Shy-Drager syndrome
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Shy-Drager syndrome is a rare, progressively degenerative disease of the autonomic nervous system. Its cause is not known, and there is no cure at this time. It is named after Dr Milton Shy and Dr Glenn Drager, who identified this syndrome in 1960. It is now considered a member of the class of closely-related degenerative diseases grouped under the term multiple system atrophy (MSA).
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Signs and symptoms
Symptoms of this syndrome can include: dizziness or fainting when standing up (known as postural or orthostatic hypotension); urinary incontinence; muscular tremors; impotence; dry mouth and skin; visual problems; trouble regulating body temperature due to abnormal sweating; breathing difficulties; Parkinson-like tremors.
As other symptoms of the "Parkinson plus syndromes" are often present, it may be hard to delineate the diagnosis. The term multiple system atrophy is therefore favoured (Consensus Committee 1996).
Prognosis
Most people with this syndrome are disabled within 7-8 years of the initial onset of symptoms; few live longer than 15 years afterwards.
Treatment
The most serious problem (the drop in blood pressure on standing up) often responds to fludrocortisone, a synthetic mineralocorticoid.
Notable patients
Johnny Cash, the country singer, was believed to have Shy-Dager syndrome when he died on September 12, 2003, although that diagnosis was later changed to one of autonomic neuropathy, associated with diabetes.
References
- Shy GM, Drager GA. A neurological syndrome associated with orthostatic hypotension: a clinical-pathologic study. Arch Neurol 1960;2:511-27. PMID 14446364.
- The Consensus Committee of the American Autonomic Society and the American Academy of Neurology. Consensus statement on the definition of orthostatic hypotension, pure autonomic failure, and multiple system atrophy. Neurology 1996;46:1470. PMID 8628505.
External link
- Emedicine article (http://www.emedicine.com/neuro/topic671.htm)