The Digital-HD study: Smartphone-Based Remote Testing to Assess Cognitive and Motor Symptoms in Huntington's Disease

Objective: The aim of this study is to evaluate the ability of smartphone-based remote testing to meaningfully quantify signs, symptoms and impairments among participants with premanifest Huntington’s disease (HD) and manifest HD and compare them with healthy controls (HC). Background: A smartphone application comprising seven active tests (Symbol Digit Modalities Test [SDMT], Stroop Word Reading [SWR], Speeded tapping, Chorea, Balance, U-turn, 2-minute walk) and continuous passive monitoring is deployed in an observational study (Digital-HD) of participants with premanifest HD, manifest HD and HC. Design/Methods: Predefined active test measurements were aggregated over Weeks 5 and 6 post-screening to minimise practice effects. Group differences between HC, premanifest HD and manifest HD were analysed using the Kruskall–Wallis test and subsequent pairwise Mann–Whitney tests. In an exploratory analysis, a broader set of features was analysed for differences between HC and participants with premanifest HD. Recruitment for Digital-HD is ongoing. Preliminary data were analysed from 18 participants with premanifest HD, 15 with manifest HD and 19 HC. Results: Predefined digital tests generally had close correspondence with their commonly used in-clinic counterparts and these significantly differed between HC and the manifest group, as well as between the premanifest HD and the manifest HD groups (p=0.01–0.05 for U-Turn speed and 2-minute walk step frequency variability, p Conclusions: This preliminary analysis suggests that the digital platform can robustly measure established features of manifest HD, and affords the opportunity for innovative measures that may have great value in assessing premanifest and prodromal phases of HD. Disclosure: Dr. Tortelli has received personal compensation for consulting, serving on a scientific advisory board, speaking, or other activities with GLG. Dr. Tortelli has received research support from Yes, a research grant to University College London by F. Hoffmann La Roche..Dr. Simillion has received personal compensation for consulting, serving on a scientific advisory board, speaking, or other activities with F. Hoffmann-La Roche Ltd.. Dr. Lipsmeier has received personal compensation for consulting, serving on a scientific advisory board, speaking, or other activities with F. Hoffmann-La Roche Ltd.. Dr. Lipsmeier has received royalty, license fees, or contractual rights payments from F. Hoffmann-La Roche Ltd.. Dr. Lipsmeier has received research support from F. Hoffmann-La Roche Ltd.. Dr. Kilchenmann has received personal compensation for consulting, serving on a scientific advisory board, speaking, or other activities with F. Hoffmann-La Roche Ltd.. Dr. Rodrigues has received personal compensation for consulting, serving on a scientific advisory board, speaking, or other activities with GLG. Dr. Byrne has received personal compensation for consulting, serving on a scientific advisory board, speaking, or other activities with Roche and Genentech. Dr. Byrne has received personal compensation in an editorial capacity for Journal of Huntington’s Disease. Dr. Byrne has received research support from Roche.Dr. Bamdadian has received personal compensation for consulting, serving on a scientific advisory board, speaking, or other activities with Inovigate GmbH, and F. Hoffmann La-Roche. Dr. Gossens has received personal compensation for consulting, serving on a scientific advisory board, speaking, or other activities with F. Hoffmann-La Roche Ltd. Dr. Gossens holds stock and/or stock options in F. Hoffmann-La Roche Ltd.Dr. Schobel has received personal compensation for consulting, serving on a scientific advisory board, speaking, or other activities with SS is a full-time employee of F. Hoffmann–LaRoche.Dr. Lindemann has received personal compensation for consulting, serving on a scientific advisory board, speaking, or other activities with Inovigate. F. Hoffmann-La Roche Ltd. Dr. Wild has received personal compensation for consulting, serving on a scientific advisory board, speaking, or other activities with F. Hoffmann La Roche Ltd, Triplet Therapeutics, Dementia Discovery Fund, Mitoconix Bio Ltd, PTC Therapeutics and Takeda. All payments were made to UCL Consultants, a wholly owned subsidiary of University College London.. Dr. Wild has received research support from Research grant to University College London by F. Hoffmann-La Roche..