Definition of Shy-Drager syndrome

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Shy-Drager syndrome: A mysterious progressive disorder of the central and sympathetic nervous systems, also called multiple system atrophy with postural hypotension. Postural (or orthostatic) hypotension is an excessive drop in blood pressure when the patient stands up, which causes dizziness or momentary blackouts. Symptoms of autonomic failure such as constipation, impotence in men, and urinary incontinence usually predominate early in the course of the disease. Constipation may be unrelenting. Other symptoms that may develop later include impaired speech, difficulties with breathing and swallowing, and inability to sweat (anhidrosis). extrapyramidal symptoms or cerebellar symptoms may predominate early in the course. There may be tremors and rigidity reminiscent of Parkinson disease. Loud snoring is common, as is sleep apnea. Shy-Drager syndrome usually ends in death within 15 years of the diagnosis. Breathing problems such as aspiration, stridor (high-pitched breathing sounds due to airway obstruction), or cardiopulmonary arrest are the common causes of death.

The condition is named after Dr. Milton Shy (1919-1967) of the National Institutes of Health and Dr Glenn Drager (1917-1967) from Baylor College of Medicine who first described the syndrome in 1960. The country singer Johnny Cash (1932-2003) had the Shy-Drager syndrome.

Common Misspellings: shy-drager syndrone