Dusejovská Magdaléna, Vareka Tomás, Macásek Jaroslav, Hrubant Karel, Zák Ales, Zeman Miroslav
Univerzita Karlova v Praze, 1. lékarská fakulta, IV. interní klinika VFN.
Cas Lek Cesk. 2010;149(5):225-8.
Shy-Drager syndrome is a rare neurological disease with a poor prognosis causing a generalised autonomy dysfunction. The disorder is also known as multiple system atrophy, the orthostatic hypotension syndrome or Shy-McGee-Drager syndrome. Patients have mainly dysautonomic symptoms. Patients suffer from orthostatic hypotension, bradycardia, anhidrosis, failure of accommodation, sialoporia, low tears secretion, gastrointestinal dysmotility and incomplete emptying of the urinary bladder. Neuropathological examination of patient's brains demonstrated neurodegenerative changes of the structures of central nervous system, mainly of brainstem. The Shy-Drager syndrome results from striatonigral and olivo-ponto-cerebellar atrophy and from accumulation of alpha-synuclein in these structures. The patients suffering from the Shy-Drager syndrome are very often misdiagnosed because of overlap of symptomatology with psychiatric and psychosomatic diseases. It is also very difficult to make the diagnosis because of complexity of symptoms. The prognosis of Shy-Drager syndrome is very poor; patients are markedly disabled and have shorter survival.
夏伊-德雷格综合征是一种罕见的神经系统疾病，预后较差，可导致全身自主神经功能障碍。该疾病也被称为多系统萎缩、直立性低血压综合征或夏伊-麦吉-德雷格综合征。患者主要有自主神经功能障碍症状。患者会出现直立性低血压、心动过缓、无汗、调节功能障碍、唾液分泌过多、泪液分泌减少、胃肠动力障碍以及膀胱排空不全。对患者大脑进行神经病理学检查显示中枢神经系统结构出现神经退行性变化，主要是脑干。夏伊-德雷格综合征是由纹状体黑质和橄榄脑桥小脑萎缩以及这些结构中α-突触核蛋白的积累引起的。由于症状与精神疾病和身心疾病重叠，患有夏伊-德雷格综合征的患者常常被误诊。由于症状复杂，做出诊断也非常困难。夏伊-德雷格综合征的预后非常差；患者明显残疾，生存期较短。
