This rare neurological disorder affects the body's involuntary processes such as movement and blood pressure.
Multiple system atrophy (MSA) is a rare, degenerative neurological disorder affecting your body's involuntary (autonomic) functions, including blood pressure, breathing, bladder function and motor control.
Formerly called Shy-Drager syndrome, olivopontocerebellar atrophy or striatonigral degeneration, 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 (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.
This is the most common type of MSA. The signs and symptoms are similar to those of Parkinson's disease, such as:
The main signs and symptoms are problems with muscle coordination (ataxia), but others may include:
In addition, the primary sign of multiple system atrophy is:
You can also develop dangerously high blood pressure levels while lying down (supine hypertension).
MSA might cause other difficulties with involuntary (autonomic) body functions, including:
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.
There's no known cause for multiple system atrophy (MSA). Some researchers are studying a possible inherited component or involvement of an environmental toxin 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.
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:
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.
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. Brain imaging scans, such as an MRI, can show signs that may suggest MSA and also help determine if there are other causes that may be contributing to your symptoms.
You may receive a referral to a neurologist or other specialist for specific evaluations that can help in making the diagnosis.
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 positioned at a 70-degree angle.
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.
Doctors may order other tests to assess your body's involuntary functions, including:
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.
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 (Orvaten) can raise your blood pressure quickly; however, it needs to be taken carefully as it can elevate pressure while lying down. You should not lie 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, others), 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.
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.
Doctors often advise using certain self-care measures to help minimize MSA symptoms, such as:
You may have your first appointment to discuss your symptoms with your family doctor, but you may be referred to a doctor who specializes in the diagnosis and treatment of conditions that affect the brain and nervous system (neurologist).
Here's some information to help you prepare for your appointment.
For multiple system atrophy, some basic questions to ask your doctor include:
Don't hesitate to ask any other questions that you have.
Your doctor is likely to ask you a number of questions, including:
While you wait for your appointment, find out if any blood relatives such as a parent, sibling, or grandparent have been diagnosed with neurological disorders such as Parkinson's disease or cerebellar ataxia. Multiple system atrophy (MSA) is not known to be an inherited condition, so a family history of a condition with similar symptoms may help your doctor rule out MSA.
June 7th, 2020