Quantification of Motor Function in Huntington Disease Patients Using Wearable Sensor Devices.

Previous studies have demonstrated the feasibility and promise of wearable sensors as objective measures of motor impairment in Parkinson disease and essential tremor. However, there are few published studies that have examined such an application in Huntington disease (HD). This report provides an evaluation of the potential to objectively quantify chorea in HD patients using wearable sensor data. Data were derived from a substudy of the phase 2 Open-PRIDE-HD study, where 17 patients were screened and 15 patients enrolled in the substudy and ultimately 10 patients provided sufficient wearable sensor data. The substudy was designed to provide high-resolution data to inform design of predictive algorithms for chorea quantification. During the entire course of the 6-month study, in addition to chorea ratings from 18 in-clinic assessments, 890 home assessments, and 1,388 responses to daily reminders, 33,000 h of high-resolution accelerometer data were captured continuously from wearable smartwatches and smartphones. Despite its limited sample size, our study demonstrates that arm chorea can be characterized using accelerometer data during static assessments. Nonetheless, the small sample size limits the generalizability of the model. The sensor-based model can quantify the chorea level with high correlation to the chorea severity reported by both clinicians and patients. In addition, our analysis shows that the chorea digital signature varies between patients. This work suggests that digital wearable sensors have the potential to support clinical development of medications in patients with movement disorders, such as chorea. However, additional data would be needed from a larger number of HD patients with a full range of chorea severity (none to severe) with and without intervention to validate this potentially predictive technology.

Copyright © 2019 by S. Karger AG, Basel.

M.F. Gordon, I.D. Grachev, P.S. Loupe, S. Fine, L. Navon-Perry, N. Gross, S. Papapetropoulos, J.-M. Savola, and M.R. Hayden are or were Teva Pharmaceutical Industries employees during the course of this study. I. Mazeh and Y. Dolan are employees of Intel. R. Reilmann is founding director and owner of the George Huntington Institute, a private research institute focused on clinical and preclinical research in HD, and QuantiMedis, a clinical research organization providing Q-Motor services in clinical trials and research. He serves as an elected member of the steering committees of the European Huntington's Disease Network and the Huntington Study Group, co-chair of the Task Force on Huntington's Disease, and member of the Task Force on Technology of the International Parkinson and Movement Disorder Society. He has provided consulting services, advisory board functions, clinical trial services, Q-Motor analyses, or lectures for Actelion Pharmaceuticals, Amarin Neuroscience, AOP Orphan Pharmaceuticals, the Cure Huntington Disease Initiative Foundation, Desitin, Hoffmann-La Roche, Ionis Pharmaceuticals, Ipsen, Lundbeck, Link Medicine, Meda Pharma, Medivation, Mitoconix, Neurocrine Biosciences, Neurosearch, Novartis, Omeros, Pfizer, Prana Biotechnology, Prilenia Therapeutics, Raptor Pharmaceuticals, Siena Biotech, Temmler Pharma, Teva Pharmaceuticals, uniQure, Vaccinex, Wave Life Sciences, and Wyeth Pharmaceuticals. He has received grant support from the Bundesministerium für Bildung und Forschung, the Cure Huntington Disease Initiative Foundation, the Deutsche Forschungsgemeinschaft, the Deutsches Zentrum fur Neurodegeneration und Entzündung, the European Union Seventh Framework Program (EU-FP7), the European Huntington's Disease Network, the High Q Foundation, and the National Science Foundation.