Multiple System Atrophy – Causes, Signs and symptoms

Multiple System Atrophy

Also called: Multi System Atrophy, MSA

Summary

Multiple system atrophy (MSA) is a neurological disorder in which patients experience degenerative symptoms that can affect movement, speech, blood pressure and other bodily functions.

There are three types of MSA:

  • Striatonigral degeneration (SND). Causes symptoms of parkinsonism, such as rigidity, slowed movements and tremors.

  • Olivopontocerebellar atrophy (OPCA). Mostly affects muscle coordination and speech. Symptoms include a loss of balance, loss of coordination of the arms and legs, and slurred speech.

  • MSA with orthostatic hypotension (formerly called Shy-Drager syndrome). Mostly affects the autonomic nervous system, which regulates involuntary body functions such as blood pressure and heart rate. Orthostatic hypotension (sudden drop in blood pressure with changing positions, such as upon standing) is common with this type of MSA.  

Symptoms can vary from person to person, as well as differ depending on the MSA type involved. Symptoms may also appear in a wide variety of combinations. MSA symptoms tend to occur in older adults and gradually get worse over a number of years. Although statistics vary, life expectancy after diagnosis of MSA is about 10 years.

The prevalence of MSA is unclear, although it is considered to be a relatively rare disorder. According to The Parkinson’s Institute, there are an estimated 25,000 to 100,000 people in the United States who have MSA. The cause of MSA remains unknown. Men aged 50 and older appear to have an increased risk of developing the disease.

There is no test that can definitively diagnose MSA. In order to diagnose MSA, a physician will likely compile a thorough medical history of the patient and perform a physical examination and neurological examination. The patient’s symptoms and the duration and severity of the symptoms will be discussed. In addition, certain other tests may help confirm a suspected diagnosis. This includes a tilt-table test, electromyography, blood tests, urine tests and imaging tests.

There is no known cure or way to prevent MSA. There is no specific therapy that can slow the progression of the disease. Treatment efforts are focused on relieving symptoms and enhancing quality of life. Medications, physical therapy and certain self-care measures are among the treatments used for symptoms of MSA.

About multiple system atrophy

Multiple system atrophy (MSA) is a neurological disorder in which patients experience degenerative symptoms that can affect movement, speech, blood pressure and other bodily functions.

MSA affects the central nervous system (CNS) and the autonomic nervous system (ANS). The CNS includes the brain and spinal cord. It coordinates the body’s responses to sensory impulses received from the peripheral nervous system (PNS). The ANS is a part of the PNS that controls involuntary functions of the body, such as blood pressure, heart rate, digestion, sweating, sexual function, and bowel and bladder control.

MSA is characterized by symptoms of parkinsonism (e.g., slow movements, rigidity and tremors), ataxia (loss of muscle coordination) and autonomic dysfunction (e.g., drop in blood pressure, breathing difficulties). These symptoms may occur in a variety of combinations. The type of MSA a person has may be determined by the predominant symptoms – that is, whether symptoms are primarily those of parkinsonism, ataxia or autonomic dysfunction.

The prevalence of MSA is unclear, although it is considered to be a relatively rare disorder. According to The Parkinson’s Institute, there are an estimated 25,000 to 100,000 people in the United States who have MSA.

MSA occurs in adults and symptoms get progressively worse over a number of years. As the disease progresses, patients are likely to experience more widespread and severe symptoms. In some cases, dementia may develop during later stages of the disease. Complications of MSA may include injury from falls (due to orthostatic hypotension or impaired balance), breathing problems and cardiac arrhythmias (irregular heart rhythms).

The speed with which MSA progresses can vary from person to person, and may differ depending on the type of MSA involved. For example, according to the National Institute of Neurological Disorders and Stroke (NINDS):

  • Patients with predominant parkinsonism (striatonigral degeneration) may have a normal life expectancy.

  • Patients with predominant ataxia symptoms (olivopontocerebellar atrophy) may experience symptoms that progress slowly, with death occurring about 20 years after symptom onset.

  • Patients with autonomic dysfunction as their primary symptom (MSA with orthostatic hypotension) usually die within 10 years after onset of symptoms.

Although statistics vary, the average life expectancy after diagnosis with MSA appears to be approximately 10 years.

Types and differences of MSA

There are three major types of multiple system atrophy (MSA). They affect different areas of the body and can produce different symptoms. The three MSA types are:

  • Striatonigral degeneration (SND). Degeneration that affects the corpus striatum and substantia nigra in the brain, both of which are part of the basal ganglia – which is involved in motion control and memory. SND produces symptoms similar to those of Parkinson’s disease, including slow movements, stiff muscles, rigidity and tremors. This type of MSA is sometimes also referred to as the Parkinsonian type.

  • Olivopontocerebellar atrophy (OPCA). Degeneration that affects three areas of the brain: the lower pair of olives (oval structures in the medulla oblongata), the pons and the cerebellum – all of which work together to control movement. Thus, the predominant symptoms of OPCA are a lack of balance and inability to coordinate movement and speech. There are many different types of OPCA, including several hereditary forms. However, the hereditary forms are not considered a type of MSA, according to the National Organization for Rare Disorders. The severity of OPCA symptoms and age of symptom onset may vary significantly, depending on the type of OPCA involved. OPCA is also sometimes referred to as the cerebellar type.
  • Multiple system atrophy with orthostatic hypotension (formerly called Shy-Drager syndrome). As its contemporary name indicates, orthostatic hypotension (sudden drop in blood pressure upon standing) is the predominant symptom of this type of MSA. This type of MSA affects the autonomic nervous system, which regulates involuntary body functions (e.g., blood pressure, heart rate, sweating, erectile function, bowel and bladder control). This MSA is sometimes referred to as the combined type, since it may also include symptoms typically associated with OPCA and/or SND.

Risk factors and causes of multiple system atrophy

The cause of multiple system atrophy (MSA) remains unknown.

It is not exactly clear what factors increase a person’s risk of developing MSA, although the following appear to play a role:

  • Age. Adults age 50 and older appear to have a higher risk of developing MSA than children and younger adults.

  • Gender. Men appear to develop MSA more often than women. Twice as many men are diagnosed with MSA as women, according to the National Dysautonomia Research Foundation.

Signs and symptoms of multiple system atrophy

Signs and symptoms of multiple system atrophy (MSA) vary, depending on the MSA type involved. In addition, even within specific MSA types, symptoms may vary from person to person. Symptoms of MSA can appear in a wide variety of combinations.

Signs and symptoms of striatonigral degeneration (SND) resemble that of Parkinson’s disease and may include:

  • Slowed movements
  • Rigidity
  • Shuffling gait
  • Impaired balance and speech
  • Deterioration in fine motor skills
  • Tremors

Signs and symptoms of olivopontocerebellar atrophy (OPCA) may include:

  • Loss of balance and muscle coordination (ataxia) of legs and arms
  • Speech difficulties (e.g., slurred speech)
  • Muscle spasms
  • Weakness or stiffness of muscles
  • Numbness or tingling in the hands and feet
  • Abnormal eye movements
  • Tremors in the hands or arms
  • Cognitive disorders, including memory loss
  • Impaired ability to pay attention
  • Bowel- or bladder-control problems
  • Fatigue
  • Trouble sleeping

Multiple system atrophy with orthostatic hypotension (formerly known as Shy-Drager syndrome) usually causes orthostatic hypotension. This is a sudden and excessive drop in blood pressure when a patient changes position, particularly when going from a lying or seated position to standing. Orthostatic hypotension can result in dizziness or fainting that may cause patients to fall and possibly injure themselves. This type of MSA is also sometimes referred to as the combination type because it may include symptoms typically seen with OPCA or SND. Additional symptoms of this type of MSA may include:

  • Urinary incontinence
  • Constipation
  • Erectile dysfunction (in men)
  • Generalized weakness
  • Vision problems (e.g., double vision)
  • Decreased ability to sweat
  • Difficulty breathing or swallowing

Diagnosis methods for multiple system atrophy

To diagnose multiple system atrophy (MSA), a physician will begin by compiling a thorough medical history of the patient and performing a complete physical examination. The patient’s symptoms, their duration and severity will be discussed. A neurological examination also may be performed.

There are no specific tests that can definitively determine whether a patient has MSA. However, certain diagnostic tests may be performed in cases of suspected MSA. These include:

  • Tilt-table test. A patient lies on a table that is gradually raised, while heart rate and blood pressure are monitored. This test can identify whether a patient experiences orthostatic hypotension. If a patient faints or temporarily loses consciousness during this test, it may indicate an illness affecting the autonomic nervous system (e.g., MSA, Parkinson’s disease, diabetes).

  • Electromyography (EMG). A test in which needles are inserted into certain muscles to test the electrical activity the muscles generate. EMGs may be helpful in confirming motor nerve damage in patients with MSA.

Urine tests, blood tests and imaging tests such as magnetic resonance imaging (MRI) also can be helpful in diagnosing MSA.

MSA is extremely difficult to diagnose because its symptoms can be similar to other diseases (e.g., Parkinson’s disease, neuropathy, supranuclear palsy). In many cases, patients with MSA may be wrongfully diagnosed as having Parkinson’s disease. In order to differentiate between the two conditions, a physician may prescribe certain medications (e.g., levodopa) known to alleviate symptoms in patients with Parkinson’s disease, but not those with MSA. If a patient’s parkinsonism symptoms fail to respond to levodopa, it may suggest MSA. Also, imaging tests that identify damage in specific areas of the brain can suggest MSA rather than another condition.

Treatment and prevention of MSA

There is no known cure or way to prevent multiple system atrophy (MSA). There is no specific therapy known to slow the progression of the disease. Rather, treatment is focused on relieving a patient’s symptoms and enhancing a patient’s quality of life.

Various medications are sometimes used to help relieve the symptoms of MSA. Parkinson’s disease medications such as levodopa (a dopamine precursor) and dopamine agonists may help relieve some of the slowness and rigidity associated with MSA. Muscle relaxant medications are sometimes used to treat muscle spasms associated with MSA. Beta blockers may be used to control tremors.

Medications may also be used to help raise the blood pressure of patients with MSA who are experiencing orthostatic hypotension (a significant drop in blood pressure with change in position, such as upon standing). These medications may include vasoconstrictors (drugs that narrow blood vessels, temporarily increasing blood pressure). They may also include medications that help the body retain fluid, raising blood pressure. In some cases, a pacemaker may be surgically implanted to increase a patient’s blood pressure.

Patients are encouraged to consult their physician about which medications and treatment options are appropriate for them.

Certain medications (e.g., Beta blockers) may treat some MSA symptoms (e.g., tremors) but exacerbate others (e.g., low blood pressure). Certain medications may have dangerous side effects (e.g., raising a patient’s blood pressure to dangerous levels when lying down). In addition, the type of MSA a patient has may affect the type of medication chosen. For example, patients with striatonigral degeneration, which involves symptoms similar to those of Parkinson’s disease, may discover their symptoms do not respond to drugs typically used to treat Parkinson’s symptoms (e.g., levodopa).  

Other symptoms of MSA (e.g., impotence, incontinence, constipation) also may be treated with medications.

Self-care methods may help alleviate some symptoms of MSA, such as orthostatic hypotension. Patients who experience a sudden decrease in blood pressure upon standing can wear tight support garments (e.g., pressure stockings) while standing. This improves the flow of blood back to the heart and reduces the risk that blood pressure will drop upon standing. In addition, patients can do the following to reduce the risk of orthostatic hypotension:

  • Sleep at a slight incline (head up)

  • Stretch, contract and release muscles in the legs before standing

  • Consume plenty of fluids daily

  • Eat small, frequent meals

  • Increase salt intake (with a physician’s approval)

  • Avoid hot weather, alcohol and dehydration (which can cause blood pressure to drop)

Patients with MSA may benefit from therapy with a speech language pathologist who can help patients improve their ability to swallow or speak. Occupational therapy may help patients regain fine motor skills. A physical therapist can help patients improve muscle tone and strength. Patients may also benefit from psychological counseling to help deal with the stress of having a degenerative disease.

Patients with MSA may eventually require certain devices to assist with movement (e.g., wheelchair). If a patient’s ability to swallow or breath becomes affected, artificial feeding tubes or breathing tubes may become necessary. Arranging for home health care or placement in a healthcare facility may be necessary.

Questions for your doctor regarding MSA

Preparing questions in advance can help patients to have more meaningful discussions with healthcare professionals regarding their condition. Patients may wish to ask their doctor the following questions related to multiple system atrophy (MSA):

  1. What symptoms may indicate I have MSA?
  2. How will you diagnose my MSA?
  3. How do you know that I have MSA and not another illness, such as Parkinson’s disease?
  4. What type of MSA do I have?
  5. How fast is my disease likely to progress?
  6. What are my treatment options?
  7. What are the benefits and risks of these treatments?
  8. Are there medications or other therapies that might help me?
  9. What symptoms am I likely to experience over time?
  10. What signs or symptoms may indicate my MSA is worsening? What should I do if these occur?
  11. What is my long-term prognosis?
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