Carroll J E, Zwillich C W, Weil J V
Neurology. 1977 Dec;27(12):1125-8. doi: 10.1212/wnl.27.12.1125.
Patients with myotonic dystrophy often develop respiratory failure caused by alveolar hypoventilation. Abnormalities in the ventilatory response to hypoxia and hypercapnia may explain this phenomenon. Accordingly, hypoxic and hypercapnic responses were measured in seven patients with myotonic dystrophy who had only mild respiratory muscle weakness. Hypoxic response was significantly reduced, while hypercapnic response was affected more irregularly. It is possible that the high incidence of respiratory failure in such patients is related to decreased hypoxic ventilatory response, occurring because of an underlying neurogenic deficit.
强直性肌营养不良患者常因肺泡通气不足而发生呼吸衰竭。对低氧和高碳酸血症的通气反应异常可能解释了这一现象。因此，对7例仅有轻度呼吸肌无力的强直性肌营养不良患者进行了低氧和高碳酸血症反应的测量。低氧反应显著降低，而高碳酸血症反应的影响则更不规则。此类患者呼吸衰竭的高发生率可能与潜在神经源性缺陷导致的低氧通气反应降低有关。
