Correspondence: M. de Carvalho, Institute of Physiology, Faculty of Medicine, University of Lisbon, Av. Professor Egas Moniz, 1648-028 Lisbon, Portugal. Fax: 351 21 7805219. E-mail: mamedemg@gmail.com (Received 12 April 2014; accepted 4 August 2014) Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 2014; Early Online: 1–4 ISSN 2167-8421 print/ISSN 2167-9223 online © 2014 Informa Healthcare DOI: 10.3109/21678421.2014.952641 In our clinic we have been using the respiratory subscore of the revised version of the ALS functional rating scale (ALSFRS-R) (6) to assess respiratory functionality in ALS. It incorporates assessments of dyspnoea (1st respiratory question, QR1), ortho- pnoea (2nd respiratory question, QR2) and the need for ventilatory support (3rd respiratory question, QR3) to the initial ALSFRS scale. Thereby, and according to the authors of the revised version, it compensates the disproportionate weighting of limb and bulbar dysfunction of the original scale (6). In the present work we aim to test the utility of the respiratory subscore and its individual questions in evaluating respiratory functionality in ALS patients. Methodology Consecutive ALS patients observed in our Unit from August 2000 were included in this study. Patients with other medical conditions including diabetes, lung disorders or dementia, as well as other causes of motor impairment were excluded. ALSFRS and its bulbar (ALSFRSb), upper limb (ALSFRSul) and lower limb (ALSFRSll) scores, as well as the Introduction Respiratory insufficiency (RI) in amyotrophic lateral sclerosis (ALS) is usually a late event during the course of the disease, although it can rarely be the presenting feature (1). Respiratory infections, aspira- tion pneumonia and bronchial impaction as well as sudden death account for the majority of deaths in ALS (2). With the advent of non-invasive ventilation (NIV), patients survive longer and, especially, with a better quality of life (3). In addition, cough aids, as mechanical insufflation-exsufflation, increase patients’ comfort (2,3). Recent trials reflect the need for early inclusion of ALS patients, when symptoms of motor neuron demise are moderate and respiratory function is normal or mildly affected. Moreover, respiratory exercise can provide benefit when the respiratory function is normal (4,5). Respiratory evaluation is, therefore, particularly important and should be assessed at diagnosis and every three months thereafter, or sooner if rapid respiratory deteriora- tion emerges or in the presence of respiratory complications (3). REPORT The R of ALSFRS-R: Does it really mirror functional respiratory involvement in amyotrophic lateral sclerosis? SUSANA PINTO 1 & MAMEDE DE CARVALHO 1,2 1 Institute of Physiology and Institute of Molecular Medicine, Faculty of Medicine, University of Lisbon, and 2 Department of Neurosciences, Hospital de Santa Maria-CHLN, Lisbon, Portugal Abstract Clinical assessment of the respiratory function is critical in amyotrophic lateral sclerosis (ALS). A standardized process is to monitor the respiratory subscore of the revised ALSFRS (RofALSFRS-R). We tested the utility of RofALSFRS-R and its individual questions in evaluating respiratory functionality. Three hundred and fifty-seven consecutive ALS patients were assessed at entry (T0), three and six months later (T1 and T2). ALSFRS and its subscores decayed significantly (p  0.001) over time. Improvement in the first respiratory question (dyspnoea, QR1) was found in about 10% of the patients in each period. Between T0 and T1 a significant negative correlation was found between decrease in gait score and QR1 (p  0.021, r  –0.395) in the subgroup of ALS patients who showed QR1 improvement (n  34). An improve- ment in the other respiratory questions was noticed in about 6% of the patients, related to non-invasive ventilation. Possibly, decreased mobility and metabolic demand can cause fewer respiratory symptoms in a subset of patients. The respiratory questions included in ALSFRS-R should be re-addressed by the ALS community. Key words: ALSFRS-R, amyotrophic lateral sclerosis, functional scale, motor neuron disease, respiratory function Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration Downloaded from informahealthcare.com by 85.138.86.208 on 09/10/14 For personal use only.