Department of Physiology, All India Institute of Medical Sciences, New Delhi, India.
Department of Physiology, VMMC & Safdarjung Hospital, New Delhi, India.
Acta Neurol Scand. 2019 Nov;140(5):350-358. doi: 10.1111/ane.13151. Epub 2019 Sep 1.
To assess the time and frequency domain measures of cardiac autonomic activity/tone in patients of genetically defined spinocerebellar ataxia (SCA) types 1 and 2, as well as to decipher the probable associations among the cardiovascular autonomic parameters and genetic and clinical characteristics.
Simultaneous 5-min recording of RR interval (RRI) and blood pressure (BP) for the calculation of heart rate variability (HRV), blood pressure variability (BPV) and baroreflex sensitivity (BRS) were performed in genotypically confirmed SCA1 (n = 31) and SCA2 (n = 40) patients and healthy controls (n = 40). Additionally, the International Cooperative Ataxia Rating Scale (ICARS) was used for scoring of clinical severity in SCA patients.
Time and frequency domain parameters of HRV, BPV and BRS were depressed in SCA1 and SCA2 subtypes as compared to controls, although there was no statistically significant difference in autonomic tone between the two SCA subtypes. On correlation analysis, autonomic tone parameters were found to be associated with the clinical and genetic features of the SCA subtypes. Also, ICARS was associated with the genotype (CAG repeat length) in SCA2 patents.
Cardiac autonomic tone is depressed in both SCA1 and 2 as compared to healthy controls while the two SCA subtypes do not differ in terms of autonomic tone. Also, a typical association exists between disease characteristics and autonomic indices.
评估遗传性脊髓小脑共济失调(SCA)1 型和 2 型患者心脏自主活动/张力的时域和频域指标,并解析心血管自主参数与遗传和临床特征之间可能存在的关联。
对基因确诊的 SCA1(n=31)和 SCA2(n=40)患者和健康对照组(n=40)进行了 5 分钟同步 RR 间期(RRI)和血压(BP)记录,以计算心率变异性(HRV)、血压变异性(BPV)和压力反射敏感性(BRS)。此外,还使用国际合作共济失调评定量表(ICARS)对 SCA 患者的临床严重程度进行评分。
与对照组相比,SCA1 和 SCA2 亚型的 HRV、BPV 和 BRS 的时域和频域参数均降低,尽管两种 SCA 亚型之间的自主张力没有统计学差异。相关性分析显示,自主张力参数与 SCA 亚型的临床和遗传特征相关。此外,ICARS 与 SCA2 患者的基因型(CAG 重复长度)相关。
与健康对照组相比,SCA1 和 SCA2 患者的心脏自主张力降低,而两种 SCA 亚型在自主张力方面没有差异。此外,疾病特征与自主指数之间存在典型的关联。