RT Journal Article
SR Electronic
T1 Orthopnea due to phrenic nerve involvement in neuralgic amyothrophy
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP P452
VO 44
IS Suppl 58
A1 R.T.M. Sprooten
A1 A.A.B. Otte
A1 N.A.M. Cobben
A1 S.C.M. Steijns
A1 G.G.U. Rohde
A1 E.F.M. Wouters
YR 2014
UL http://erj.ersjournals.com/content/44/Suppl_58/P452.abstract
AB Introduction: Phrenic nerve involvement in uni- or bilateral diaphragm palsy is a rare feature in patients with neuralgic amyotrophy (NA). Features of respiratory failure and usefulness of non-invasive ventilation (NIV) in NA are scarcely reported.Methods: We describe the respiratory findings of 11 patients (9 male, mean age 54y; 38-72) with NA and uni- (n=4 right, 1 left sided) or bilateral (n=6) diaphragm involvement who presented since 2008 consecutively.Results: All patients presented with orthopnea, were sleeping in sitting position and had morning and daytime fatigue. Lung function (LF) was done in sitting and supine position, only 2 patients in sitting position due to orthopnea. Mean FVC sitting position was 57 %predicted (SD 15.5), FVC supine position 36 %predicted (SD 15.9), FVC delta%(sit-sup) 43% (SD 16.2). 3 patients had daytime hypercapnia. Nocturnal hypoventilation was found in 9 patients: mean pCO2 6.6 kPa (5.5-7.7), bicarbonate 28.2 mmol/l (23.2-32,1), SpO2 91.8% (89-95). We initiated NIV because of severe symptoms (orthopnea) or nocturnal hypoventilation. One patient did not tolerate NIV. The remaining ten experienced no orthopnea during sleep, better daytime functioning and in most patients the nocturnal hypoventilation improved. After 2 years 1 patient recovered enough to sleep in supine position without NIV. The longest follow-up was up to 5 years with still good results.Conclusion: Orthopnea and necessity to sleep in supine position associated with a decline of greater than 20% of FVC in supine position strongly suggests phrenic nerve involvement with nocturnal hypovnentilation. The initiation of NIV in these patients can lead to improved nocturnal hypoventilation and day time activities.