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

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; MND-NET consensus group

doi:10.1186/s42466-022-00224-6

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

Neurol Res Pract. 2022 Dec 15;4(1):60. doi: 10.1186/s42466-022-00224-6.

Authors

André Maier¹, Matthias Boentert^{2

3}, Peter Reilich⁴, Simon Witzel⁵, Susanne Petri⁶, Julian Großkreutz⁷, Moritz Metelmann⁸, Paul Lingor⁹, Isabell Cordts⁹, Johannes Dorst⁵, Daniel Zeller¹⁰, René Günther^{11

12}, Tim Hagenacker¹³, Torsten Grehl¹⁴, Susanne Spittel¹⁵, Joachim Schuster^{5

16}, Albert Ludolph^{5

16}, Thomas Meyer^{17

15}; MND-NET consensus group

Affiliations

¹ Department of Neurology, Center for ALS and Other Motor Neuron Disorders, Charité - Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Berlin, Germany. andre.maier@charite.de.
² Department of Neurology, Universitätsklinikum Münster, Münster, Germany.
³ Department of Medicine, UKM-Marienhospital Steinfurt, Steinfurt, Germany.
⁴ Friedrich-Baur-Institut und Neurologische Klinik und Poliklinik, LMU Klinikum, Ludwig-Maximilians-Universität München, Munich, Germany.
⁵ Klinik für Neurologie, Universitätsklinikum Ulm, Ulm, Germany.
⁶ Hannover Medical School, Department of Neurology, Hannover, Germany.
⁷ Department of Neurology, Campus Lübeck, Universitätsmedizin Schleswig-Holstein, Lübeck, Germany.
⁸ Department of Neurology, Universitätsklinikum Leipzig, Leipzig, Germany.
⁹ Department of Neurology, Klinikum rechts der Isar, Technical University Munich, Munich, Germany.
¹⁰ Department of Neurology, Universitätsklinikum Würzburg, Würzburg, Germany.
¹¹ Department of Neurology, University Hospital Carl Gustav Carus, Technische Universität Dresden, Dresden, Germany.
¹² DZNE, German Center for Neurodegenerative Diseases, Research Site Dresden, Dresden, Germany.
¹³ Klinik für Neurologie und Center for Translational Neuro- and Behavioral Science, Universitätsmedizin Essen, Essen, Germany.
¹⁴ Department of Neurology, Centre for ALS and Other Motor Neuron Disorders, Alfried Krupp Krankenhaus, Essen, Germany.
¹⁵ Ambulanzpartner Soziotechnologie APST GmbH, Berlin, Germany.
¹⁶ DZNE, German Centre for Neurodegenerative Diseases, Research Site Ulm, Ulm, Germany.
¹⁷ Department of Neurology, Center for ALS and Other Motor Neuron Disorders, Charité - Universitätsmedizin Berlin, Corporate Member of Freie Universität Berlin, Humboldt-Universität zu Berlin, and Berlin Institute of Health, Berlin, Germany.

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.

Keywords: ALSFRS-R; Amyotrophic lateral sclerosis; Digital medicine; Patient reported outcomes.