3244 The Autonomic Nervous System as a Potential Therapeutic Target in Huntington Disease

OBJECTIVES/SPECIFIC AIMS: This study (1) investigated the presence and severity of autonomic nervous system (ANS) dysfunction in patients with pre-symptomatic Huntington Disease (HD) and (2) determined if pharmacologic manipulation of the ANS could modify the progression of HD. METHODS/STUDY POPULATION: Using a unique data set of children at-risk for HD (the Kids-HD study), markers of autonomic function (resting heart rate [rHR], blood pressure [BP], and core body temperature [CBT]) were compared between pre-symptomatic, gene-expanded children (psGE) and healthy developing children using mixed models analyses controlling for sex, age, and body mass index. Included participants had to be < 18 years old and be at least 10 years from their predicted motor diagnosis of HD. Using the Enroll-HD database, inverse-propensity score weighted, Cox Regression analyses investigated the effects of beta-blockers on the timing of motor diagnosis of presymptomatic, adult patients with HD. RESULTS/ANTICIPATED RESULTS: Compared to healthy controls, the psGE participants had significantly (p