Multiple System Atrophy (MSA)

Multiple system atrophy results from degeneration of several parts of the brain and spinal cord:

The cause of the degeneration is unknown, but degeneration in multiple system atrophy probably results when synuclein changes shape (misfolds) and accumulates in support cells in the brain. Synuclein is a protein in the brain that helps nerve cells communicate, but whose function is not yet fully understood. Abnormal synuclein can also accumulate in people with pure autonomic failure Pure Autonomic Failure Pure autonomic failure is dysfunction of many of the processes controlled by the autonomic nervous system, such as control of blood pressure. It is not fatal. Pure autonomic failure is caused... read more , Parkinson disease Parkinson Disease (PD) Parkinson disease is a slowly progressive degenerative disorder of specific areas of the brain. It is characterized by tremor when muscles are at rest (resting tremor), increased muscle tone... read more , or dementia with Lewy bodies Dementia With Lewy Bodies and Parkinson Disease Dementia Dementia with Lewy bodies is progressive loss of mental function characterized by the development of Lewy bodies in nerve cells. Parkinson disease dementia is loss of mental function characterized... read more .

Rapid eye movement (REM) sleep behavior disorder Rapid eye movement sleep behavior disorder Parasomnias are unusual behaviors that occur just before falling asleep, during sleep, or when waking up. (See also Overview of Sleep.) Various unconscious and largely unremembered behaviors... read more often occurs in people with disorders that involve accumulation of synuclein, including multiple system atrophy.

Multiple system atrophy is a progressive disorder. Early symptoms of multiple system atrophy vary, depending on which part and how much of the brain is affected first. The disorder causes three groups of symptoms.

Parkinsonism Parkinsonism Parkinsonism refers to symptoms of Parkinson disease (such as slow movements and tremors) that are caused by another condition. Parkinsonism is caused by brain disorders, brain injuries, or... read more —symptoms that resemble those of Parkinson disease—may occur. These symptoms result from degeneration in the basal ganglia. Muscles are stiff (rigid), and movements become slow, shaky, and difficult to initiate. When walking, people may shuffle and not swing their arms. People feel unsteady and off balance, making them more likely to fall. Posture may be stooped. Limbs may tremble jerkily, usually when they are held in one position. But people with multiple system atrophy are less likely to have tremors during rest than people with Parkinson disease. Articulating words is difficult, and the voice may become high-pitched and quaver.

Loss of coordination may occur. It results from degeneration in the cerebellum. People lose their balance. Later, they may be unable to control movements of their arms and legs. Consequently, they have difficulty walking and take wide, irregular steps. When reaching for an item, they may reach beyond it. When sitting, they may feel unstable. People may have difficulty focusing their eyes on and following objects. Tasks that require rapidly alternating movements, such as turning a door knob or screwing in a light bulb, also become difficult.

Malfunction of internal body processes, controlled by the autonomic nervous system, may also occur. Blood pressure may decrease dramatically when a person stands up, causing dizziness, light-headedness, or fainting—a condition called orthostatic hypotension Dizziness or Light-Headedness When Standing Up In some people, particularly older people, blood pressure drops excessively when they sit or stand up (a condition called orthostatic or postural hypotension). Symptoms of faintness, light-headedness... read more . Blood pressure may increase when a person lies down.

People may need to urinate urgently or frequently or may pass urine involuntarily (urinary incontinence Urinary Incontinence in Adults Urinary incontinence is involuntary loss of urine. Incontinence can occur in both men and women at any age, but it is more common among women and older people, affecting about 30% of older women... read more ). They may have difficulty emptying the bladder (urinary retention Urinary Retention Urinary retention is inability to urinate or incomplete emptying of the bladder. People who have incomplete emptying of the bladder may have urinary frequency or urinary incontinence. If the... read more ). Constipation Constipation in Adults Constipation is difficult or infrequent bowel movements, hard stool, or a feeling that the rectum is not totally empty after a bowel movement (incomplete evacuation). (See also Constipation... read more is common. Vision becomes poor. Men may have difficulty initiating and maintaining an erection (erectile dysfunction Erectile Dysfunction (ED) Erectile dysfunction (ED) is the inability to attain or sustain an erection satisfactory for sexual intercourse. (See also Overview of Sexual Dysfunction in Men.) Every man occasionally has... read more ).

Other symptoms of autonomic malfunction may include the following:

Many people are confined to a wheelchair or are otherwise severely disabled within 5 years after symptoms begin. The disorder results in death 9 to 10 years after symptoms begin.

The diagnosis of multiple system atrophy is based on a doctor's evaluation and results of certain tests. For example, doctors may suspect multiple system atrophy if parkinsonian symptoms are rapidly worsening and levodopa (used to treat Parkinson disease) has little or no effect on symptoms.

Magnetic resonance imaging (MRI) is usually done to check for certain changes in the brain.

Tests to evaluate the autonomic nervous system Diagnosis The autonomic nervous system regulates certain body processes, such as blood pressure and the rate of breathing. This system works automatically (autonomously), without a person’s conscious... read more are done. They include the thermoregulatory sweat test and measurement of blood pressure while the person is sitting and after the person stands to check for orthostatic hypotension.

If MRI shows changes that suggest multiple system atrophy and test results are abnormal, multiple system atrophy is likely.

No treatment can cure multiple system atrophy. However, a combination of simple measures and drugs may help relieve symptoms.

As the multiple system atrophy progresses, people may need a breathing tube, feeding tube (usually surgically inserted), or both.

Physical, occupational, and speech therapists can teach people ways to compensate when walking, doing daily activities, and speaking become difficult. Social workers can help people find support groups and, when symptoms become disabling, home health care or hospice services.