A Wearable Sensor Platform for Remote Monitoring of Motor Impairment in Huntingtons Disease and Prodromal Huntingtons Disease - ABSTRACT The principal means of measuring motor impairment in Huntington disease (HD) is the Unified Huntington’s Disease Rating Scale total motor score (UHDRS-TMS). Although valuable, the scale is subjective and categorical, and requires significant training to administer correctly. To address some of the challenges and shortcomings associated with UHDRS-TMS, we have developed a wearable sensor, called HDWear, for continuous monitoring of chorea and physical activity in individuals with HD. As a part of this development, we have enrolled 41 participants (21 with clinically diagnosed HD, 8 classified as prodromal HD, and 12 controls age-matched to HD participants) at the University of Rochester Medical Center who agreed to periodic monitoring over 12 months using HDWear. This study represents one of the longest monitoring of individuals with HD and prodromal HD using wearable sensors ever conducted and validated the utility of HDWear for monitoring physical activity and chorea in HD patients. In this study, HD participants exhibited no significant change in UHDRS-TMS, while there was a significant decrease in physical activity level from baseline to 12- month. However, physical activity level in prodromal HD participants did not change over the 12-month monitoring period, highlighting the need to develop more sensitive metrics to capture progression of motor impairment in these individuals during early onset of the disease. This is especially important since most therapeutics under development are targeting prodromal and early-stages of HD to slow down the progression of the disease. In this regard, our preliminary results, as well as other recent published papers, suggest that speech and cognitive function changes, along with motor assessment, can lead to better characterization of the onset of symptoms in prodromal HD. In this Direct Phase II SBIR, we propose to build upon HDWear by developing a comprehensive remote monitoring solution for individuals with HD and prodromal HD called HDWear+. In addition to a wearable sensor, HDWear+ includes digital speech and cognitive assessments and enables the collection of patient-reported outcomes (PROs) related to quality of life and functional ability, as well as a medication diary. We will use HDWear+ to monitor and follow a large cohort of HD and prodromal HD (including 25 HD, 25 prodromal HD and 25 controls) for 12 months. We will evaluate the sensitivity of HDWear+ for monitoring changes in motor, speech, and cognitive function in individuals with prodromal HD and HD. Moreover, we will develop a machine learning- based model for automatic assessment of dysarthria in HD. In accordance with regulatory processes for the adoption of novel technologies, we will conduct interviews with participants at baseline, 6, and 12 months using the Technology Acceptance Model (TAM) to examine perception of benefit, technology acceptance, technological anxiety, trust, facilitating conditions, perceived risk, and attitude of use from the point of view of patients. We will also use Usability Metric for User Experience to measure the usability of HDWear+.
