F030 Disease progression of individuals with Huntington’s disease (HD) assessed via the HD digital motor score

<h3>Background</h3> Huntington’s disease (HD) is typically assessed in the clinic every 6–12 months. Because of measurement uncertainty and limited sensitivity, trends in disease progression for people only become apparent over several years. Remote digital monitoring has the potential to sensitively monitor disease progression. <h3>Aims</h3> We aim to determine whether frequent measurement of motor function can reveal distinguishable patterns of disease progression, and whether these HD progression patterns are associated with clinical and demographic parameters. <h3>Methods/Techniques</h3> We measured motor symptoms of HD across consecutive two-week windows for 16 months in 232 individuals with HD in the placebo arm of the GENERATION HD1 trial using the HD Digital Motor Score (HDDMS). We characterized the time course of progression by fitting polynomials to the data of individuals, and grouped them by their coefficients. We then studied the associations between the groups and various clinical and demographic factors. <h3>Results/Outcome</h3> The HDDMS produces reasonably smooth, interpretable disease progression trajectories at the level of individuals at the time scale of months. We distinguish six different progression patterns based on best-fitting polynomial functions. One group with initial fast progression that later stabilizes has significantly higher scores at baseline across several scales (e.g. chorea, TMS). <h3>Conclusions</h3> The HDDMS allows sensitive assessment of disease progression in the clinical trial context, and may also enable more sensitive assessment of the disease course of people with HD in clinical practice.