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

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.

Population-based Evidence for Using Serum Neurofilaments as Individual Diagnostic and Prognostic Biomarkers in Amyotrophic Lateral Sclerosis

“…Compared to this biomarker-based approach, the validated ENCALS prediction model enables a reliable prognosis of survival based on eight clinical parameters 40 . However, a limitation of the ENCALS model lies in the use of ALSFRS-R-based disease progression and diagnostic delay as variables, bearing a risk of bias [41][42][43][44] , as well as forced vital capacity, which typically decreases particularly in later disease stages. Thus, the ALS Z-Score-based approach might be less prone to bias and could possibly provide improved prognostic information, especially in earlier disease stages.…”

Neurofilament light‐chain response during therapy with antisense oligonucleotide tofersen in SOD1‐related ALS: Treatment experience in clinical practice

“…The ALSFRS-R is a 12-item disease-specific instrument that measures bulbar, gross, and fine motor functions and respiratory symptoms. 15,16…”

Der Einsatz von Patient-reported Outcome Measures (PROM) und die Perspektive digitaler Biomarker bei der Amyotrophen Lateralsklerose

“…Eine Anpassungen von Formulierungen innerhalb der ALSFRS-R ermöglichte den Einsatz als PROM [9], der wiederholt eine gute bis sehr gute Übereinstimmungen in der Erhebung zwischen ALS-Betroffenen und medizinischen Fachkräften zeigte [9,10]. Um den Unterschied zwischen einer Interview-basierten Erhebung der ALS-FRS-R und der Selbstbewertung zu reduzieren und damit die Interoperabilität zu erhöhen, wurde in Deutschland eine selbsterklärende Version (ALSFRS-R-SE) entwickelt und im deutschen ALS-Netzwerk (MND-NET) konsentiert [11].…”

“…Diese Effekte können verstärkt werden, wenn die PROM digital erhoben werden [28]. Gleichzeitig erhöhen Maßnahmen wie die Adaption der verwendeten Sprache [11] oder Schaffung eines Mehrwertes für die Patienten selbst (z. B. des besseren Verständnisses der eigenen Erkrankung) die Akzeptanz [28].…”