Enhancing the Objective Assessment of Friedreich Ataxia Severity: A Multiview IMU-Based Approach.

Riedreich ataxia (FRDA) is a progressive neurodegenerative disease resulting in ataxia, affecting both limb movement and balance control. Most measurements disease severity measure these separately, but there is a need in clinical trials and clinical research for measures that account for all these domains in a single score. This study introduces a novel multiview framework leveraging inertial measurement unit (IMU) data from three instrumented devices, the Ataxia Instrumented Measures (AIMs) - cup (AIM-C), spoon (AIM-S), and pendent (AIM-P)-to enhance ataxia severity assessment of FRDA. Regression models based on individual devices demonstrated Pearson correlations of 0.83, 0.749, and 0.77 with the modified Friedreich Ataxia Rating Scale (mFARS), respectively. A multiview model integrating data across all three devices yielded a significantly improved correlation of 0.94, generating the Aggregated IMU Multiview Score (AIM-M). AIM-M offers a robust, scalable, and objective metric for quantifying FRDA severity, outperforming existing single-view approaches. These findings indicate that multiview IMU-based systems can provide sensitive and reliable assessments of severity of ataxia in FRDA.Clinical relevance-This study presents a multiview IMU-based approach that can enhances the objective assessment of severity of ataxia in FRDA.