Interní Med. 2010; 12(7-8): 382-384

Orthostatic hypotension in Shy-Drager syndrome

MUDr.Tomáš Vařeka, MUDr.Magdaléna Dušejovská, MUDr.Jaroslav Macášek, prof.MUDr.Aleš Žák, DrSc., doc.MUDr.Miroslav Zeman, CSc.

IV. interní klinika, 1. lékařská fakulta Univerzity Karlovy v Praze a VFN, Praha

Chronic arterial hypotension refers to states when the blood pressure (BP) readings are below < 100/65 mm Hg in a long term. It is encountered

in organic damage to the central nervous system (CNS) or in diseases of the peripheral nervous system (PNS); drug-induced

hypotension is most commonly seen. Of major clinical significance is orthostatic hypotension that significantly limits the patients in their

activities of daily living. A case of a patient with organic damage to the CNS, Shy-Drager syndrome (SDS), with orthostatic hypotension as

a limiting factor, is described in the present case report. SDS is a rare and serious type of autonomic vegetative dysfunction, also referred

to as multiple system atrophy (MSA) or neurogenic orthostatic hypotension. It is a group of symptoms caused by the involvement of the

autonomic nervous system. The characteristic features are orthostatic hypotension, bradycardia, accommodation disorder, anhidrosis,

impaired tear and saliva secretion, gastrointestinal tract (GIT) dysmotility and voiding dysfunction. The underlying pathophysiology of

the disease involves neurodegenerative changes in the CNS in the brainstem region with resulting atrophy and pathological deposits

of alpha-synuclein in these CNS areas. The prognosis is serious; the disease decreases the quality of life and significantly reduces the

survival of patients.

Keywords: Shy-Drager syndrome, multiple system atrophy, orthostatic hypotension, catecholamines, autonomic dysfunction

Published: August 12, 2010  Show citation