Written by Ziyang Zhao Edited by Dr. Hayley McLoughlin
A newly developed smartphone application will allow patients to assess ataxia at home.
There’s an interesting problem in science that’s often overshadowed in the scientific community. It’s not as flashy or as newsworthy as most scientific headlines, like the eradication of Polio or the creation of the coronavirus vaccine, but its importance looms nonetheless. That problem is the monumental task of getting people to assess themselves.
Take this interesting bit: The American Cancer Society found that nearly 100% of Americans are aware of the benefits of monthly screenings for Colorectal Cancer — a preventable and treatable form of cancer, if detected early — yet nearly 50,000 Colorectal Cancer-related deaths occur each year in the United States (American Cancer Society, 2016). Alongside that first statistic, the American Cancer Society had also asked why an unscreened individual chooses to remain so. An important reason, they noted, was patient concern over the complexities of taking a test: taking time off from work, getting a ride home, and high out-of-pocket expenses.
In Ataxia-based diseases, testing is similarly cumbersome and accessibility for assessment is not readily available. The most common way to measure the degree of one’s level of Ataxia is through the Scale for assessment and rating of ataxia (SARA) score, which evaluates 9 ataxia-affected abilities to produce a composite score. The problem, however, is that the SARA test is cumbersome. It’s a costly assessment that requires the patient to travel to their local hospital and meet with a testing expert.
In this study, the researchers devised an Ataxia assessment matching the SARA test that can be performed at home, which they call SARAhome. While the original SARA test assessed 8 attributes, this new Ataxia test only assessed 5, including gait, stance, speech, nose-finger test, fast alternating hand movements. To make SARAhome even easier to take at home, the researchers also incorporated some modifications to their selected 5 tests from the original SARA test, including reducing required walking distances, performing fast-alternating movement and nose-finger tests on a chair, and replacing an investigator’s finger in the nose finger test with a tape-mark on the wall. These video recordings would be sent to an experienced rater, who would subsequently produce the score.
With these changes, the researchers set out to evaluate their new test in a prospective validation study of 50 subjects. The process of prospective validation, in essence, is to verify that a system of protocols works as intended before a product becomes implemented. In this study, it’s to make sure that SARAhome correlates with SARA. They brought their subjects to the lab and gave each oral instruction, allowing them to independently perform the tests on a tablet. The results were promising, correlating nearly perfectly with patient SARA scores.
A follow-up pilot study to assess the feasibility of the test at home was also conducted, following 12 ataxia patients. The researchers gave each subject verbal instructions alongside an infographic containing detailed instructions (see below). They then tracked the patients for 14 days, having them test twice daily using the app, finding similar positive results. With these promising datasets in mind, the scientists implemented their video-based test into a mobile app that can be downloaded on tablets and phones alike.
While more testing of Ataxia may have limited benefits to patients directly, the benefits to scientists and researchers are enormous. With access to repeat measurements, the data becomes more granular and scientists are better able to track Ataxia symptoms over time. For example, most clinical trials today in Spinocerebellar Ataxia Type 3 use SARA as their primary measure of effectiveness, with measurements in monthly intervals.
If, say, the scientists were able to measure SARAhome on a weekly basis to complement SARA measurements, they would have far more data to power their studies and assess patient responses to novel drugs and therapies. Studies have also shown that SARA scores vary significantly by psychological, physiological, cognitive, environmental, and technical factors and by whether a patient conducts the assessment supervised or unsupervised (Warmerdam et al., 2020). The authors have noted that SARAhome could potentially be an appropriate tool to study these variations.
While these new developments are exciting, there are a few caveats. Firstly, the test must still be assessed by a trained practitioner. This adds an element of subjectivity and uncertainty that plagues the SARA test as well. Secondly, patients that have progressed past a certain point may not be able to operate the app, and thus may require an assistant to use this new test.
In summary, researchers have developed a proprietary Ataxia test and have found that it closely correlates with SARA scores. The SARAhome test is comprised of a subset of the SARA assessments, with changes tailored to make tests more feasible to be conducted at home. Through the mobile app, patients are able to conduct SARAhome at home. This new mode of testing will allow for more data and better granularity, an exciting prospect to researchers working on clinical trials for Ataxia-related diseases.
Ataxia: A loss of muscular control, leading to abnormal walking, speech changes and irregular eye movements.
Spinocerebellar Ataxia: A form of ataxia where brain damage is localized to the cerebellum and spinal cord.
Colorectal Cancer: A cancer of the colon or rectum, located at the digestive tract’s lower end.
Cerebellum: A structure located near the rear of the brain. It helps with the coordination and movement related to motor skills, especially involving the hands and feet. Learn more in this Snapshot.
Spinal cord: A long set of nerves and cells that span from the brain to the lower back. Transports signals between the brain and the rest of the body.
Clinical Trials: Research studies aiming to evaluate medical, surgical, or behavioral intervention in human subjects. Learn more in this Snapshot.
Conflict of Interest Statement
The author and editor declare no conflict of interest.
Citation of Article Reviewed
Grobe‐Einsler, M., Taheri Amin, A., Faber, J., Schaprian, T., Jacobi, H., Schmitz‐Hübsch, T., Diallo, A., Tezenas du Montcel, S., & Klockgether, T. (2021). Development of sara home , a new video‐based tool for the assessment of ataxia at home. Movement Disorders, 36(5), 1242–1246. https://doi.org/10.1002/mds.28478
American Cancer Society. (2016). Top 5 reasons people don’t get screened for colorectal cancer. American Cancer Society MediaRoom. Retrieved November 10, 2021, from http://pressroom.cancer.org/Top5reasonscolorectalcancer.
Warmerdam, E., Hausdorff, J. M., Atrsaei, A., Zhou, Y., Mirelman, A., Aminian, K., Espay, A. J., Hansen, C., Evers, L. J., Keller, A., Lamoth, C., Pilotto, A., Rochester, L., Schmidt, G., Bloem, B. R., & Maetzler, W. (2020). Long-term unsupervised mobility assessment in movement disorders. The Lancet Neurology, 19(5), 462–470. https://doi.org/10.1016/s1474-4422(19)30397-7