Early sympathetic hyperactivity in Huntington's disease

Early sympathetic hyperactivity in Huntington's disease
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DOI:
10.1111/j.1468-1331.2004.00894.x
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发表时间:
2004-12-01
影响因子:
5.1
通讯作者:
Peterlin, B
Peterlin, B
中科院分区:
医学3区
文献类型:
--
作者:
Kobal, J;Meglic, B;Peterlin, B

文献摘要

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为了探讨亨廷顿病(HD)是否影响自主神经系统(ANS)功能,对33例基因检测阳性的受试者进行了研究。根据Shoulson和Fahn(S&女)HD残疾分级标准,将受试者分为3个亚组:亚组1(8例无症状基因携带者)、亚组2(13例轻残HD患者)和亚组3(8例中残HD患者和4例重度HD患者)。进行心血管自主神经试验(Valsalva动作、深呼吸试验、持续握力试验、立位试验)和心率变异性(HRV)频谱分析。将结果与一组匹配的对照组进行比较。亚组1低频功率(LFB)较高(P<0.05)。亚组2的LFB功率增加,LFB/高频段(HFB)系数增加,持续握手的血压反应增强(P<0.05)。亚组3对持续握手、下呼吸道(P<0.05)和立位比(P<0.01)的血压反应显著降低。我们的结果表明,无症状基因携带者和轻度残疾的HD患者存在交感神经功能亢进。相反,晚期HD患者存在ANS功能减退。
To investigate whether Huntington's disease (HD) affects autonomic nervous system (ANS) functioning 33 subjects with positive genetic test results were studied. The subjects were classified according to Shoulson and Fahn (S&F) HD disability scale into three subgroups: subgroup 1 (eight asymptomatic gene carriers), subgroup 2 (13 mildly disabled HD patients) and subgroup 3 (eight moderately and four severely disabled HD patients). A battery of cardiovascular autonomic tests (Valsalva maneuver, deep breathing test, sustained handgrip test, orthostatic test) and the spectral analysis of heart rate variability (HRV) were performed. The results were compared with a group of matched controls. In subgroup 1, there was a higher power of low-frequency band (LFB) (P < 0.05). In subgroup 2 a higher power of LFB was detected, LFB/high-frequency band (HFB) coefficient was increased and the blood pressure response to sustained handgrip was elevated (P < 0.05). Subgroup 3 showed significantly lower blood pressure response to sustained handgrip, lower respiratory (P < 0.05) and orthostatic ratio (P < 0.01). Our results suggest that sympathetic hyperfunction is present in asymptomatic gene carriers and mildly disabled HD patients. Contrary to that, ANS hypofunction was found in advanced HD patients.