Shy-Drager syndrome

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.^[1][2]

The American Autonomic Society and the American Academy of Neurology deprecated it as a disease entity in 1996,^[3] 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.

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Symptoms

• Problems with the autonomic nervous system, such as incontinence, hypotension, gastroparesis, and erectile dysfunction, among others (see autonomic neuropathy).
• Parkinsonian symptoms
• Other nonspecific neurologic symptoms such as ataxia and pyramidal symptoms^{[citation needed]}

Treatment

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).^{[citation needed]}

Notable sufferers

• Johnny Cash (possible misdiagnosis of autonomic neuropathy)^{[citation needed]}

References

1. ^ synd/875 at Who Named It
2. ^ Shy GM, Drager GA (1960). "A neurological syndrome associated with orthostatic hypotension: a clinical-pathologic study". Arch. Neurol. 2: 511–27. PMID 14446364.
3. ^ (1996) "Consensus statement on the definition of orthostatic hypotension, pure autonomic failure, and multiple system atrophy". Neurology 46 (5): 1470. PMID 8628505.