Cardiovascular autonomic control in myotonic dystrophy type 1: a correlative study with clinical and genetic data
Cardiovascular autonomic control in myotonic dystrophy type 1: a correlative study with clinical and genetic data
复制标题
DOI：
10.1016/j.nmd.2003.11.002
复制
发表时间：
2004-02-01
影响因子：
2.8
通讯作者：
Vita, G
中科院分区：
文献类型：
--
作者：
Di Leo, R;Rodolico, C;Vita, G
强直性肌营养不良患者的自主神经系统评价结果相互矛盾，其与心脏紊乱的关系尚不清楚。23例强直性肌营养不良1型患者进行了调查的电池的六个心血管自主神经测试和功率谱分析的心率变异性。虽然15例患者（65%）在某些测试中显示异常或临界结果，但只有1例患者有明确的自主神经损伤，如两个或两个以上异常测试所示。作为一个群体，强直性肌营养不良1型患者在深呼吸时心率变异性显著降低（P < 0.0001）。副交感神经测试的独家参与表明，轻度迷走神经功能障碍发生在一些强直性肌营养不良1型患者。结果表明，这种自主神经异常不是：（1）周围神经病变的一部分;（2）与胞嘧啶-胸腺嘧啶-鸟嘌呤重复大小或呼吸模式有关。功率谱分析显示仰卧位低频带减少，这是但不完全是交感神经活动的标志。与病程呈负相关（P < 0.04），提示随着病程的进展，病情也在加重。站立后记录的低频功率与心脏受累显著相关（P < 0.02）。我们的研究结果表明，混合，特别是副交感神经，自主神经功能障碍可能发生在强直性肌营养不良1型，虽然这不是一个主要的发现。它可能在心脏异常的发生中起作用，或增加突发心血管事件的风险。(C)2003 Elsevier B. V.保留所有权利。
The autonomic nervous system has been evaluated in myotonic dystrophy with contradictory results and its relationship with heart disturbances remains unclear. Twenty-three patients with myotonic dystrophy type 1 were investigated by a battery of six cardiovascular autonomic tests and power spectral analysis of heart rate variability. Although 15 patients (65%) revealed abnormal or borderline results in some tests, only one patient had a definite autonomic damage, as indicated by two or more abnormal tests. As a group, myotonic dystrophy type 1 patients showed a significant reduction of heart rate variability during deep breathing (P < 0.0001). The exclusive involvement of parasympathetic tests suggests that a mild vagal dysfunction occurs in some myotonic dystrophy type 1 patients. The results indicate that such autonomic abnormalities are not: (1) part of a peripheral neuropathy; (2) related to cytosine-thymine-guanine repeat size or breathing pattern. Power spectral analysis showed a reduction of supine low-frequency band, which is, but not exclusively, a marker of sympathetic activity. It was inversely correlated to disease duration (P < 0.04), suggesting a progression as the disease advances. A low-frequency power, recorded after standing, was significantly associated (P < 0.02) with presence of heart involvement. Our findings suggest that a mixed, especially parasympathetic, autonomic dysfunction may occur in myotonic dystrophy type 1, although it is not a major finding. It could play a role in the occurrence of cardiac abnormalities, or increase the risk of sudden cardiovascular events. (C) 2003 Elsevier B.V. All rights reserved.