Flügel K A, Kügelgen B
Fortschr Neurol Psychiatr Grenzgeb. 1978 Mar;46(3):107-43.
The most prominent symptom of Shy-Drager syndrome is the asympathicotonic orthostatic (postural) hypotension, which is associated with a number of additional autonomic and neurological disturbances: disorders of micturition, sphincter disturbances, impotence, anhidrosis, hypokinesia, rigidity, pyramidal symptoms, cerebellar dysfunction and nuclear pareses due to anterior horn cell degeneration. The various disorders are not caused by ischemia or hypotension, but they represent parts of a multisystemic disease of still unknown etiology. According to different extension and neuropathological criteria it has been suggested to distinguish two types of neurogenic (idiopathic) orthostatic hypotension. Moreover, differential diagnosis of the Shy-Drager syndrome has to consider postural hypotension occuring as a symptom in some neuropathies and Parkinson's disease. Symptomatology, course, prognosis and treatment of Shy-Drager syndrome are described, as well as relevant findings of apparative investigations, pharmacological and hemodynamic tests and neuropathological findings in autopsied cases reported in the literature. This review was initiated by two clinically investigated cases of Shy-Drager syndrome.
夏伊-德雷格综合征最突出的症状是失交感神经张力性直立性（体位性）低血压，它还伴有许多其他自主神经和神经功能障碍：排尿障碍、括约肌功能紊乱、阳痿、无汗、运动减少、僵硬、锥体束征、小脑功能障碍以及由于前角细胞变性导致的核性轻瘫。这些各种功能障碍并非由缺血或低血压引起，而是一种病因仍不明的多系统疾病的一部分。根据不同的累及范围和神经病理学标准，有人建议区分两种类型的神经源性（特发性）直立性低血压。此外，夏伊-德雷格综合征的鉴别诊断必须考虑在某些神经病变和帕金森病中作为症状出现的体位性低血压。本文描述了夏伊-德雷格综合征的症状学、病程、预后和治疗，以及文献中报道的尸检病例的器械检查、药理学和血液动力学测试的相关结果及神经病理学发现。这篇综述由两例经临床研究的夏伊-德雷格综合征病例引发。
