Shy-Drager Syndrome
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Shy-Drager Syndrome

(Multiple System Atrophy With Postural Hypotension; Striatonigral Degeneration)

En Español (Spanish Version)

Definition

Shy-Drager syndrome is a degenerative disorder of the nervous system. Multiple areas of the nervous system can be affected, such as the parts that control motor and involuntary functions. The motor system controls movement and balance. The involuntary, or autonomic, nervous system controls body functions (for example, blood pressure, bowel and bladder function). Shy-Drager syndrome may cause postural hypotension. Postural hypotension is a drop in blood pressure after changing position that can result in dizziness or fainting.

Nervous System

NC3D00004_96472_1_Anatomy of the Nervous System.jpg

© 2008 Nucleus Medical Art, Inc.

Causes

It is unknown what causes the degeneration or nerve loss in Shy-Drager syndrome.

Risk Factors

A risk factor is something that increases your chance of getting a disease or condition.

Risk factors for Shy-Drager syndrome include:

  • Age: middle age or older

Symptoms

Symptoms associated with the involuntary nerves usually occur first. Motor problems similar to those found with Parkinson’s disease may develop later. Symptoms include:

  • Dizziness or fainting when standing or sitting up, or changing positions
  • Dizziness may be worse in the morning or after meals
  • Fatigue after physical exertion
  • Muscle weakness
  • Neck ache, which may radiate to the shoulders
  • Constipation or diarrhea
  • Erectile dysfunction in men
  • Loss of sex drive
  • Inability to control bowels or bladder ( incontinence )
  • Urinary frequency and urgency that may be especially bothersome at night (occurs early in the disease)
  • Gasping or noisy breathing
  • Short stops in breathing during sleep ( sleep apnea )
  • Impaired temperature control
  • Reduced sweating
  • Dry skin
  • Vision problems
  • Abnormal eye movements
  • Slurred, faint speech
  • Trouble swallowing
  • Poor balance
  • Unsteady walking
  • Tremors
  • Stiffness
  • Slow movements
  • Loss of facial expression
  • Dementia (occurs late in the disease)

Diagnosis

Your doctor will ask about your symptoms and medical history, and perform a physical exam. This disease is often hard to diagnose. There is no specific test for Shy-Drager syndrome, but testing helps rule out other conditions.

Tests may include:

  • Blood tests—to measure electrolytes and other blood chemistries.
  • MRI scan—a test that uses magnetic waves to make pictures of the inside of the body.
  • Electromyogram (EMG)—a test that measures and records the electrical activity of the muscles at rest and when contracted. It can check for skeletal muscle weakness.
  • Tilt-table test—measures changes in blood pressure as your body position changes
  • Sleep studies—to check for disorders such as sleep apnea.
  • Barium swallow—a series of x-rays of the upper digestive tract taken after you swallow a barium substance, which will show up on x-ray. In people with Shy-Drager syndrome, this test is used to assess swallowing problems.

Treatment

No cure exists for Shy-Drager syndrome. Doctors are often able to help you manage the blood pressure problem. But no treatment exists to stop the decline in nerve function. Therapies aim to control symptoms.

Treatment may include:

Medication

Options may include:

  • Drugs to prevent drops in blood pressure, including:
    • Fludrocortisone (Florinef)
    • Indomethacin (Indocid)
    • Midodrine (ProAmatine)
    • Beta blockers such as:
      • Propranolol (Inderal)
      • Pindolol (Sectral)
  • Drugs that treat symptoms of Parkinson's disease such as carbidopa/levodpa.
  • Laxatives or stool softeners to treat constipation
  • Drugs to treat urinary symptoms, including oxybutynin, tolterodine, or propantheline—However, these can cause worsening of the constipitation.
  • Drugs to treat impotence, such as yohimbine or sildenafil—Sildenafil must be used with care as it can worsen the low blood pressure that occurs with the disease, causing dizziness and fainting.

Self-care

Diet and exercise guidelines include:

  • Increase salt and fluid to help keep blood pressure up when standing.
    • One recent study demonstrated that drinking about 12 ounces of tap water once daily in the early morning can decrease otherwise difficult to treat low blood pressure in multiple system atrophy.
  • Drink beverages containing caffeine after meals.
  • Eat small, frequent meals.
  • Eat soft foods, which may be easier to swallow.
  • Increase fiber to help with constipation. Avoid straining when going to the bathroom.
  • Avoid drinking alcoholic beverages.
  • Exercise only moderately. Avoid vigorous exercise.

Lifestyle modifications include:

  • Sleep with the head of your bed raised to help prevent drops in blood pressure when arising.
  • Get up slowly and change positions slowly.
  • Wear elastic support stockings and abdominal binders or a body suit to help maintain blood pressure.
  • Avoid too much heat in your home (including when bathing) or environment.

To help you manage specific symptoms and side effects:

  • Try speech therapy for help dealing with swallowing and speech problems.
  • If swallowing is a problem, ask your doctor about tube feedings.
  • Join a support group for patients and/or family members.
  • Ask your doctor about a penile implant and other treatments for impotence.
  • Ask your doctor about intermittent catheterization for urinary problems.
  • Ask your doctor before taking any drugs. Many patients with Shy-Drager syndrome are very susceptible to drug side effects even at low doses.

Breathing Support

If you have severe breathing problems that cannot be relieved in other ways, your doctor may advise that you have a tracheostomy. This is a surgically-created hole in the windpipe, usually a treatment of last resort.

Prevention

There are no guidelines for preventing Shy-Drager syndrome because the cause is unknown.

RESOURCES:

National Dysautonomia Research Foundation
http://www.ndrf.org

We Move (Worldwide Education and Awareness for Movement Disorders)
http://www.wemove.org

CANADIAN RESOURCES:

Parkinson Society Canada
http://www.parkinson.ca/home.html

The Canadian Brain and Nerve Health Coalition
http://www.ccns.org/ccns_information/brain_nerve.html

References:

Campbell's Urology . 7th ed. WB Saunders Co; 1998.

Cecil Textbook of Medicine . 21st ed. WB Saunders Co; 2000.

Deguchi K, Ikeda K, Sasaki I, et al. Effects of daily water drinking on orthostatic and postprandial hypotension in patients with multiple system atrophy. J Neurol. 2007;254:735-740

Golbe LI. Multiple System Atropy. In: Gilman S, ed. MedLink Neurology website. Available at: http://www.medlink.com . Accessed February 23, 2008.

Multiple system atrophy. Medical Clinics of North America . 1999.

National Institute of Neurological Disorders and Stroke website. Available at: http://www.ninds.nih.gov/ .

Practice of Geriatrics . 3rd ed. WB Saunders Co; 1998.

Textbook of Clinical Neurology . WB Saunders Co; 1999.



Last reviewed February 2008 by J. Thomas Megerian, MD, PhD, FAAP

Please be aware that this information is provided to supplement the care provided by your physician. It is neither intended nor implied to be a substitute for professional medical advice. CALL YOUR HEALTHCARE PROVIDER IMMEDIATELY IF YOU THINK YOU MAY HAVE A MEDICAL EMERGENCY. Always seek the advice of your physician or other qualified health provider prior to starting any new treatment or with any questions you may have regarding a medical condition.

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