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[一例Shy-Drager综合征病例报告]

[A case report of Shy-Drager disease].

作者信息

Stepień A, Maksymiuk G

机构信息

Kliniki Psychoneurologicznej, Wojskowego Instytutu Medycyny Lotniczej, Warszawie.

出版信息

Neurol Neurochir Pol. 1997 Mar-Apr;31(2):335-41.

PMID:9380263
Abstract

Shy and Drager in 1960 first described a disease of adult onset characterized by the progressive development of autonomic deficits. The most frequent symptoms are related to orthostatic hypotension, impaired gastrointestinal motility, and urinary and sexual dysfunction. Recently the syndrome has been linked to other degenerative disorders like striatonigral degeneration and olivopontocerebellar atrophy. This combination of degenerative diseases has come to be known as multiple system atrophy (MSA). Shy-Drager disease is most often met in 5 and 6 decade in men and women equally. Apart from autonomic dysfunction symptoms we can find a combination of signs connected with lesions localized in different regions of brain and cord. However, hypertonic-akinetic syndrome is the most often met in patients as an additional symptom. According to this to make a differential diagnosis is often difficult and possible only after autopsy. We describe the case of 57 years old male patient who was admitted to our Clinic because of episodes of orthostatic hypotension, urinary incontinence, sexual impotence; and parkinsonism. In MRI there were changes described as degenerative localized in different parts of brain an cord. Treatment of hypertonic-akinetic syndrome with levodopa/carbidopa and agonists of dopamine receptors increased the frequency of hypotonic episodes. High level of sensitivity to antiparkinsonian drugs is well known feature found in patient with Shy-Drager disease.

摘要

1960年,夏伊和德雷格首次描述了一种成年发病的疾病,其特征为自主神经功能障碍的进行性发展。最常见的症状与体位性低血压、胃肠动力受损以及泌尿和性功能障碍有关。最近,该综合征已与其他退行性疾病如纹状体黑质变性和橄榄脑桥小脑萎缩联系起来。这种退行性疾病的组合已被称为多系统萎缩(MSA)。夏伊-德雷格病最常见于50和60岁的男性和女性。除了自主神经功能障碍症状外,我们还能发现与脑和脊髓不同区域病变相关的体征组合。然而,张力亢进-运动不能综合征是患者最常出现的附加症状。据此,进行鉴别诊断往往很困难,而且只有在尸检后才有可能。我们描述了一名57岁男性患者的病例,他因体位性低血压发作、尿失禁、性功能障碍以及帕金森综合征而入住我们的诊所。磁共振成像显示脑和脊髓不同部位存在退行性病变。用左旋多巴/卡比多巴和多巴胺受体激动剂治疗张力亢进-运动不能综合征增加了低血压发作的频率。对帕金森病药物高度敏感是夏伊-德雷格病患者的一个众所周知的特征。

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