Sympathetic cardioneuropathy in dysautonomias
Sympathetic cardioneuropathy in dysautonomias
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DOI：
10.1056/nejm199703063361004
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发表时间：
1997-03-06
影响因子：
158.5
通讯作者：
Kopin, IJ
中科院分区：
文献类型：
--
作者：
Goldstein, DS;Holmes, C;Kopin, IJ
背景植物神经功能异常的分类一直很混乱，其病理生理学也不清楚。我们使用胸部正电子发射断层扫描和评估交感神经递质去甲肾上腺素进入心脏静脉引流（心脏去甲肾上腺素溢出）的速率，检查了获得性特发性植物神经功能异常患者心脏的交感神经支配。我们相关的实验室检查结果的迹象交感神经循环衰竭（体位性低血压和异常血压反应与Valsalva动作），中枢神经变性，并与左旋多巴，卡比多巴（Sinemet）治疗的反应性。方法心脏扫描后获得静脉注射6-[F-18]氟多巴在26例自主神经功能障碍。14名患者出现交感神经循环衰竭，其中3名患者没有中枢神经退行性变的迹象（纯自主神经功能衰竭），2名患者出现对左旋多巴-卡比多巴治疗有反应的帕金森综合征，9名患者出现对左旋多巴-卡比多巴治疗无反应的中枢神经退行性变（Shy-Drager综合征）。心脏去甲肾上腺素溢出率估计的基础上静脉输注的[H-3]去甲肾上腺素的浓度在导管的右侧heart.Results患者与纯粹的自主神经功能衰竭或帕金森综合征和交感神经循环衰竭没有心肌6-[F-18]氟多巴胺衍生的放射性或心脏去甲肾上腺素溢出，表明心肌交感神经末梢的损失，而Shy-Drager综合征患者的6-[F-18]氟多巴胺衍生放射性水平升高，表明交感神经末梢完整，神经交通缺失，没有交感神经循环衰竭的自主神经功能障碍患者的6-[F-18]氟多巴胺水平正常。结论6-[F-18]氟多巴胺正电子能显像结果显示，发射断层摄影术和神经化学分析支持基于交感神经循环衰竭的发生、中枢神经变性的迹象和对左旋多巴-卡比多巴的反应性的自主神经机能障碍的新临床病理生理学分类。(C)1997年，马萨诸塞州医学会。
Background The classification of dysautonomias has been confusing, and the pathophysiology obscure, We examined sympathetic innervation of the heart in patients with acquired, idiopathic dysautonomias using thoracic positron-emission tomography and assessments of the entry rate of the sympathetic neurotransmitter norepinephrine into the cardiac venous drainage (cardiac norepinephrine spillover). We related the laboratory findings to signs of sympathetic neurocirculatory failure (orthostatic hypotension and abnormal blood-pressure responses associated with the Valsalva maneuver), central neural degeneration, and responsiveness to treatment with levodopa-carbidopa (Sinemet).Methods Cardiac scans were obtained after intravenous administration of 6-[F-18]fluorodopamine in 26 patients with dysautonomia. Fourteen had sympathetic neurocirculatory failure - three with no signs of central neurodegeneration (pure autonomic failure), two with parkinsonism responsive to treatment with levodopa-carbidopa, and nine with central neurodegeneration unresponsive to treatment with levodopa-carbidopa (the Shy-Drager syndrome). The rates of cardiac norepinephrine spillover were estimated on the basis of concentrations of intravenously infused [H-3]norepinephrine during catheterization of the right side of the heart.Results Patients with pure autonomic failure or parkinsonism and sympathetic neurocirculatory failure had no myocardial 6-[F-18]fluorodopamine-derived radioactivity or cardiac norepinephrine spillover, indicating loss of myocardial sympathetic-nerve terminals, whereas patients with the Shy-Drager syndrome had increased levels of 6-[F-18]fluorodopamine-derived radioactivity, indicating intact sympathetic terminals and absent nerve traffic, Patients with dysautonomia who did not have sympathetic neurocirculatory failure had normal levels of 6-[F-18]fluorodopamine-derived radioactivity in myocardium and normal rates of cardiac norepinephrine spillover.Conclusions The results of 6-[F-18]fluorodopamine positron-emission tomography and neurochemical analyses support a new clinical pathophysiologic classification of dysautonomias, based on the occurrence of sympathetic neurocirculatory failure, signs of central neurodegeneration, and responsiveness to levodopa-carbidopa. (C) 1997, Massachusetts Medical Society.