Shy-Drager syndrome

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Definition

Shy-Drager syndrome (SDS) is a rare condition that causes progressive damage to the autonomic nervous system. The autonomic nervous system controls vital involuntary body functions such as heart rate, breathing, and intestinal, urinary, and sexual functions. The autonomic nervous system also controls skin and body temperature, and how the body responds to stress. Shy-Drager syndrome leads to dizziness or fainting when standing up, urinary incontinence, impotence, and muscle tremors.

Description

SDS was named for neurologists Milton Shy, M.D., from the National Institutes of Health, and Glenn Drager, M.D., from the Baylor College of Medicine, who first described the condition in 1960. It typically affects those between ages 50-70. It affects more men than women. In severe cases, the person cannot even stand up. Symptoms can be mild as well. Sometimes, people with mild cases are misdiagnosed as having anxiety or hypertension.

Many nonprescription drugs, such as cold medicines and diet capsules, can trigger extremely high blood pressure spikes in patients with SDS, even in very low doses. Therefore, these patients are at risk for strokes and excessive bleeding (hemorrhage) if they take even the recommended dosage of these drugs.

— Carol A. Turkington

A progressive disorder of the brain and spinal cord affecting the autonomic nervous system and characterized by low blood pressure, atrophy of the iris, incontinence, the absence of sweating, impotence in males, tremor, and muscle wasting.

It has been suggested that this article or section be merged into Multiple system atrophy. (Discuss)

Shy-Drager syndrome
Classification and external resources
ICD-10	G90.3
ICD-9	333.0
DiseasesDB	12042
eMedicine	neuro/671
MeSH	D012791

Shy-Drager syndrome is the term formerly used for what is now considered a clinical variant of multiple system atrophy.

The syndrome was 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 Shy-Drager syndrome as a disease entity in 1996, and existing cases were redefined as multiple system atrophy with autonomic phenomena.^[3] The name "Shy-Drager syndrome" is still used occasionally for multiple system atrophy when the primary symptoms are autonomic failure.

Shy-Drager syndrome is characterized by parkinsonian features and characteristic autonomic insufficiency. Autonomic insufficiency is the primary cause of symptoms such as postural hypotension that can lead to syncopal episodes, anhidrosis (decreased or absent sweating), disturbance of sphincter control including loss of bladder control, and impotence. Signs of more widespread neurologic involvement such as lower motor neuron signs and cerebellar deficits may also be present.

There is no treatment at this point for Shy-Drager syndrome. However, postural hypotension can be treated with waist high stockings, fludrocortisone, midodrine or increasing salt in the diet. ^[4]

Main Article

See also: multiple system atrophy

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. ^ "Consensus statement on the definition of orthostatic hypotension, pure autonomic failure, and multiple system atrophy". Neurology 46 (5): 1470. 1996. PMID 8628505. 
4. ^ "Aminoff MJ, Greenberg DA, Simon RP, "Chapter 7. Movement Disorders" (Chapter). Aminoff MJ, Greenberg DA, Simon RP: Clinical Neurology, 6th Edition: http://www.accessmedicine.com/content.aspx?aID=2082488.". http://www.accessmedicine.com/content.aspx?aID=2082488. Retrieved on 04/20/2009. 

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