Shy-Drager syndrome
Authors:
Magdaléna Dušejovská; Tomáš Vařeka; Jaroslav Macášek; Karel Hrubant; Aleš Žák; Miroslav Zeman
Authors‘ workplace:
Univerzita Karlova v Praze, 1. lékařská fakulta, IV. interní klinika VFN
Published in:
Čas. Lék. čes. 2010; 149: 225-228
Category:
Case Report
Overview
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.
Key words:
Shy-Drager syndrome, multiple system atrophy, catecholamines, parkinsonian syndrome, autonomy dysfunction, orthostatic hypotension.
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Journal of Czech Physicians
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