RT Journal Article
SR Electronic
T1 Upper airway dysfunction in amyotrophic lateral sclerosis
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP PA4610
DO 10.1183/13993003.congress-2015.PA4610
VO 46
IS suppl 59
A1 Kaltsakas, Georgios
A1 Rentzos, Michael
A1 Alexakis, Theodoros
A1 Zouvelou, Vassiliki
A1 Palamidas, Anastasios F.
A1 Gennimata, Sofia Antiopi
A1 Xirou, Sophia
A1 Evdokimidis, Ioannis
A1 Koulouris, Nickolaos G.
YR 2015
UL http://erj.ersjournals.com/content/46/suppl_59/PA4610.abstract
AB The ALS Functional Rating Scale (ALSFRS-R) is a validated rating instrument for monitoring the progression of disability in patients with ALS. The ALSFRS-R incorporates questions for the assessment of dyspnoea, orthopnea, and the need for ventilatory support. However, studies on the relationship of ALSFRS-R with objective measurements of respiratory function are scanty. Therefore, we set out to investigate the relationship of ALSFRS-R with respiratory function indices in ALS patients.We studied 49 consecutive, ambulatory, Caucasian patients with ALS. Five patients did not satisfactorily perform the lung function testing and they were excluded. All subjects were free from any major co morbidities. ALSFRS-R was assessed. Routine lung function tests, maximum static expiratory (Pemax) and inspiratory (Pimax) mouth pressures were measured. Respiratory muscle strength (RMS) was also calculated.ALSFRS-R (mean±SD) was 34.0±8.4. Patients had: age, y=62±10, FEV1, %pred=80±21, FVC, %pred=82±21, TLC, %pred=91±14, and DLCO, %pred=86±20. Pemax, %pred was 72±30, Pimax, %pred was 65±32, and RMS, %pred was 68±29. These pressures were below the normal limits in 25, 33, and 28 patients, respectively. ALSFRS-R was significantly correlated with Pemax, %pred, RMS%, pred, FEF50/FIF50, and FEV1/FIV1 (r=0.45, p<0.01; r=0.39, p<0.01; r= -0.50, p<0.001; n=27, r= -0.63, p<0.001, respectively). These correlations were substantially closer in the subgroup of patients without bulbar involvement (r=0.74, p<0.01; r=0.78, p<0.001; r= -0.59, p=0.02; n=10, r= -0.81, p<0.01, respectively).In conclusion, FEV1/FIV1 index significantly correlates with ALSFRS-R. This correlation is substantial closer in the subgroup of ALS patients without bulbar involvement.