The ALSFRS-R: a revised ALS functional rating scale that incorporates assessments of respiratory function

The ALS Functional Rating Scale (ALSFRS) is a validated rating instrument f or monitoring the progression of disability in patients with amyotrophic la teral sclerosis (ALS). One weakness of the ALSFRS as originally designed wa s that it granted disproportionate weighting to limb and bulbar, as compare d to respiratory, dysfunction. We have now validated a revised version of t he ALSFRS, which incorporates additional assessments of dyspnea, orthopnea, and the need for ventilatory support. The Revised ALSFRS (ALSFRS-R) retain s the properties of the original scale and shows strong internal consistenc y and construct validity. ALSFRS-R scores correlate significantly with qual ity of life as measured by the Sickness Impact Profile, indicating that the quality of function is a strong determinant of quality of life in ALS. (C) 1999 Elsevier Science B.V. All rights reserved.