MSA Basics

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.

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.

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

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

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 

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.

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 6 to 9 years after multiple system atrophy symptoms first appear. However, the survival rate with MSA varies widely. Occasionally, people can live for 10 to 12 years or longer with the disease. Death is often due to respiratory problems.

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.

Treatment

There's no cure for multiple system atrophy. Managing the disease involves treating signs and symptoms to make you as comfortable as possible and to maintain your body functions.

To treat specific signs and symptoms, your doctor may recommend:

·         Medications to raise blood pressure. The corticosteroid fludrocortisone and other medications can increase your blood pressure by helping your body retain more salt and water.

The drug pyridostigmine (Mestinon) can raise your standing blood pressure without increasing your blood pressure while you're lying down.

Midodrine can raise your blood pressure quickly; however, it needs to be taken carefully as it can elevate pressure while lying down so people should not lay flat for four hours after taking the medication.

The FDA has approved droxidopa (Northera) for treating orthostatic hypotension. The most common side effects of droxidopa include headache, dizziness and nausea.

·         Medications to reduce Parkinson's disease-like signs and symptoms. Certain medications used to treat Parkinson's disease, such as combined levodopa and carbidopa (Duopa, Sinemet), can be used to reduce Parkinson's disease-like signs and symptoms, such as stiffness, balance problems and slowness of movement. These medications can also improve overall well-being.

However, not everyone with multiple system atrophy responds to Parkinson's drugs. They may also become less effective after a few years.

·         Pacemaker. Your doctor may advise implanting a heart pacemaker to keep your heart beating at a rapid pace, which can increase your blood pressure.

·         Impotence drugs. Impotence can be treated with a variety of drugs, such as sildenafil (Revatio, Viagra), designed to manage erectile dysfunction.

·         Steps to manage swallowing and breathing difficulties. If you have difficulty swallowing, try eating softer foods. If swallowing or breathing becomes increasingly problematic, you may need a surgically inserted feeding or breathing tube. In advanced MSA, you may require a tube (gastrostomy tube) that delivers food directly into your stomach.

·         Bladder care. If you're experiencing bladder control problems, medications can help in the earlier stages. Eventually, when the disease becomes advanced, you may need to have a soft tube (catheter) inserted permanently to allow you to drain your bladder.

·         Therapy. A physical therapist can help you maintain as much of your motor and muscle capacity as possible as the disorder progresses.

A speech-language pathologist can help you improve or maintain your speech.