Gugger M
Pneumologische Abteilung Medizinischen Universitätsklinik, Inselspital, Bern.
Ther Umsch. 1990 Nov;47(11):878-84.
In end stage neuromuscular disease respiratory muscle fatigue can lead to respiratory failure. There is no close correlation between the degree of peripheral muscle weakness and the weakness of the respiratory muscles. Early in the disease the maximum in- and expiratory pressures can be abnormal even though the results of spirometry are normal. Progressive weakness of the respiratory muscles results in a decrease in vital capacity and eventually in alveolar hypoventilation, especially during sleep. General muscle weakness and fatigue may be the reasons why sleep hypoventilation is often overlooked in its early stages. Dyspnea may be a early symptom but it is not unusual that alveolar hypoventilation remains undetected until complications ensue (Cor pulmonale, polycythemia, altered alertness).
在终末期神经肌肉疾病中，呼吸肌疲劳可导致呼吸衰竭。外周肌肉无力的程度与呼吸肌无力之间没有密切关联。在疾病早期，即使肺活量测定结果正常，最大吸气和呼气压力也可能异常。呼吸肌进行性无力导致肺活量下降，并最终导致肺泡通气不足，尤其是在睡眠期间。全身肌肉无力和疲劳可能是睡眠通气不足在早期常被忽视的原因。呼吸困难可能是早期症状，但在并发症出现（肺心病、红细胞增多症、意识改变）之前，肺泡通气不足仍未被发现的情况并不少见。
