In 2021, Ataxia UK co-funded a research project with the National Ataxia Foundation, ‘Assessment of ataxia severity under real-life conditions with SARAhome: A multicenter study in spinocerebellar ataxia type 3 (SCA3)’. This project was led by Dr Marcus Grobe-Einsler and Dr Thomas Klockgether at the German Center for Neurodegenerative Diseases. Following this project, the researchers have published a new paper about SARAhome. SARAhome is an at-home version of the SARA scale, which uses a video-based mobile app to track ataxia symptoms.
The Scale for Assessment and Rating of Ataxia (SARA) is a well-established tool used by clinicians to measure the level of ataxia symptoms at a single point in time. It gives a score from 0 – 40, with higher scores indicating higher severity. Due to it only measuring symptoms at one time point, it does not capture short-term changes in symptoms, limiting its sensitivity to change when used in clinical trials. To accurately capture fluctuations in symptoms of ataxia, repeated measurements at short intervals, ideally at home, are required. Remote assessment of ataxia symptoms also enables inclusion of more severely affected individuals who may otherwise have limited access to clinical trial sites.
To address these issues, Dr Grobe-Einsler and his team tested SARAhome in 65 participants with SCA3 from the European SCA3 Initiative (ESMI). Participants were recruited from the UK, Germany, the Netherlands and the Azores (Portugal). Participants were asked to use the app twice daily for 14 days to capture short-term fluctuations in symptoms in the real-world setting. The researchers also followed 11 participants over a longer period to assess the sensitivity of SARAhome to change over the longer term. The researchers also collected information on the factors influencing the fluctuations in SCA3 symptoms.
The Researchers first took a baseline SARA score. The SARAhome assessments then involved participants completing five out of the eight SARA tasks to assess gait, stance, rapid hand movements, speech, and the nose-finger test. Participants were then asked to record themselves completing these 5 tasks twice daily for 14 days using an iPad and a tripod. They were asked beforehand to provide a subjective assessment of their ataxia severity. These subjective ratings were collated as a reported patient global impression (PGI) of severity over seven categories (very much worse, much worse, minimally worse, no change, minimally improved or very much improved). This way, the participants’ perception of their ataxia could be compared to the SARAhome measurements. If participants said their symptoms had worsened, or improved, they were asked to share why they thought that might be.
1459 SARAhome recordings were taken in total, from 65 people with SCA3. The researchers found that SARAhome is a feasible tool to remotely measure ataxia severity, with a mean response rate of over 80% throughout the testing period. The highest adherence was seen for the first 4 days. The researchers also agreed that 4 days is a sufficient time period in which to adequately capture fluctuations. The measured fluctuations were remarkably high, with a median of three out of 28 possible points on the SARAhome scale.
Ataxia severity was reported as subjectively unchanged in 988 recordings, improved in 103 (minimally improved in 96, much improved in 5 and very much improved in 2), and worsened in 322 (minimally worse in 248, much worse in 71 and very much worse in 3). In those that reported improvements in severity, the SARAhome score decreased by 0.5, whilst it increased by 0.25 or 0.5 in those who rated severity as much worse or very much worse. Reported reasons for subjective improvement included restedness and good mood, whereas those for worsening included tiredness, exhaustion, and physical therapy/training. The researchers found that SARAhome aligns closely with the conventional SARA scale. The researchers found that the average score of a 4-day recording using SARAhome was more able to detect changes in ataxia severity than the conventional SARA score obtained in a single study visit.
In the 11 participants who were followed up (after about one year), conventional SARA scores did not significantly increase from baseline to follow-up, whilst the SARAhome scores did.
Overall, the researchers found that SARAhome is a feasible tool to measure ataxia symptoms, and effectively captures fluctuations, making it a suitable tool for clinical trials in SCA3.
Read the paper here.
