The ALSFRS-R: a revised ALS functional rating scale that incorporates assessments of respiratory function. BDNF ALS Study Group (Phase III)

J M Cedarbaum; N Stambler; E Malta; C Fuller; D Hilt; B Thurmond; A Nakanishi

doi:10.1016/s0022-510x(99)00210-5

The ALSFRS-R: a revised ALS functional rating scale that incorporates assessments of respiratory function. BDNF ALS Study Group (Phase III)

J Neurol Sci. 1999 Oct 31;169(1-2):13-21. doi: 10.1016/s0022-510x(99)00210-5.

Authors

J M Cedarbaum¹, N Stambler, E Malta, C Fuller, D Hilt, B Thurmond, A Nakanishi

Affiliation

¹ Regeneron Pharmaceuticals, Inc., 777 Old Saw Mill River Rd., Tarrytown, NY 10591, USA.

PMID: 10540002
DOI: 10.1016/s0022-510x(99)00210-5

Abstract

The ALS Functional Rating Scale (ALSFRS) is a validated rating instrument for monitoring the progression of disability in patients with amyotrophic lateral sclerosis (ALS). One weakness of the ALSFRS as originally designed was that it granted disproportionate weighting to limb and bulbar, as compared to respiratory, dysfunction. We have now validated a revised version of the ALSFRS, which incorporates additional assessments of dyspnea, orthopnea, and the need for ventilatory support. The Revised ALSFRS (ALSFRS-R) retains the properties of the original scale and shows strong internal consistency and construct validity. ALSFRS-R scores correlate significantly with quality 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.

Publication types

Clinical Trial
Multicenter Study
Research Support, Non-U.S. Gov't

MeSH terms

Activities of Daily Living*
Adult
Aged
Amyotrophic Lateral Sclerosis*
Disease Progression
Factor Analysis, Statistical
Female
Follow-Up Studies
Humans
Male
Middle Aged
Motor Neuron Disease / physiopathology
Quality of Life*
Respiration*
Severity of Illness Index
Survival Analysis