Disease: Multiple system atrophy (MSA)

    Overview

    Multiple system atrophy (MSA) is a rare, degenerative neurological disorder affecting your body's involuntary (autonomic) functions, including blood pressure, breathing, bladder function and muscle control.

    Formerly called Shy-Drager syndrome, MSA shares many Parkinson's disease-like symptoms, such as slow movement, rigid muscles and poor balance.

    Treatment includes medications and lifestyle changes to help manage symptoms, but there is no cure. The condition progresses gradually and eventually leads to death.

    Multiple system atrophy care at Mayo Clinic

    Source: http://www.mayoclinic.com

    Symptoms

    Multiple system atrophy (MSA) affects many parts of your body. Symptoms typically develop in adulthood, usually in the 50s or 60s.

    MSA is classified by two types: parkinsonian and cerebellar. The type depends on the symptoms you have at diagnosis.

    Parkinsonian type

    This is the most common type of MSA. The signs and symptoms are similar to those of Parkinson's disease, such as:

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

    Cerebellar type

    The main signs and symptoms are problems with muscle coordination (ataxia), but others may include:

    • Impaired 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.

    MSA might cause other difficulties with involuntary (autonomic) body functions, including:

    Urinary and bowel dysfunction

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

    Sweating abnormalities

    • Reduced production of sweat, tears and saliva
    • Heat intolerance due to reduced sweating
    • Impaired body temperature control, often causing cold hands or feet

    Sleep disorders

    • Agitated sleep due to "acting out" 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, such as laughing or crying inappropriately

    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

    Causes

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

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

    Under a microscope, the damaged brain tissue of people with MSA shows 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

    Diagnosis

    Diagnosing multiple system atrophy (MSA) can be challenging. 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. The clinical examination, with various autonomic tests and imaging studies, can help your doctor determine whether the diagnosis is probable MSA or possible MSA.

    As a result, some people are never properly diagnosed. However, doctors are increasingly aware of the disease and more likely to use physical examination and autonomic tests to determine if MSA is the most likely cause of your 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 or shrinkage (atrophy) is 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

    This test can 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.

    During the test, your blood pressure and heart rate are monitored. The findings can document both the extent of blood pressure irregularities and whether they occur with a change in physical position.

    Tests to assess autonomic functions

    Doctors may order other tests to assess your body's involuntary functions, including:

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

    If you have sleep irregularities, especially interrupted breathing or snoring, your doctor may recommend an evaluation in a sleep laboratory. This can help diagnose an underlying and treatable sleep disorder, such as sleep apnea.

    Source: http://www.mayoclinic.com

    Complications

    The progression of MSA varies, but the condition does not go into remission. As the disorder progresses, daily activities become increasingly difficult.

    Possible complications include:

    • Breathing abnormalities during sleep
    • Injuries from falls caused by poor balance 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
    • Vocal cord paralysis, which makes speech and breathing difficult
    • Increased difficulty swallowing

    People typically live about seven to 10 years after multiple system atrophy symptoms first appear. However, the survival rate with MSA varies widely. Occasionally, people can live for 15 years or longer with the disease. Death is often due to respiratory problems.

    Source: http://www.mayoclinic.com

    Lifestyle and home remedies

    Doctors often advise using certain self-care measures to help minimize MSA symptoms, 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 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. Eat small, low-carbohydrate 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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