Haas H, Johnson L R, Gill T H, Armentrout T S
Am Rev Respir Dis. 1981 Apr;123(4 Pt 1):465-7. doi: 10.1164/arrd.1981.123.4.465.
Ventilatory failure occurred in a patient suffering from proximal neurogenic muscular atrophy resembling the adult form of Kugelberg-Welander syndrome. Diaphragmatic paralysis was diagnosed when patient was found to be unable to generate a transdiaphragmatic pressure difference on deep inspiration. Post-mortem examination revealed nearly complete replacement of the diaphragm by fat and fibrous tissue. Although uncommonly reported in the medical literature, ventilatory failure secondary to diaphragm dysfunction should be added to the list of complications encountered in the clinical setting of chronic proximal spinal muscular atrophy.
一名患有类似成人型库格尔贝格 - 韦兰德综合征的近端神经源性肌肉萎缩的患者发生了呼吸衰竭。当发现患者在深吸气时无法产生跨膈压差时，诊断为膈肌麻痹。尸检显示膈肌几乎完全被脂肪和纤维组织替代。尽管医学文献中对此报道较少，但在慢性近端脊髓性肌萎缩的临床情况下，应将继发于膈肌功能障碍的呼吸衰竭列入所遇到的并发症清单中。
