Department of Psychiatry, Carver College of Medicine at the University of Iowa, Iowa City, IA, USA; Department of Neurology, Carver College of Medicine at the University of Iowa, Iowa City, IA, USA; University of Iowa College of Pharmacy, Iowa City, IA, USA.
Stead Family Children's Hospital at the University of Iowa, Iowa City, IA, USA.
Auton Neurosci. 2021 Mar;231:102775. doi: 10.1016/j.autneu.2021.102775. Epub 2021 Feb 5.
Autonomic nervous system (ANS) dysfunction has been described in adults with motor-manifest Huntington's Disease (HD) or those who are near their predicted motor onset. It is unclear if ANS dysfunction is present years prior to the onset of motor symptoms of HD. To bridge this gap in knowledge, we compared crude markers of ANS function between children with the gene-expansion that causes HD (GE group) who were decades from their predicted motor onset and gene-non-expanded children (GNE group).
We included participants from the Kids-HD study who were <18 years old. Linear mixed effects regression models were constructed that controlled for sex, age, and BMI, and included a random effect per participant and per family. We compared resting heart rate (rHR), core body temperature (CBT), systolic blood pressure (SBP), and diastolic blood pressure (DBP) between the GE (n = 84) and GNE (n = 238) groups. We then grouped participants from the GE group based on their predicted years to onset (YTO) and compared their vital signs to the GNE group.
The GE group had higher rHR (∆ = 3.83, p = 0.0064), SBP (∆ = 2.38, p = 0.032), and CBT (∆ = 0.16, t = 2.92, p = 0.007). The mean rHR and CBT became significantly elevated compared to the GNE group in participants who had 15-25 YTO and those who had <15 YTO. The mean SBP of participants who had 25-35 YTO was significantly elevated compared to the GNE group.
ANS dysfunction in HD seems to occur approximately 20 years prior to the predicted onset of motor symptoms of HD.
自主神经系统(ANS)功能障碍已在运动表现亨廷顿病(HD)的成人或接近运动发病的成人中描述。尚不清楚 ANS 功能障碍是否在出现 HD 运动症状之前多年就存在。为了弥补这一知识空白,我们比较了具有导致 HD 的基因扩展(GE 组)的数十年后才预计会出现运动症状的儿童和基因未扩展的儿童(GNE 组)之间的 ANS 功能的粗略标志物。
我们纳入了来自 Kids-HD 研究的年龄<18 岁的参与者。构建了线性混合效应回归模型,这些模型控制了性别、年龄和 BMI,并包括每个参与者和每个家庭的随机效应。我们比较了 GE 组(n=84)和 GNE 组(n=238)的静息心率(rHR)、核心体温(CBT)、收缩压(SBP)和舒张压(DBP)。然后,我们根据 GE 组的预计发病年限(YTO)将参与者分组,并将他们的生命体征与 GNE 组进行比较。
GE 组的 rHR(∆=3.83,p=0.0064)、SBP(∆=2.38,p=0.032)和 CBT(∆=0.16,t=2.92,p=0.007)更高。在 YTO 为 15-25 年和<15 年的参与者中,rHR 和 CBT 的平均值与 GNE 组相比显著升高。YTO 为 25-35 年的参与者的平均 SBP 与 GNE 组相比显著升高。
HD 中的 ANS 功能障碍似乎在预计出现 HD 运动症状之前大约 20 年就发生了。