PT  - JOURNAL ARTICLE
AU  - Georgios Kaltsakas
AU  - Michael Rentzos
AU  - Theodoros Alexakis
AU  - Vassiliki Zouvelou
AU  - Anastasios F. Palamidas
AU  - Sofia Antiopi Gennimata
AU  - Sophia Xirou
AU  - Ioannis Evdokimidis
AU  - Nickolaos G. Koulouris
TI  - Upper airway dysfunction in amyotrophic lateral sclerosis
AID  - 10.1183/13993003.congress-2015.PA4610
DP  - 2015 Sep 01
TA  - European Respiratory Journal
PG  - PA4610
VI  - 46
IP  - suppl 59
4099  - http://erj.ersjournals.com/content/46/suppl_59/PA4610.short
4100  - http://erj.ersjournals.com/content/46/suppl_59/PA4610.full
SO  - Eur Respir J2015 Sep 01; 46
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.