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Validation of the German version of the extended ALS functional rating scale as a patient-reported outcome measure

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Abstract

The revised Amyotrophic Lateral Sclerosis Functional Rating Scale (ALSFRS-R) is a well-established rating instrument to assess the functional status of ALS patients. A recent innovation was the addition of three further items designed to improve its sensitivity at lower levels of physical function (ALSFRS-Extension, ALSFRS-EX). Neither the ALSFRS-R nor the ALSFRS-EX has been validated in German yet. The aim of the present study was the validation of the German version of a self-administered form of the ALSFRS-EX. Seventy-six patients participated in the study. Psychometric analysis included reliability assessment and factorial analysis. To evaluate convergent validity, correlations between ALSFRS-EX items and the MRC score, spasticity, tongue movement, pulmonary function, ALSAQ-40 and Borg dyspnoea scales (upright and supine) were performed. Internal consistency as measured by Cronbach’s alpha (total scale 0.868, subscales 0.690–0.938) and corrected item to total correlations (all above 0.50) was high. Test–retest reliability assessed by Spearman’s rho (0.882–0.972) and Cohen’s Kappa (0.63–0.92) was also high. Principal component analysis with varimax rotation yielded a four-factor solution accounting for approximately 79 % of the variance. Clinical parameters were strongly correlated with respective items and subscores of the ALSFRS-EX (muscle strength 0.568–0.833 p < 0.01; spasticity −0.236 to −0.376 p < 0.05; tongue movement 0.437–0.818 p < 0.01; pulmonary function 0.485–0.577 p < 0.01). ALSAQ-40 and Borg score correlated highly with the corresponding ALSFRS-EX items. The German self-report version of the ALSFRS-EX possesses very good psychometric properties similar to the original scale including high internal consistency and test–retest reliability as well as excellent convergent validity.

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Acknowledgments

The authors wish to express their gratitude to the patients who participated in this study. This work was supported by the Stiftung für Medizinische Wissenschaft, Frankfurt am Main (to SV) and by the excellence program of the state of Saxony-Anhalt (to HJH).

Conflicts of interest

On behalf of all authors, the corresponding author states that there is no conflict of interest.

Ethical standards

The study was approved by the institutional ethics committees of Hannover Medical School and the University of Magdeburg and has, therefore, been performed in accordance with the ethical standards laid down in the 1964 Declaration of Helsinki and its later amendments.

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Authors and Affiliations

  1. Department of Neurology, Otto-von-Guericke University Magdeburg, Leipziger Str. 44, 39120, Magdeburg, Germany

    Susanne Abdulla, Stefan Vielhaber & Hans-Jochen Heinze

  2. Department of Neurology, Hannover Medical School, Carl-Neuberg-Str. 1, 30625, Hannover, Germany

    Susanne Abdulla, Sonja Körner, Reinhard Dengler & Susanne Petri

  3. German Center for Neurodegenerative Diseases, Leipziger Str. 44, 39120, Magdeburg, Germany

    Susanne Abdulla, Stefan Vielhaber, Judith Machts & Hans-Jochen Heinze

  4. Leibniz Institute for Neurobiology, Brenneckestraße 6, 39118, Magdeburg, Germany

    Hans-Jochen Heinze

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  1. Susanne Abdulla
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Corresponding author

Correspondence to Susanne Abdulla.

Appendix

Appendix

Der ALSFRS-EX ist eine Bewertungsskala und wird verwendet, um die aktuellen Beeinträchtigungen von alltäglichen Fähigkeiten und körperlichen Funktionen bei Patienten mit ALS festzustellen.

Es werden 15 Bereiche betrachtet. Zu jedem Bereich gibt es 5 Auswahlmöglichkeiten (von 0 bis 4) um einzuschätzen, ob eine Beeinträchtigung vorliegt und wie stark diese ausgeprägt ist. Die rechte Spalte erläutert die jeweilige Aussage genauer und mit Beispielen und soll Ihnen helfen die zutreffende Aussage auszuwählen.

Lesen Sie bitte jeden Bereich und die dazugehörigen 5 Aussagen genau durch. Bitte wählen Sie die Aussage aus, welche Ihre aktuelle Situation zutreffend beschreibt und markieren Sie diese Aussage mit einem Kreuz in dem daneben stehenden Kreis.

Sollten Sie Schwierigkeiten beim Ausfüllen der Bewertungsskala haben, lassen Sie sich bitte dabei helfen.

Die Beantwortung ist freiwillig. Ihre Angaben werden streng vertraulich behandelt.

Vielen Dank fürs Mitmachen!

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Abdulla, S., Vielhaber, S., Körner, S. et al. Validation of the German version of the extended ALS functional rating scale as a patient-reported outcome measure. J Neurol 260, 2242–2255 (2013). https://doi.org/10.1007/s00415-013-6955-6

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  • DOI: https://doi.org/10.1007/s00415-013-6955-6

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