾病和慢性阻塞性肺疾病中发现的情况。结论是，我们的强直性肌营养不良患者的呼吸模式特征是在静息清醒时和睡眠期间均出现中枢性呼吸暂停。
