Autonomic Changes in Juvenile-Onset Huntington's Disease.
Autonomic Changes in Juvenile-Onset Huntington's Disease.
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DOI：
10.3390/brainsci10090589
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发表时间：
2020-08-26
期刊：
影响因子：
3.3
通讯作者：
Nopoulos PC
中科院分区：
文献类型：
--
作者：
Schultz JL;Nopoulos PC
成人起病亨廷顿病(AOHD)患者被发现存在自主神经系统功能障碍，这被认为是继发于神经退行性变导致脑心轴功能障碍。然而，这种关系在青少年起病HD(JOHD)患者中尚未被研究。本研究的目的是比较JOHD患者(n=27名访问过的参与者)和没有导致HD的基因扩展的参与者(Gne组；n=259名参与者，395次访问)之间的简单生理指标。使用来自Kids-JOHD研究的数据，我们比较了JOHD组和GNOE组之间的平均静息心率(RHR)、收缩压(SBP)和舒张压(DBP)。我们还将JOHD组分为儿童期JOHD组(13岁前接受运动诊断[n=16])和青春期JOHD组(13岁或13岁后接受运动诊断[n=11])。我们使用线性混合效应模型来比较群体平均数，同时控制年龄、性别和父母的社会经济地位，并包括每个参与者和家庭的随机影响。对于初步分析，我们发现JOHD组与GNOE组相比，他们的RHR显著增加。相反，JOHD组的SBP显著低于GNOE组。与GNOE组相比，JOHD组的DBP也较低，但结果未达显著水平。SBP、DBP随JOHD病程延长而降低，RHR不再持续升高。与SBP相比，静息心率对自主神经功能的变化更敏感。因此，这些结果似乎表明，早期中枢自主神经退行性改变可能导致RHR增加，而JOHD进展后期可能导致血压变化。我们假设，这些后来的血压变化是继发于脑干区域(如延髓)的神经变性。
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.
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