Cognitive and autonomic dysfunction in presymptomatic and early Huntington's disease
Cognitive and autonomic dysfunction in presymptomatic and early Huntington's disease
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DOI：
10.1007/s00415-014-7319-6
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发表时间：
2014-06-01
影响因子：
6
通讯作者：
Strucl, Martin
中科院分区：
文献类型：
--
作者：
Kobal, Jan;Melik, Ziva;Strucl, Martin
亨廷顿舞蹈病的特点是运动、认知和行为障碍。亨廷顿病患者的自主神经系统会出现异常变化，这些变化甚至在其他症状出现之前就被发现。轻微的认知功能障碍可能在其他临床表现之前出现。本研究的目的是调查亨廷顿病症状前和早期的自主神经系统对心算的反应以及自主神经和认知/运动功能之间的关系。我们检查了 15 名症状前亨廷顿病基因携带者 (PHD)、15 名早期亨廷顿病患者 (EHD) 和 30 名健康对照者。 PHD 和 EHD 组根据统一亨廷顿病评定量表 (UHDRS) 运动评分确定。在休息期间和简单的心算测试期间测量心电图、心率、收缩压和舒张压以及皮肤激光多普勒通量。 UHDRS 认知测试组用于确定认知功能障碍。在心算过程中，PHD/EHD 的心率增加明显低于对照组。 EHD 中发现微血管对心算的反应降低。在 PHD/EHD 组中，发现激光多普勒通量响应与符号数字模态测试分数之间以及激光多普勒通量响应与 UHDRS 运动分数之间存在显着相关性。亨廷顿病中心血管系统的中枢自主神经失调似乎与其他中枢神经系统的退化同时发生。这一发现具有重要意义，因为它可以对疾病进展进行简单且无创的检测。
Huntington's disease is characterized by disorders of movement, cognition and behavior. Individuals with Huntington's disease display aberrant changes in the autonomic nervous system that are detected even before the onset of other symptoms. Subtle cognitive dysfunction may start before other clinical manifestations. The aim of the present study was to investigate the autonomic nervous system response to mental arithmetic and the relationship between the autonomic and cognitive/motor function in presymptomatic and early Huntington's disease. We examined 15 presymptomatic Huntington's disease gene carriers (PHD), 15 early Huntington's disease patients (EHD) and 30 healthy controls. PHD and EHD groups were determined according to Unified Huntington's Disease Rating Scale (UHDRS) motor score. ECG, heart rate, systolic and diastolic blood pressure, and cutaneous laser Doppler flux were measured during rest and during a simple mental arithmetic test. UHDRS cognitive test battery was applied to determine cognitive dysfunction. During mental arithmetic, the heart rate of PHD/EHD increased significantly less than that of controls. Decreased microvascular response to mental arithmetic was found in EHD. Significant correlations for the PHD/EHD group were found between laser Doppler flux response and Symbol Digit Modalities Test score, and between laser Doppler flux response and UHDRS motor score. It seems that central autonomic dysregulation of cardiovascular system in Huntington's disease goes along with the degeneration of other central neuronal systems. This finding is relevant as it could enable simple and noninvasive testing of disease progression.