Estimating clinical stage of amyotrophic lateral sclerosis from the ALS functional rating scale

Balendra, Rubika, Jones, Ashley, Jivraj, Naheed, Knights, Catherine, Ellis, Catherine M, Burman, Rachel, Turner, Martin R, Leigh, Nigel, Shaw, Christopher E and Al-Chalabi, Ammar (2014) Estimating clinical stage of amyotrophic lateral sclerosis from the ALS functional rating scale. Amyotrophic Lateral Sclerosis and Frontotemporal Degeneration, 15 (3-4). pp. 279-284. ISSN 2167-8421

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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.

Item Type: Article
Schools and Departments: Brighton and Sussex Medical School > Neuroscience
Subjects: R Medicine
R Medicine > RC Internal medicine > RC0321 Neurosciences. Biological psychiatry. Neuropsychiatry > RC0346 Neurology. Diseases of the nervous system Including speech disorders
Depositing User: Patricia Butler
Date Deposited: 25 Aug 2015 12:36
Last Modified: 21 Oct 2019 14:02
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