Marcus Grobe-Einsler1,2, Arian Taheri Amin1,2, Jennifer Faber1,2, Tamara Schaprian1, Heike Jacobi1,3, Tanja Schmitz-Hübsch4, Alhassane Diallo5,6, Sophie Tezenas du Montcel7, Thomas Klockgether1,2. 1. German Center for Neurodegenerative Diseases (DZNE), Bonn, Germany. 2. Department of Neurology, University Hospital Bonn, Bonn, Germany. 3. Department of Neurology, University Hospital of Heidelberg, Heidelberg, Germany. 4. Experimental and Clinical Research Center, A Cooperation of Charité - Universitätsmedizin Berlin and Max-Delbrueck Center for Molecular Medicine, Berlin, Germany. 5. INSERM U1137-IAME, Department of Biostatistical Modeling, Clinical Investigation, Pharmacometrics in Infectious Diseases, University Paris Diderot, Paris, France. 6. Department of Epidemiology, Biostatistics and Clinical Research, APHP Bichât-Claude-Bernard Hospital, Paris, France. 7. Sorbonne Université, Institut, Pierre Louis d'Epidémiologie et de Santé Publique, Assistance Publique-Hôpitaux de Paris, Institut National de la Santé et de la Recherche Médicale, University Hospital Pitié-Salpêtrière, Paris, France.
Abstract
BACKGROUND: Clinical scales such as the Scale for the Assessment and Rating of Ataxia (SARA) cannot be used to study ataxia at home or to assess daily fluctuations. The objective of the current study was to develop a video-based instrument, SARAhome , for measuring ataxia severity easily and independently at home. METHODS: Based on feasibility of self-application, we selected 5 SARA items (gait, stance, speech, nose-finger test, fast alternating hand movements) for SARAhome (range, 0-28). We compared SARAhome items with total SARA scores in 526 patients with spinocerebellar ataxia types 1, 2, 3, and 6 from the EUROSCA natural history study. To prospectively validate the SARAhome , we directly compared the self-applied SARAhome and the conventional SARA in 50 ataxia patients. To demonstrate feasibility of independent home recordings in a pilot study, 12 ataxia patients were instructed to obtain a video each morning and evening over a period of 14 days. All videos were rated offline by a trained rater. RESULTS: SARAhome extracted from the EUROSCA baseline data was highly correlated with conventional SARA (r = 0.9854, P < 0.0001). In the prospective validation study, the SARAhome was highly correlated with the conventional SARA (r = 0.9254, P < 0.0001). Five of 12 participants of the pilot study obtained a complete set of 28 evaluable videos. Seven participants obtained 13-27 videos. The intraindividual differences between the lowest and highest SARAhome scores ranged from 1 to 5.5. CONCLUSION: The SARAhome and the conventional SARA are highly correlated. Application at home is feasible. There was a considerable degree of intraindividual variability of the SARAhome scores.
BACKGROUND: Clinical scales such as the Scale for the Assessment and Rating of Ataxia (SARA) cannot be used to study ataxia at home or to assess daily fluctuations. The objective of the current study was to develop a video-based instrument, SARAhome , for measuring ataxia severity easily and independently at home. METHODS: Based on feasibility of self-application, we selected 5 SARA items (gait, stance, speech, nose-finger test, fast alternating hand movements) for SARAhome (range, 0-28). We compared SARAhome items with total SARA scores in 526 patients with spinocerebellar ataxia types 1, 2, 3, and 6 from the EUROSCA natural history study. To prospectively validate the SARAhome , we directly compared the self-applied SARAhome and the conventional SARA in 50 ataxiapatients. To demonstrate feasibility of independent home recordings in a pilot study, 12 ataxiapatients were instructed to obtain a video each morning and evening over a period of 14 days. All videos were rated offline by a trained rater. RESULTS: SARAhome extracted from the EUROSCA baseline data was highly correlated with conventional SARA (r = 0.9854, P < 0.0001). In the prospective validation study, the SARAhome was highly correlated with the conventional SARA (r = 0.9254, P < 0.0001). Five of 12 participants of the pilot study obtained a complete set of 28 evaluable videos. Seven participants obtained 13-27 videos. The intraindividual differences between the lowest and highest SARAhome scores ranged from 1 to 5.5. CONCLUSION: The SARAhome and the conventional SARA are highly correlated. Application at home is feasible. There was a considerable degree of intraindividual variability of the SARAhome scores.
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