Multiple system atrophy

Conditions

Multiple system atrophy (MSA) is a progressive disease that causes symptoms to worsen over time. It usually starts with problems with walking and balance, followed by muscle weakness and stiffness, difficulty swallowing, and trouble speaking. Eventually, people may lose their ability to move their arms and legs, and eventually become unable to breathe on their own.

Multiple system atrophy (MSA) is a rare neurodegenerative disorder that shares many symptoms with Parkinson’s disease, such as slow movements, stiff muscles, and poor balance.

Treatment includes medication and lifestyle changes to help manage symptoms, but there is no cure. The disease progresses until it causes death.

Symptoms of multiple system atrophy

SMA affects many parts of your body. Symptoms usually develop in adulthood, usually between the ages of 50 and 60.

SMA is classified into two types: parkinsonian and cerebellar. The type depends on the symptoms you have at the time of diagnosis.

Parkinsonian type

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

  • Rigid muscles
  • Difficulty bending arms and legs
  • Slow movement (bradykinesia)
  • Tremors (rare in SMA)
  • Posture and balance issues

Cerebellar type

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

  • Impaired movement and coordination, such as an unsteady gait and loss of balance
  • Blurred, slow speech (dysarthria)
  • Visual disturbances, such as blurred or double vision and difficulty fixing your eyes on something.
  • Difficulty swallowing (dysphagia) or chewing

General signs and symptoms

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

  • Orthostatic hypotension, a form of low blood pressure that causes dizziness, or even fainting, when rising from a sitting or lying position

You may also have dangerously high blood pressure levels when lying down.

Multiple system atrophy can cause other difficulties with involuntary (autonomic) bodily functions, including:

Urinary and intestinal dysfunction

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

sweating abnormalities

  • Reduced production of sweat, tears and saliva
  • Heat intolerance due to reduced sweating
  • Loss of body temperature control, resulting in cold hands or feet

sleep disorders

  • Agitated sleep
  • Abnormal nocturnal breathing

Sexual dysfunction

  • Inability to get or keep an erection (impotence)
  • Loss of libido

Cardiovascular problems

  • Blood accumulates in the hands and feet and causes color changes.
  • Cold hands and feet

Psychiatric problems

  • Difficulty controlling emotions, such as laughing or crying inappropriately

Causes of multiple system atrophy

There is no known cause for multiple system atrophy (MSA). Some researchers are investigating the possible involvement of an environmental toxin in the disease process, but there is no substantial evidence to support these theories.

Multiple sclerosis causes deterioration and shrinkage (atrophy) of parts 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 SMA 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 SMA 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, which can lead to secondary problems such as deterioration of the condition of your skin
  • Loss of ability to take care of oneself in daily activities
  • Paralysis of the vocal cords, which makes it difficult to speak and breathe
  • Increased difficulty swallowing (dysphagia)

People typically live about seven to ten years after several symptoms of systemic atrophy appear. However, the survival rate with multiple system atrophy varies widely. Sometimes people can live for 15 years or more with the disease. Death is often due to respiratory problems.

How to Diagnose Multiple System Atrophy

Diagnosing multiple system atrophy can be difficult. Some signs and symptoms of SMA – such as muscle rigidity and unsteady gait – also occur with other disorders, such as Parkinson’s disease, making diagnosis more difficult. Physical examination, along with various tests and imaging exams, can help your doctor determine if the diagnosis of AMS is likely or possible.

As a result, some people are never correctly diagnosed. However, doctors are becoming more aware of the condition and are more likely to use physical examination and self-testing to determine if MSA is the most likely cause of your symptoms.

If your doctor suspects multiple system atrophy, they’ll take a medical history, perform a physical exam, and possibly order blood tests. Brain scans, such as an MRI, can show signs that may suggest SMA 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 make the diagnosis.

Tilt table test

This test can help determine if you have a blood pressure control problem. In this procedure, you are placed on a motorized table and strapped in place. Then the table is tilted upwards so that your body is positioned at a 70 degree angle.

During the test, your blood pressure and heart rate are monitored. The results can assess both the extent of blood pressure irregularities and whether these occur during 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 up
  • A test to assess perspiration
  • Tests to assess your bladder and bowel function
  • Electrocardiogram to track your heart’s electrical signals

If you have trouble sleeping, especially interrupted breathing or snoring, your doctor may recommend an evaluation at a sleep lab. This can help diagnose an underlying, treatable sleep disorder, such as sleep apnea.