Shy–Drager syndrome

Shy–Drager syndrome
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Synonyms	N/A
Pronounce	N/A
Specialty	N/A
Symptoms	Orthostatic hypotension, urinary incontinence, erectile dysfunction, muscle rigidity, tremor
Complications	Parkinsonism, autonomic dysfunction
Onset	Middle age
Duration	Progressive
Types	N/A
Causes	Unknown
Risks
Diagnosis	Clinical evaluation, MRI
Differential diagnosis	N/A
Prevention	N/A
Treatment	Symptomatic management
Medication	N/A
Prognosis	Poor
Frequency	Rare
Deaths	N/A

Shy–Drager syndrome is a rare neurodegenerative disorder that is now considered a form of multiple system atrophy (MSA). It is characterized by a combination of parkinsonism, autonomic dysfunction, and cerebellar ataxia. The syndrome was first described by Dr. Milton Shy and Dr. Glen Drager in 1960.

Clinical Features

Patients with Shy–Drager syndrome typically present with a variety of symptoms due to the degeneration of multiple systems in the body. The most prominent features include:

• Autonomic Dysfunction: This includes severe orthostatic hypotension, which is a significant drop in blood pressure upon standing, leading to dizziness or fainting. Other autonomic symptoms include urinary incontinence, erectile dysfunction, and sweating abnormalities.

• Parkinsonism: Patients may exhibit symptoms similar to Parkinson's disease, such as muscle rigidity, tremor, and bradykinesia (slowness of movement).

• Cerebellar Ataxia: Some patients may experience problems with coordination and balance, leading to a wide-based, unsteady gait.

Pathophysiology

The exact cause of Shy–Drager syndrome is unknown, but it involves the progressive degeneration of nerve cells in specific areas of the brain, including the basal ganglia, cerebellum, and autonomic nervous system. This degeneration leads to the characteristic symptoms of the disorder.

Diagnosis

Diagnosis of Shy–Drager syndrome is primarily clinical, based on the presence of the characteristic symptoms. Magnetic resonance imaging (MRI) may be used to rule out other conditions and to look for changes in the brain that are consistent with multiple system atrophy.

Treatment

There is no cure for Shy–Drager syndrome, and treatment is focused on managing symptoms. This may include:

• Medications: To manage blood pressure, such as fludrocortisone or midodrine.
• Physical Therapy: To help with mobility and balance.
• Lifestyle Modifications: Such as increasing salt and fluid intake to help with blood pressure.

Prognosis

The prognosis for individuals with Shy–Drager syndrome is generally poor, as the condition is progressive and can lead to severe disability. The average life expectancy after diagnosis is approximately 7 to 10 years.

History

Shy–Drager syndrome was first described in 1960 by Dr. Milton Shy and Dr. Glen Drager, who identified the combination of parkinsonism and autonomic dysfunction in a series of patients. It was later recognized as a form of multiple system atrophy.

Also see

• Multiple system atrophy
• Parkinson's disease
• Autonomic nervous system
• Orthostatic hypotension

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WikiMD neurology

External links

• Comprehensive information from the National Institute of health.
  

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