[Shy-Drager syndrome]. 2010

Magdaléna Dusejovská, and Tomás Vareka, and Jaroslav Macásek, and Karel Hrubant, and Ales Zák, and Miroslav Zeman
Univerzita Karlova v Praze, 1. lékarská fakulta, IV. interní klinika VFN.

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.

UI MeSH Term Description Entries
D008297 Male Males
D008875 Middle Aged An adult aged 45 - 64 years. Middle Age
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D012791 Shy-Drager Syndrome A progressive neurodegenerative condition of the central and autonomic nervous systems characterized by atrophy of the preganglionic lateral horn neurons of the thoracic spinal cord. This disease is generally considered a clinical variant of MULTIPLE SYSTEM ATROPHY. Affected individuals present in the fifth or sixth decade with ORTHOSTASIS and bladder dysfunction; and later develop FECAL INCONTINENCE; anhidrosis; ATAXIA; IMPOTENCE; and alterations of tone suggestive of basal ganglia dysfunction. (From Adams et al., Principles of Neurology, 6th ed, p536) Autonomic Failure, Progressive,Idiopathic Orthostatic Hypotension, Shy-Drager Type,Orthostatic Hypotension, Dysautonomic,Dysautonomia-Orthostatic Hypotension Syndrome,Syndrome, Dysautonomia-Orthostatic Hypotension,Autonomic Failures, Progressive,Dysautonomia Orthostatic Hypotension Syndrome,Dysautonomia-Orthostatic Hypotension Syndromes,Dysautonomic Orthostatic Hypotension,Dysautonomic Orthostatic Hypotensions,Failure, Progressive Autonomic,Failures, Progressive Autonomic,Hypotension Syndrome, Dysautonomia-Orthostatic,Hypotension Syndromes, Dysautonomia-Orthostatic,Hypotension, Dysautonomic Orthostatic,Hypotensions, Dysautonomic Orthostatic,Idiopathic Orthostatic Hypotension, Shy Drager Type,Orthostatic Hypotensions, Dysautonomic,Progressive Autonomic Failure,Progressive Autonomic Failures,Shy Drager Syndrome,Syndrome, Dysautonomia Orthostatic Hypotension,Syndrome, Shy-Drager,Syndromes, Dysautonomia-Orthostatic Hypotension

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