Digital Health Technologies for Monitoring Motor Function in Progressive Supranuclear Palsy and Dementia with Lewy Bodies

About This Grant

PROJECT SUMMARY Progressive supranuclear palsy (PSP) is a severe and rapidly progressive Frontotemporal Lobar Degeneration (FTLD) syndrome that lacks effective treatment and leads to a rapid onset of dementia, disability, and death. Dementia with Lewy Bodies (DLB) is the second most common type of neurodegenerative dementia after Alzheimer’s disease. Both PSP and DLB cause significant motor impairment, resulting in progressive loss of independence in activities of daily living, such as eating, dressing, and writing. There are no FDA-approved treatments for FTLD syndromes (including PSP) or DLB. One of the key challenges in the development of new effective therapies for DLB syndromes is the lack of validated instruments and biomarkers to measure disease severity, disease progression, and the effect of therapeutic interventions. Since 2019, BioSensics, Massachusetts General Hospital (MGH) and Johns Hopkins University (JHU) have partnered to develop a wearable-based remote monitoring solution for FTLD syndromes. The solution uses PAMSysTM, a patented and validated pendant sensor for monitoring physical activity, posture, and walking parameters during activities of daily living. Through a Direct Phase II SBIR award (R44AG080861) from the National Institute on Aging, we have demonstrated the feasibility of using PAMSys for tracking ambulatory change in PSP. In this project, we will extend our work by using both PAMSys and PAMSys ULMTM wrist sensors to enable comprehensive monitoring of upper and lower limb function. PAMSys ULM is a patented wrist sensor solution for monitoring goal-directed movements of the upper limb during activities of daily living. The primary goal of the project is to qualify digital monitoring biomarkers of motor function for both PSP and DLB with the FDA Biomarker Qualification Program (BQP). Context of Use: COU1 and COU2) Monitoring by working biomarkers to track ambulatory change over time in PSP and DLB, COU3 and COU4) Monitoring biomarkers to track changes in upper limb function over time in PSP and DLB. We will work with CurePSP, The Association for Frontotemporal Dementia, and the Lewy Body Dementia Association to recruit study participants and leverage an NIH-funded platform trial in PSP to facilitate patient recruitment and assure enrollment diversity. We will demonstrate the accuracy (analytical validation) of both PAMSys and PAMSys ULM in PSP and DLB. In addition, we will conduct a proof-of-concept study to demonstrate the feasibility and reliability of monitoring motor function in PSP and DLB using PAMSys and PAMSys ULM. Afterward, we will conduct a multisite longitudinal clinical validation of digital health technologies for monitoring motor function in a sizeable and diverse cohort of PSP and DLB patients and compare this to functional scales and patient-reported outcome measures. Moreover, as recommended by the FDA BQP, we will conduct interviews with all participants using the Technology Acceptance Model to examine the perception of benefit, technology acceptance and anxiety, and attitude toward use from patients’ point of view.