Poster 14: Earliest Functional Declines in Huntington's Disease

Objective Declines in functional skills are observed in patients with Huntington's disease (HD) and have both clinical and research implications. However, the specific areas of early decline have not been investigated. We sought to determine which skill domains weaken most frequently in patients prior to formal diagnosis, as well as the clinical correlates of these early declines. Methods Using a large cohort of prediagnosed patients (i.e., DCL Results Occupational decline was the most frequently reported decline, with 30.10% (TFC) and 26.32% (FAS) of patients reporting some loss of ability to engage in their typical work. Inability to manage finances (TFC 21.87%, FAS 17.42%) and inability to drive safely (FAS 16.24%) were other common functional declines. A minority of patients with DCL = 0 or 1 reported functional decline (TFC 15.84%, FAS 14.26%); however, a majority of those with a DCL = 2 reported functional decline (TFC 77.54%, FAS 71.03%). The most common functional declines showed relationships with motor functioning, cognitive tests, and depression in a multivariate analysis where all 3 factors were considered simultaneously. Conclusions Our analysis of the TFC and FAS provides additional clues as to the earliest functional losses in patients at risk for HD. Fruitful areas for expanded assessment of functional status are performance at work, ability to manage finances, and driving changes. Additionally, existing functional measures may have limited utility when detecting changes in patients with no or minimal motor signs.