Determining the Validity of Conducting Rating Scales in Friedreich Ataxia through Video

Abstract

BackgroundBackground: The Friedreich Ataxia Rating Scale (FARS) and the Scale for the Assessment and Rating of Ataxia (SARA) are commonly used neurological rating scales in Friedreich ataxia (FRDA). The modified Friedreich Ataxia Rating Scale (mFARS) has been accepted as an appropriate outcome measure for clinical trials in FRDA. Objectives Objectives: The COVID-19 pandemic has resulted in limited face-to-face interactions with individuals involved in natural history studies and clinical trials. The aim of this study was to determine the validity of conducting the mFARS and SARA through video. Methods Methods: Individuals who had the mFARS administered face-to-face in the previous 6 months were invited to participate. Participants were sent instructions and asked to have a carer present to assist. The mFARS and SARA were then administered by video. Differences between face-to-face and video scores and the reliability between scores obtained face-to-face and by video were examined. ResultsResults: The mFARS and SARA were conducted by video with 19 individuals. Excellent test-retest reliability was seen in the mFARS lower limb coordination (ICC = 0.96, 95% CI 0.90-0.98) and upright stability sections (ICC = 0.97, 95% CI 0.93-0.99), total mFARS (ICC = 0.97, 95% CI 0.92-0.99) and SARA scores (ICC = 0.98, 95% CI 0.95-0.99). Conclusions Conclusions: Excellent test-retest reliability was demonstrated in the majority of the mFARS sections, and in the total mFARS and SARA scores, suggesting that video is a valid method of conducting these scales. This method enables inclusion of participants who are unable to travel to study sites. A larger cohort will be required to further validate the use of video mFARS and SARA for future studies.

Prediction of the disease course in Friedreich ataxia

“…While the trial investigated a remote examination and non-FRDA ataxias, it nonetheless supports the idea that a score of a clinical examination is not as static as it might appear. In contrast, research seeking to quantify retest-reliability of the modified Friedreich Ataxia Rating Scale as well as the SARA in patients of FRDA did find rather high retest-reliability 37,38 . Despite standardised operating procedures effects due to multiple and changing examiners as well as shifts in subjective criteria where examinations depend on a clinician's rating are concerns over the long-term and ways to address these issues should be sought.…”

Comparison of Live and Remote Video Ratings of the Scale for Assessment and Rating of Ataxia

“…Demonstration of feasibility of SARA home recordings and comparability of self‐performed SARA scores at home to examinations by a physician in the clinic have already been conducted in smaller cohorts. 6 , 7 Moving an examination, or at least parts of it, to patient's home is not only well accepted, but it is also closer to patients’ daily life surroundings, easier to integrate in their daily life, and allows higher frequent sampling, which might in turn, allow a higher signal to noise ratio. 6 , 7 , 10 In addition, from the patient's perspective, home recordings reduce the burden of travel and risk of infection.…”

“… 6 , 7 Moving an examination, or at least parts of it, to patient's home is not only well accepted, but it is also closer to patients’ daily life surroundings, easier to integrate in their daily life, and allows higher frequent sampling, which might in turn, allow a higher signal to noise ratio. 6 , 7 , 10 In addition, from the patient's perspective, home recordings reduce the burden of travel and risk of infection. From the examiner's perspective, home recordings are resource efficient, and therefore, allow for a higher frequency of examinations, for example in the context of treatment surveillance and/or symptom fluctuation.…”

“…Recently, video recordings of neurologic examinations and clinical scales have become a frequent means for patient care, rater‐training, and centralized ratings of different movement disorders scales 2–5 . In analogy, remote video assessment of SARA examinations are increasingly used to counteract inter‐rater variability (centralized ratings), for training purposes and to assess ataxia severity at patient's home 6,7 . Contact restrictions during the corona pandemic, but also the mobility and travel impairments inherent in patients with movement disorders, stress the need for remote assessments.…”

“… 2 , 3 , 4 , 5 In analogy, remote video assessment of SARA examinations are increasingly used to counteract inter‐rater variability (centralized ratings), for training purposes and to assess ataxia severity at patient's home. 6 , 7 Contact restrictions during the corona pandemic, but also the mobility and travel impairments inherent in patients with movement disorders, stress the need for remote assessments. Despite the advantages of video ratings, differences between live ratings and remote video‐ratings have not been systematically investigated for SARA.…”