Estimating clinical stage of amyotrophic lateral sclerosis from the ALS Functional Rating Scale.

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...

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Main Authors: Balendra, R, Jones, A, Jivraj, N, Knights, C, Ellis, C, Burman, R, Turner, M, Leigh, P, Shaw, C, Al-Chalabi, A
Format: Journal article
Language:English
Published: 2014
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author Balendra, R
Jones, A
Jivraj, N
Knights, C
Ellis, C
Burman, R
Turner, M
Leigh, P
Shaw, C
Al-Chalabi, A
author_facet Balendra, R
Jones, A
Jivraj, N
Knights, C
Ellis, C
Burman, R
Turner, M
Leigh, P
Shaw, C
Al-Chalabi, A
author_sort Balendra, R
collection OXFORD
description 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% confidence interval 0.88-0.94). There was no systematic bias towards over-staging or under-staging using the algorithm. In conclusion, we have shown that clinical stage in ALS can be reliably estimated using the ALSFRS-R in historical data and in current data where stage has not been recorded.
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spelling oxford-uuid:49a1d342-4f9c-44ff-b571-b362b40ad94d2022-03-26T15:32:43ZEstimating clinical stage of amyotrophic lateral sclerosis from the ALS Functional Rating Scale.Journal articlehttp://purl.org/coar/resource_type/c_dcae04bcuuid:49a1d342-4f9c-44ff-b571-b362b40ad94dEnglishSymplectic Elements at Oxford2014Balendra, RJones, AJivraj, NKnights, CEllis, CBurman, RTurner, MLeigh, PShaw, CAl-Chalabi, AALS 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% confidence interval 0.88-0.94). There was no systematic bias towards over-staging or under-staging using the algorithm. In conclusion, we have shown that clinical stage in ALS can be reliably estimated using the ALSFRS-R in historical data and in current data where stage has not been recorded.
spellingShingle Balendra, R
Jones, A
Jivraj, N
Knights, C
Ellis, C
Burman, R
Turner, M
Leigh, P
Shaw, C
Al-Chalabi, A
Estimating clinical stage of amyotrophic lateral sclerosis from the ALS Functional Rating Scale.
title Estimating clinical stage of amyotrophic lateral sclerosis from the ALS Functional Rating Scale.
title_full Estimating clinical stage of amyotrophic lateral sclerosis from the ALS Functional Rating Scale.
title_fullStr Estimating clinical stage of amyotrophic lateral sclerosis from the ALS Functional Rating Scale.
title_full_unstemmed Estimating clinical stage of amyotrophic lateral sclerosis from the ALS Functional Rating Scale.
title_short Estimating clinical stage of amyotrophic lateral sclerosis from the ALS Functional Rating Scale.
title_sort estimating clinical stage of amyotrophic lateral sclerosis from the als functional rating scale
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