Disease: Multiple system atrophy (MSA)

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Multiple system atrophy (MSA) is a rare neurological disorder that impairs your body's involuntary (autonomic) functions, including blood pressure, heart rate, bladder function and digestion.

Formerly called Shy-Drager syndrome, the condition shares many Parkinson's disease-like symptoms, such as slowness of movement, muscle rigidity and poor balance.

Multiple system atrophy is a degenerative disease that develops in adulthood, usually in the 50s or 60s.

Treatment for MSA includes medications and lifestyle changes to help manage symptoms. The condition progresses gradually and eventually leads to death.

Source: http://www.mayoclinic.com

Multiple system atrophy (MSA) is so named because its signs and symptoms affect multiple parts of your body. Previously called Shy-Drager syndrome, MSA is classified by two types: parkinsonian and cerebellar, depending on which types of symptoms predominate at the time of evaluation.

Parkinsonian type

Predominant signs and symptoms are those of Parkinson's disease, such as:

  • Rigid muscles and difficulty bending your arms and legs
  • Slow movement (bradykinesia)
  • Tremors (rare in MSA compared with classic Parkinson's disease)
  • Impaired posture and balance

Cerebellar type

Predominant signs and symptoms are lack of muscle coordination (ataxia). Signs and symptoms may include:

  • Impairment of movement and coordination, such as unsteady gait and loss of balance
  • Slurred, slow or low-volume speech (dysarthria)
  • Visual disturbances, such as blurred or double vision and difficulty focusing your eyes
  • Difficulty swallowing (dysphagia) or chewing

General signs and symptoms

In addition, the primary sign of multiple system atrophy is:

  • Postural (orthostatic) hypotension, a form of low blood pressure that makes you feel dizzy or lightheaded, or even faint, when you stand up from sitting or lying down.

You also can develop dangerously high blood pressure levels while lying down.

People with multiple system atrophy may have other difficulties with body functions that occur involuntarily (autonomic), including:

Urinary and bowel dysfunction

  • Constipation
  • Loss of bladder or bowel control (incontinence)

Sweating abnormalities

  • A reduction in the production of perspiration, tears and saliva
  • Impaired control of body temperature, often causing cold hands or feet as well as heat intolerance due to impaired sweating

Sleep disorders

  • Agitated sleep due to "acting out" one's dreams
  • Abnormal breathing at night

Sexual dysfunction

  • Inability to achieve or maintain an erection (impotence)
  • Loss of libido

Cardiovascular problems

  • Irregular heartbeat

Psychiatric problems

  • Difficulty controlling emotions

When to see a doctor

If you develop any of the signs and symptoms associated with multiple system atrophy, see your doctor for an evaluation and diagnosis. If you've already been diagnosed with the condition, contact your doctor if new symptoms occur or if existing symptoms worsen.

Source: http://www.mayoclinic.com

There's no known cause for brain changes in multiple system atrophy (MSA). Some researchers are studying whether there's an inherited component or environmental toxin involved in the disease process, but there's no substantial evidence to support these theories.

MSA is associated with deterioration and shrinkage (atrophy) of portions of your brain (cerebellum, basal ganglia and brainstem) that regulate internal body functions, digestion and motor control.

Evaluation under a microscope of damaged brain tissue of people with MSA reveals nerve cells (neurons) that contain an abnormal amount of a protein called alpha-synuclein. Some research suggests that this protein may be overexpressed in multiple system atrophy.

Source: http://www.mayoclinic.com

Appointments & care

At Mayo Clinic, we take the time to listen, to find answers and to provide you the best care.

Diagnosis of multiple system atrophy (MSA) can be challenging because there's no test that can make or confirm the diagnosis. At the same time, certain signs and symptoms of MSA — such as muscle rigidity and unsteady gait — also occur with other disorders, such as Parkinson's disease, making the diagnosis more difficult.

As a result, some people are never properly diagnosed, although doctors are increasingly aware of the disease and, thus, more likely to identify its symptoms.

If your doctor suspects multiple system atrophy, he or she will obtain a medical history, perform a physical examination, and possibly order blood tests and brain-imaging scans, such as an MRI, to determine whether brain lesions are present that may be triggering symptoms.

You may receive a referral to a neurologist or other specialist for specific evaluations that can help in making the diagnosis.

Tilt table test

One procedure that can aid in the diagnostic process is a tilt table test to help determine if you have a problem with blood pressure control. In this procedure, you're placed on a motorized table and strapped in place. Then the table is tilted upward so that your body is nearly vertical.

Throughout this maneuver, your blood pressure and heart rate are monitored. The findings can document not only the extent of blood pressure irregularities but also whether they occur with a change in physical position.

Tests to assess autonomic functions

Doctors may order certain other tests, to determine how your body is functioning. Tests to assess your body's involuntary functions may include:

  • Blood pressure measurement, lying down and standing
  • A sweat test to evaluate perspiration
  • Eye exam
  • Tests to assess your bladder and bowel function
  • Electrocardiogram to track the electrical signals of your heart
  • Nerve and muscular examination

If you have sleep irregularities, particularly if they involve interrupted breathing or snoring, your doctor may recommend having you evaluated in a sleep laboratory to determine if you have an underlying and treatable sleep disorder, such as sleep apnea.

Source: http://www.mayoclinic.com

The rate of progression of multiple system atrophy varies from person to person, but the condition does not go into remission. As the disorder progresses, daily activities become increasingly difficult.

You may experience the following complications:

  • Breathing abnormalities during sleep
  • Injuries from falls associated with impaired walking or fainting
  • Progressive immobility that can lead to secondary problems such as a breakdown of your skin
  • Loss of ability to care for yourself in day-to-day activities (from bathing to brushing teeth)
  • Vocal cord paralysis, which makes speech and breathing difficult
  • Increased difficulty swallowing

People typically live about seven to nine years after multiple system atrophy symptoms first appear. Ten-year survival is rare. Death is often due to respiratory problems.

Source: http://www.mayoclinic.com

Doctors often advise using certain self-care measures to help minimize symptoms associated with multiple system atrophy, such as:

  • Take steps to raise your blood pressure. Add a little salt to the diet and drink more fluids. Salt and fluids can increase blood volume and thus raise your blood pressure. Drink coffee and other caffeinated fluids to raise your blood pressure.
  • Elevate the head of your bed. Raising the head of your bed to about a 30-degree angle will minimize increases in blood pressure when you sleep. Get up slowly from a reclining position.
  • Make dietary changes. Add more fiber to your diet in order to ease constipation. You may also benefit from over-the-counter laxatives. Avoid alcohol, and consume small meals.
  • Avoid getting too hot. Stay in air-conditioned rooms on very hot days. Avoid excessive amounts of heat in the bathroom when bathing.
  • Wear elastic support stockings up to your waist. This can help keep your blood pressure from dropping.

Source: http://www.mayoclinic.com

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