Shy-Drager syndrome

Shy-Drager syndrome


Caption =
DiseasesDB = 12042
ICD10 = ICD10|G|90|3|g|90
ICD9 = ICD9|333.0
MedlinePlus =
eMedicineSubj =
eMedicineTopic =
MeshID = D012791

Shy-Drager syndrome is a rare, progressively degenerative disease of the autonomic nervous system. It is named after Dr Milton Shy and Dr Glenn Drager, who identified this syndrome in 1960. [WhoNamedIt|synd|875] [cite journal |author=Shy GM, Drager GA |title=A neurological syndrome associated with orthostatic hypotension: a clinical-pathologic study |journal=Arch. Neurol. |volume=2 |issue= |pages=511–27 |year=1960 |pmid=14446364 |doi=]

The American Autonomic Society and the American Academy of Neurology deprecated it as a disease entity in 1996, [cite journal |author= | title=Consensus statement on the definition of orthostatic hypotension, pure autonomic failure, and multiple system atrophy |journal=Neurology |volume=46 |issue=5 |pages=1470 |year=1996 |pmid=8628505 |doi=] and existing cases were redefined as multiple system atrophy with autonomic phenomena. The name "Shy-Drager syndrome" is still used occasionally for multiple system atrophy when the primary symptoms are autonomic failure.


*Problems with the autonomic nervous system, such as dysphagia, incontinence, hypotension (specifically orthostatic hypotension), gastroparesis, and erectile dysfunction, among others (see autonomic neuropathy).
*Parkinsonian symptoms
*Other nonspecific neurologic symptoms such as ataxia and pyramidal symptomsFact|date=December 2007


No definitive treatment currently exists. Medications used for Parkinson's disease have not shown to be useful. Treatments are generally geared to give symptomatic control, especially to maintain adequate blood pressure (adrenergic agonists, and mineralocorticoid analogs such as fludrocortisone).Fact|date=December 2007

Notable sufferers

* Johnny Cash (possible misdiagnosis of autonomic neuropathy) Fact|date=January 2008


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