Abstract
Patients with adult-onset Huntington’s Disease (AOHD) have been found to have dysfunction of the autonomic nervous system that is thought to be secondary to neurodegeneration causing dysfunction of the brain–heart axis. However, this relationship has not been investigated in patients with juvenile-onset HD (JOHD). The aim of this study was to compare simple physiologic measures between patients with JOHD (n = 27 participants with 64 visits) and participants without the gene expansion that causes HD (GNE group; n = 259 participants with 395 visits). Using data from the Kids-JOHD study, we compared mean resting heart rate (rHR), systolic blood pressure (SBP), and diastolic blood pressure (DBP) between the JOHD and GNE groups. We also divided the JOHD group into those with childhood-onset JOHD (motor diagnosis received before the age of 13, [n = 16]) and those with adolescent-onset JOHD (motor diagnosis received at or after the age of 13 [n = 11]). We used linear mixed-effects models to compare the group means while controlling for age, sex, and parental socioeconomic status and including a random effect per participant and family. For the primary analysis, we found that the JOHD group had significant increases in their rHR compared to the GNE group. Conversely, the JOHD group had significantly lower SBP compared to the GNE group. The JOHD group also had lower DBP compared to the GNE group, but the results did not reach significance. SBP and DBP decreased as disease duration of JOHD increased, but rHR did not continue to increase. Resting heart rate is more sensitive to changes in autonomic function as compared to SBP. Therefore, these results seem to indicate that early neurodegenerative changes of the central autonomic network likely lead to an increase in rHR while later progression of JOHD leads to changes in blood pressure. We hypothesize that these later changes in blood pressure are secondary to neurodegeneration in brainstem regions such as the medulla.
Highlights
Huntington’s Disease (HD) is an inherited, neurodegenerative disease that causes motor, cognitive, and behavioral symptoms [1]
We found that the juvenile-onset HD (JOHD) group had significant increases in their resting heart rate (rHR) compared to the GNE group
We have demonstrated for the first time that patients with JOHD have significant changes in physiologic measures of autonomic nervous system (ANS) function compared to healthy controls
Summary
Huntington’s Disease (HD) is an inherited, neurodegenerative disease that causes motor, cognitive, and behavioral symptoms [1]. These symptoms are thought to be caused by striatal degeneration, which is the hallmark of HD [2,3]. Dysfunction of the autonomic nervous system (ANS) has been described in patients with adult-onset HD (AOHD) [4,5,6,7,8]. Patients with AOHD seem to have enhanced sympathetic tone compared to healthy controls. Patients with juvenile-onset HD (JOHD) represent a rare group of individuals with significant neurodegeneration that begins very early in life, but physiologic measures of ANS
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