ALS is a progressive neurodegenerative disease. The stage of disease reached can be described using a simple system based on the number of central nervous system regions involved. Historically, datasets have not attempted to record clinical stage, but being able to re-analyse the data by stage would have several advantages. We therefore explored the possibility of using an algorithm based on the revised ALS Functional Rating Scale (ALSFRS-R), which is commonly used in clinical practice, to estimate clinical stage. We devised an algorithm to convert ALSFRS-R score into clinical stage. ALSFRS-R domains were mapped to equivalent CNS regions. Stage 4 is reached when gastrostomy or non- invasive ventilation is needed, but as a proxy we used provision. We collected ALSFRS-R from clinic visits, and compared the estimation of clinical stage from the ALSFRS-R with the actual stage. Results showed that the agreement between staging by the two methods was excellent with an intraclass correlation coefficient of 0.92 (95
作者:Rubika, Balendra;Ashley, Jones;Naheed, Jivraj;Catherine, Knights;Catherine M, Ellis;Rachel, Burman;Martin R, Turner;P Nigel, Leigh;Christopher E, Shaw;Ammar, Al-Chalabi
来源:Amyotrophic lateral sclerosis & frontotemporal degeneration 2014 年 15卷 3-4期