Skip to main content
Key Specialties
Cardiology Diabetology Endocrinology Gastroenterology Geriatrics and Gerontology Gynecology and Obstetrics Hematology Infectious Disease Internal Medicine Nephrology Neurology Oncology Pediatrics Respiratory Medicine Rheumatology Surgery
Congress Coverage
2024 ACC Congress 2023 ESMO Congress 2023 EASD Congress 2023 ERS Congress 2023 ESC Congress
Clinical Collections
Advances in Alzheimer’s

At a glance: The ONWARDS insulin icodec trials

A round-up of the ONWARDS phase 3 clinical trials evaluating the novel weekly insulin icodec in people with diabetes.
ADVANCED SEARCH
Log in
Top
Neurological Research and Practice
Published in: Neurological Research and Practice 1/2022

Open Access 01-12-2022 | Amyotrophic Lateral Sclerosis | Research Article

ALSFRS-R-SE: an adapted, annotated, and self-explanatory version of the revised amyotrophic lateral sclerosis functional rating scale

Authors: André Maier, Matthias Boentert, Peter Reilich, Simon Witzel, Susanne Petri, Julian Großkreutz, Moritz Metelmann, Paul Lingor, Isabell Cordts, Johannes Dorst, Daniel Zeller, René Günther, Tim Hagenacker, Torsten Grehl, Susanne Spittel, Joachim Schuster, Albert Ludolph, Thomas Meyer, for the MND-NET consensus group

Published in: Neurological Research and Practice | Issue 1/2022

Login to get access

Abstract

Background

The ALS Functional Rating Scale in its revised version (ALSFRS-R) is a disease-specific severity score that reflects motor impairment and functional deterioration in people with amyotrophic lateral sclerosis (ALS). It has been widely applied in both clinical practice and ALS research. However, in Germany, several variants of the scale, each differing slightly from the others, have developed over time and are currently in circulation. This lack of uniformity potentially hampers data interpretation and may decrease item validity. Furthermore, shortcomings within the standard ALSFRS-R questions and answer options can limit the quality and conclusiveness of collected data.

Methods

In a multistage consensus-building process, 18 clinical ALS experts from the German ALS/MND network analyzed the ALSFRS-R in its current form and created an adapted, annotated, and revised scale that closely adheres to the well-established standardized English version.

Results

Ten German-language variants of the ALSFRS-R were collected, three of which contained instructions for self-assessment. All of these variants were compiled and a comprehensive linguistic revision was undertaken. A short introduction was added to the resulting scale, comprising general instructions for use and explanations for each of the five reply options per item. This adapted version of the scale, named ALSFRS-R-SE (with the “SE” referring to “self-explanatory”), was carefully reviewed for language and comprehensibility, in both German and English.

Conclusion

An adapted and annotated version of the ALSFRS-R scale was developed through a multistage consensus process. The decision to include brief explanations of specific scale items and reply options was intended to facilitate ALSFRS-R-SE assessments by both healthcare professionals and patients. Further studies are required to investigate the accuracy and utility of the ALSFRS-R-SE in controlled trials and clinical real-world settings.
Appendix
Available only for authorised users
Literature
1.
Cedarbaum, J. M., Stambler, N., Malta, E., Fuller, C., Hilt, D., Thurmond, B., & Nakanishi, A. (1999). The ALSFRS-R: a revised ALS functional rating scale that incorporates assessments of respiratory function. J Neurol Sci, 169(1–2), 13–21.CrossRef
2.
Bakker, L. A., Schröder, C. D., van Es, M. A., Westers, P., Visser-Meily, J. M. A., & van den Berg, L. H. (2017). Assessment of the factorial validity and reliability of the ALSFRS-R: a revision of its measurement model. J Neurol, 264(7), 1413–1420.CrossRef
3.
Kaufmann, P. (1996). The amyotrophic lateral sclerosis functional rating scale. Arch Neurol, 53(2), 555.
4.
Kaufmann, P., Levy, G., Montes, J., Buchsbaum, R., Barsdorf, A. I., Battista, V., Arbing, R., Gordon, P. H., Mitsumoto, H., Levin, B., Thompson, J. L. P., Kaufmann, P., Levy, G., Montes, J., Buchsbaum, R., Barsdorf, A. I., Battista, V., Arbing, R., Gordon, P. H., … Thompson, J. L. P. (2009). Excellent inter-rater, intra-rater, and telephone-administered reliability of the ALSFRS-R in a multicenter clinical trial. Amyotroph Lateral Scler, 8(1), 42–46.CrossRef
5.
Gordon, P. H., Miller, R. G., & Moore, D. H. (2009). Alsfrs-R. Amyotroph Lateral Scler Other Motor Neuron Disord, 5(sup1), 90–93.CrossRef
6.
Chew, S., Burke, K. M., Collins, E., Church, R., Paganoni, S., Nicholson, K., Babu, S., Scalia, J. B., De Marchi, F., Ellrodt, A. L., Moura, L. M. V. R., Chan, J., & Berry, J. D. (2021). Patient reported outcomes in ALS: characteristics of the self-entry ALS functional rating scale-revised and the activities-specific balance confidence scale. Amyotroph Lateral Scler Front Degener, 22(7–8), 467–477.CrossRef
7.
Kasarskis, E. J., Dempsey-Hall, L., Thompson, M. M., Luu, L. C., Mendiondo, M., & Kryscio, R. (2009). Rating the severity of ALS by caregivers over the telephone using the ALSFRS-R. Amyotroph Lateral Scler, 6(1), 50–54.CrossRef
8.
Montes, J., Levy, G., Albert, S., Kaufmann, P., Buchsbaum, R., Gordon, P. H., & Mitsumoto, H. (2006). Development and evaluation of a self-administered version of the ALSFRS-R. Neurology, 67(7), 1294–1296.CrossRef
9.
Maier, A., Holm, T., Wicks, P., Steinfurth, L., Linke, P., Münch, C., Meyer, R., & Meyer, T. (2012). Online assessment of ALS functional rating scale compares well to in-clinic evaluation: a prospective trial. Amyotroph Lateral Scler, 13(2), 210–216.CrossRef
10.
McElhiney, M., Rabkin, J. G., Goetz, R., Katz, J., Miller, R. G., Forshew, D. A., David, W., Cudkowicz, M., Glass, J. D., Appel, S., Simpson, E., & Mitsumoto, H. (2014). Seeking a measure of clinically meaningful change in ALS. Amyotroph Lateral Scler Front Degener, 15(5–6), 398–405.CrossRef
11.
Prell, T., Gaur, N., Steinbach, R., Witte, O. W., & Grosskreutz, J. (2020). Modelling disease course in amyotrophic lateral Sclerosis: pseudo-longitudinal insights from cross-sectional health-related quality of life data. Health Quality Life Outcomes, 18(1), 569.CrossRef
12.
Westeneng, H.-J., Debray, T. P. A., Visser, A. E., van Eijk, R. P. A., Rooney, J. P. K., Calvo, A., Martin, S., McDermott, C. J., Thompson, A. G., Pinto, S., Kobeleva, X., Rosenbohm, A., Stubendorff, B., Sommer, H., Middelkoop, B. M., Dekker, A. M., van Vugt, J. J. F. A., van Rheenen, W., Vajda, A., … van den Berg, L. H. (2018). Prognosis for patients with amyotrophic lateral sclerosis: development and validation of a personalised prediction model. Lancet Neurol, 17(5), 423–433.CrossRef
13.
Kimura, F., Fujimura, C., Ishida, S., Nakajima, H., Furutama, D., Uehara, H., Shinoda, K., Sugino, M., & Hanafusa, T. (2006). Progression rate of ALSFRS-R at time of diagnosis predicts survival time in ALS. Neurology, 66(2), 265–267.CrossRef
14.
Kiernan, M. C., Vucic, S., Talbot, K., McDermott, C. J., Hardiman, O., Shefner, J. M., Al-Chalabi, A., Huynh, W., Cudkowicz, M., Talman, P., Van den Berg, L. H., Dharmadasa, T., Wicks, P., Reilly, C., & Turner, M. R. (2020). Improving clinical trial outcomes in amyotrophic lateral sclerosis. Nat Rev Neurol, 17(2), 104–118.CrossRef
15.
Fang, T., Al Khleifat, A., Stahl, D. R., La Lazo, T. C., Murphy, C., Young, C., Shaw, P. J., Leigh, P. N., & Al-Chalabi, A. (2017). Comparison of the King’s and MiToS staging systems for ALS. Amyotroph Lateral Scler Front Degener, 18(3–4), 227–32.CrossRef
16.
Crockford, C., Newton, J., Lonergan, K., Chiwera, T., Booth, T., Chandran, S., Colville, S., Heverin, M., Mays, I., Pal, S., Pender, N., Pinto-Grau, M., Radakovic, R., Shaw, C. E., Stephenson, L., Swingler, R., Vajda, A., Al-Chalabi, A., Hardiman, O., & Abrahams, S. (2018). ALS-specific cognitive and behavior changes associated with advancing disease stage in ALS. Neurology, 91(15), e1370–e1380.CrossRef
17.
Jenkinson, R. F. C. B. C. (2009). Evidence for the validity and reliability of the ALS assessment questionnaire: the ALSAQ-40. Amyotroph Lateral Scler Other Motor Neuron Disord., 1(1), 33–40.CrossRef
18.
Peters, N., Dal Bello-Haas, V., Packham, T., Chum, M., O’Connell, C., Johnston, W. S., MacDermid, J., Turnbull, J., Van Damme, J., & Kuspinar, A. (2021). Do generic preference-based measures accurately capture areas of health-related quality of life important to individuals with amyotrophic lateral sclerosis: a content validation study. Patient Related Outcome Meas, 12, 191–203.CrossRef
19.
Bakker, L. A., Schröder, C. D., Tan, H. H. G., Vugts, S. M. A. G., van Eijk, R. P. A., van Es, M. A., Visser-Meily, J. M. A., & van den Berg, L. H. (2020). Development and assessment of the inter-rater and intra-rater reproducibility of a self-administration version of the ALSFRS-R. J Neurol Neurosurg Psychiatry., 91(1), 75–81.CrossRef
20.
Voustianiouk, A., Seidel, G., Panchal, J., Sivak, M., Czaplinski, A., Yen, A., Appel, S. H., & Lange, D. J. (2008). ALSFRS and appel ALS scores: discordance with disease progression. Muscle Nerve., 37(5), 668–672.CrossRef
21.
Koc, G. G., Dagsuyu, C., Kokangul, A., & Koc, F. (2021). Evaluation of ALSFRS-R scale with fuzzy method in amyotrophic lateral sclerosis. Arch Neuropsych, 2, 966.
22.
Bakers, J. N. E., de Jongh, A. D., Bunte, T. M., Kendall, L., Han, S. S., Epstein, N., Lavrov, A., Beelen, A., Visser-Meily, J. M. A., van den Berg, L. H., & van Eijk, R. P. A. (2021). Using the ALSFRS-R in multicentre clinical trials for amyotrophic lateral sclerosis: potential limitations in current standard operating procedures. Amyotroph Lateral Scler Front Degener, 2, 1–8.
23.
Pinto, S., & de Carvalho, M. (2014). The R of ALSFRS-R: does it really mirror functional respiratory involvement in amyotrophic lateral sclerosis? Amyotroph Lateral Scler Front Degener, 16(1–2), 120–123.
24.
Theme 09-CLINICAL TRIALS AND TRIAL DESIGN. Amyotroph Lateral Scler Front Degener. 2021;22(sup2):150–73.
25.
Franchignoni, F., Mandrioli, J., Giordano, A., & Ferro, S. (2015). A further Rasch study confirms that ALSFRS-R does not conform to fundamental measurement requirements. Amyotroph Lateral Scler Front Degener., 16(5–6), 331–337.CrossRef
26.
Rooney, J., Burke, T., Vajda, A., Heverin, M., & Hardiman, O. (2017). What does the ALSFRS-R really measure? A longitudinal and survival analysis of functional dimension subscores in amyotrophic lateral sclerosis. J Neurol Neurosurg Psychiatry, 88(5), 381–385.CrossRef
27.
Abdulla, S., Vielhaber, S., Körner, S., Machts, J., Heinze, H.-J., Dengler, R., & Petri, S. (2013). Validation of the German version of the extended ALS functional rating scale as a patient-reported outcome measure. J Neurol, 260(9), 2242–2255.CrossRef
28.
Wicks, P., Massagli, M. P., Wolf, C., & Heywood, J. (2009). Measuring function in advanced ALS: validation of ALSFRS-EX extension items. Eur J Neurol, 16(3), 353–359.CrossRef
29.
Campos, T. S., Rodríguez-Santos, F., Esteban, J., Vázquez, P. C., Mora Pardina, J. S., & Carmona, A. C. (2010). Spanish adaptation of the revised amyotrophic lateral sclerosis functional rating scale (ALSFRS-R). Amyotroph Lateral Scler, 11(5), 475–477.CrossRef
30.
Koc, F., Balal, M., Demir, T., Alparslan, Z. N., & Sarica, Y. (2016). Adaptation to Turkish and reliability study of the revised amyotrophic lateral sclerosis functional rating scale (ALSFRS-R). Noro Psikiyatri Arsivi, 53(3), 229–233.CrossRef
31.
Ohashi, Y., Tashiro, K., Itoyama, Y., Nakano, I., Sobue, G., Nakamura, S., Sumino, S., & Yanagisawa, N. (2001). Study of functional rating scale for amyotrophic lateral sclerosis: revised ALSFRS(ALSFRS-R) Japanese version. Brain Nerve, 53(4), 346–55.
32.
Manera, U., Cabras, S., Daviddi, M., Vasta, R., Torrieri, M. C., Palumbo, F., Bombaci, A., Grassano, M., Solero, L., Peotta, L., Iazzolino, B., Canosa, A., Calvo, A., Chiò, A., & Moglia, C. (2020). Validation of the Italian version of self-administered ALSFRS-R scale. Amyotroph Lateral Scler Front Degener, 22(1–2), 151–153.
33.
Guedes, K., Pereira, C., Pavan, K., & Valério, B. C. O. (2010). Cross-cultural adaptation and validation of als functional rating scale-revised in Portuguese language. Arquivos de Neuro-Psiquiatria, 68(1), 44–47.CrossRef
34.
Rashed, H. R., Tork, M. A., Soliman, R., Serag, R., & Fahmy, N. (2020). Arabic adaptation and validation of the revised amyotrophic lateral sclerosis functional rating scale (ALSFRS-R): Egyptian study. Amyotroph Lateral Scler Front Degener, 22(3–4), 220–222.
35.
Liu, X. X., Fan, D. S., Zhang, J., Zhang, S., & Zheng, J. Y. (2009). Revised amyotrophic lateral sclerosis functional rating scale at time of diagnosis predicts survival time in amyotrophic lateral sclerosis. Zhonghua Yi Xue Za Zhi, 89(35), 2472–2475.
36.
Maessen, M., Post, M. W., Maillé, R., Lindeman, E., Mooij, R., Veldink, J. H., & Van Den Berg, L. H. (2009). Validity of the Dutch version of the Amyotrophic Lateral Sclerosis Assessment Questionnaire, ALSAQ-40, ALSAQ-5. Amyotroph Lateral Scler, 8(2), 96–100.CrossRef
37.
Benaim, C., Desnuelle, C., & Fournier-Mehouas, M. (2006). Functional scales and motor assessment in amyotrophic lateral sclerosis. Rev Neurol (Paris), 162, 4S131–4S7.
38.
List, D. (2016). The consensus group technique in social research. Field Methods, 13(3), 277–290.CrossRef
39.
Maier A, Gaudlitz M, Grehl T, Weyen U, Steinbach R, Großkreutz J, Rödiger A, Koch JC, Lengenfeld T, Weydt P, Günther R, Wolf J, Baum P, Metelmann M, Dorst J, Ludolph AC, Kettemann D, Norden J, Koc RY, Walter B, Hildebrandt B, Münch C, Meyer T, Spittel S. 2022.
40.
van Eijk, R. P. A., Beelen, A., Kruitwagen, E. T., Murray, D., Radakovic, R., Hobson, E., Knox, L., Helleman, J., Burke, T., Rubio Pérez, M. Á., Reviers, E., Genge, A., Steyn, F. J., Ngo, S., Eaglesham, J., Roes, K. C. B., van den Berg, L. H., Hardiman, O., & McDermott, C. J. (2021). A road map for remote digital health technology for motor neuron disease. J Med Internet Res, 23(9), 666.
41.
Connaghan, K. P., Green, J. R., Paganoni, S., Chan, J., Weber, H., Collins, E., Richburg, B., Eshghi, M., Onnela, J. P., & Berry, J. D. (2019). Use of Beiwe smartphone app to identify and track speech decline in amyotrophic lateral sclerosis (ALS). Interspeech, 2019, 4504–8.
42.
Baxi, E. G., Thompson, T., Li, J., Kaye, J. A., Lim, R. G., Wu, J., Ramamoorthy, D., Lima, L., Vaibhav, V., Matlock, A., Frank, A., Coyne, A. N., Landin, B., Ornelas, L., Mosmiller, E., Thrower, S., Farr, S. M., Panther, L., Gomez, E., … Rothstein, J. D. (2022). Answer ALS, a large-scale resource for sporadic and familial ALS combining clinical and multi-omics data from induced pluripotent cell lines. Nat Neurosci, 25(2), 226–237.CrossRef
43.
Rutkove, S. B. (2015). Clinical measures of disease progression in amyotrophic lateral sclerosis. Neurotherapeutics, 12(2), 384–393.CrossRef
44.
Meyer, T., Spittel, S., Grehl, T., Weyen, U., Steinbach, R., Kettemann, D., Petri, S., Weydt, P., Günther, R., Baum, P., Schlapakow, E., Koch, J. C., Boentert, M., Wolf, J., Grosskreutz, J., Rödiger, A., Ilse, B., Metelmann, M., Norden, J., … Maier, A. (2022). Remote digital assessment of amyotrophic lateral sclerosis functional rating scale-a multicenter observational study. Amyotroph Lateral Scler Front Degener., 6, 1–10.
45.
Rutkove, S. B., Narayanaswami, P., Berisha, V., Liss, J., Hahn, S., Shelton, K., Qi, K., Pandeya, S., & Shefner, J. M. (2020). Improved ALS clinical trials through frequent at-home self-assessment: a proof of concept study. Ann Clini Transl Neurol, 7(7), 1148–1157.CrossRef
46.
Eijk, R. P. A. (2020). Frequent self-assessments in ALS Clinical Trials: worthwhile or an unnecessary burden for patients? Ann Clini Transl Neurol, 7(10), 2074–2075.CrossRef
47.
Gromicho, M., Leão, T., Oliveira Santos, M., Pinto, S., Carvalho, A. M., Madeira, S. C., & De Carvalho, M. (2022). Dynamic Bayesian networks for stratification of disease progression in amyotrophic lateral sclerosis. Eur J Neurol, 29(8), 2201–2210.CrossRef
48.
Metadata
Title
ALSFRS-R-SE: an adapted, annotated, and self-explanatory version of the revised amyotrophic lateral sclerosis functional rating scale
Authors
André Maier
Matthias Boentert
Peter Reilich
Simon Witzel
Susanne Petri
Julian Großkreutz
Moritz Metelmann
Paul Lingor
Isabell Cordts
Johannes Dorst
Daniel Zeller
René Günther
Tim Hagenacker
Torsten Grehl
Susanne Spittel
Joachim Schuster
Albert Ludolph
Thomas Meyer
for the MND-NET consensus group
Publication date
01-12-2022
Publisher
BioMed Central
Published in
Neurological Research and Practice / Issue 1/2022
Electronic ISSN: 2524-3489
DOI
https://doi.org/10.1186/s42466-022-00224-6

Other articles of this Issue 1/2022

Neurological Research and Practice 1/2022 Go to the issue

Research article

Recovery of intracranial stenoses in varicella zoster virus vasculitis after long-term treatment with valacyclovir and prednisolone

Research article

COVID-19 outcomes in hospitalized Parkinson’s disease patients in two pandemic waves in 2020: a nationwide cross-sectional study from Germany

Research article

Real-world evidence on siponimod treatment in patients with secondary progressive multiple sclerosis

Research article

Intra-arterial application of nimodipine in reversible cerebral vasoconstriction syndrome: a neuroradiological method to help differentiate from primary central nervous system vasculitis

Research article

The impact of apraxia and neglect on early rehabilitation outcome after stroke

Letter to the editor

Intravenous thrombolysis for acute ischemic stroke associated with known left ventricular thrombus: safe or not?