Multiple system atrophy

Overview

Multiple system atrophy, also called MSA, causes people to lose coordination and balance or become slow and stiff. It also causes changes in speech and loss of control of other bodily functions.

MSA is a rare condition. It sometimes shares symptoms with Parkinson's disease, including slow movement, rigid muscles and poor balance.

Treatment includes medicines and lifestyle changes to help manage symptoms, but there is no cure. The condition gets worse over time and eventually leads to death.

In the past, this condition has been called Shy-Drager syndrome, olivopontocerebellar atrophy or striatonigral degeneration.

Symptoms

Multiple system atrophy (MSA) symptoms affect many parts of the body. Symptoms start in adulthood, usually in the 50s or 60s.

There are two types of MSA: parkinsonian and cerebellar. The type depends on the symptoms a person has when diagnosed.

Parkinsonian type

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

• Stiff muscles.
• Trouble bending the arms and legs.
• Slow movement, known as bradykinesia.
• Tremors at rest or when moving the arms or legs.
• Slurred, slow or soft speech, known as dysarthria.
• Trouble with posture and balance.

Cerebellar type

The main symptoms of the cerebellar type involve poor muscle coordination, known as ataxia. Symptoms may include:

• Trouble with movement and coordination. This includes loss of balance and not being able to walk steadily.
• Slurred, slow or soft speech, known as dysarthria.
• Changes in vision. This may include blurred or double vision and not being able to focus the eyes.
• Trouble chewing or swallowing, known as dysphagia.

General symptoms

For both types of multiple system atrophy, the autonomic nervous system doesn't work properly. The autonomic nervous system controls involuntary functions in the body, such as blood pressure. When this system doesn't work properly, it can cause the following symptoms.

Postural hypotension, also known as orthostatic hypotension

Postural hypotension is a form of low blood pressure. People who have this type of low blood pressure feel dizzy or lightheaded when they stand up after sitting or lying down. They may even faint. Not everyone with MSA has postural hypotension.

People with MSA also can develop dangerously high blood pressure levels while lying down. This is called supine hypertension.

Urinary and bowel symptoms

These symptoms include:

• Constipation.
• Loss of bladder or bowel control, known as incontinence.

Changes in sweat production

People with multiple system atrophy may:

• Produce less sweat.
• Have heat intolerance because they sweat less.
• Have poor body temperature control, often causing cold hands or feet.

Conditions that affect sleep

Sleep symptoms may include:

• Agitated sleep due to "acting out" dreams. This is known as rapid eye movement (REM) sleep behavior disorder.
• Breathing that stops and starts during sleep, known as sleep apnea.
• A high-pitched whistling sound while breathing, called stridor.

Sexual symptoms

These symptoms may include:

• Trouble getting or keeping an erection, known as erectile dysfunction.
• Trouble with lubrication during sex and having an orgasm.
• Loss of interest in sex.

Cardiovascular symptoms

MSA may cause:

• Color changes in the hands and feet.

Psychiatric symptoms

People with multiple system atrophy also may experience:

• Trouble controlling emotions, such as laughing or crying when not expected.

When to see a doctor

If you develop any of the symptoms of multiple system atrophy, see your healthcare professional. If you have already been diagnosed with MSA, contact your healthcare professional if your symptoms become worse or if new symptoms occur.

Causes

There is no known cause for multiple system atrophy (MSA). Some researchers are studying the possible role of genetics or environmental causes such as a toxin in MSA. But there's no substantial evidence to support these theories.

MSA causes parts of the brain to shrink. This is known as atrophy. The areas of the brain that shrink due to MSA include the cerebellum, basal ganglia and brainstem. The atrophy of these parts of the brain affect internal body functions and movement.

Under a microscope, the brain tissue of people with MSA shows a buildup of a protein called alpha-synuclein. Some research suggests that the buildup of this protein leads to multiple system atrophy.

Risk factors

A risk factor for multiple system atrophy (MSA) is having rapid eye movement (REM) sleep behavior disorder. People with this disorder act out their dreams. Most people who have MSA have a history of REM sleep behavior disorder.

Another risk factor is having a condition caused by the autonomic nervous system not working properly. Symptoms such as urinary incontinence could be an early sign of MSA. The autonomic nervous system controls involuntary functions.

Complications

Complications of multiple system atrophy (MSA) vary from person to person. But for everyone with the disease, MSA symptoms get worse over time. The symptoms can make daily activities harder as time goes on.

Possible complications include:

• Worsening breathing symptoms during sleep.
• Injuries from falls caused by poor balance or fainting.
• The breakdown of the skin in people who have trouble moving or can't move.
• Not being able to care for yourself in day-to-day activities.
• Vocal cord paralysis, which affects speech and breathing.
• Increased trouble swallowing.

People typically live about 7 to 10 years after multiple system atrophy symptoms first appear. However, the survival rate with MSA varies widely. Death is often due to trouble breathing, infections or blood clots in the lungs.