Background: Phrenic nerve palsy leads to disfunction of the main respi
ratory muscle. With bilateral palsy dyspnoea in the supine body positi
on will typically occur. With one-sided lesion symptoms will only appe
ar when a second pulmonary disease is present. Patients: We refer of 6
patients with bilateral and 5 with unilateral diaphragmatic paresis.
In 3 patients neuralgic shoulder-arm-amyotrophy was diagnosed, in furt
her 4 there was suspicion of it. Amyotrophic lateral sclerosis develop
ed in 2 after 4 respectively 8 months. In 1 case a cervical operation
led to palsy, mediascinitis in 1 case. Lung function tests showed a re
strictive pattern, especially in bilateral palsy. Results: Vital capac
ity was reduced by almost 50% respectively 25% in the supine body posi
tion. Mouth occlusion pressure reduction was apparent. Near normal to
marked hyperkapnia developed in bilateral paresis. In 5 cases non-inva
sive intermittent ventilation is instituted (2 volume controlled, 3 pr
essure supported). Two patients died (1 patient with amyotrophic later
al sclerosis after 13 months, 1 with malignant pleurisy after 4 months
). Three patients continued non-invasive intermittent ventilation sinc
e 14 to 64 months.