Pure autonomic failure is caused by abnormal accumulation of synuclein in the brain.
Blood pressure may decrease when people stand, and they may sweat less and may have eye problems, retain urine, become constipated, or lose control of bowel movements.
Doctors do a physical examination and tests to look for signs of autonomic malfunction.
Treatment focuses on relieving symptoms.
(See also Overview of the Autonomic Nervous System Overview of the Autonomic Nervous System 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 .)
In pure autonomic failure (previously called idiopathic orthostatic hypotension or Bradbury-Eggleston syndrome), many processes regulated by the autonomic nervous system Overview of the Autonomic Nervous System 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 malfunction. These processes malfunction because the number of nerve cells that control them decreases. The affected cells are located in clusters (called autonomic ganglia) on either side of the spinal cord or near or in internal organs. Only the autonomic ganglia are affected. No other nerves are affected, and the brain and spinal cord are not affected.
Pure autonomic failure affects more women and tends to begin in a person’s 40s or 50s. It does not lead to death.
Pure autonomic failure is caused by abnormal accumulation of synuclein (a protein in the brain that helps nerve cells communicate, but whose function is not yet fully understood). Synuclein also accumulates in people who have 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 , multiple system atrophy Multiple System Atrophy (MSA) Multiple system atrophy is a progressive, fatal disorder that makes muscles stiff (rigid) and causes problems with movement, loss of coordination, and malfunction of internal body processes... 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 . Some people who have pure autonomic failure eventually develop multiple system atrophy Multiple System Atrophy (MSA) Multiple system atrophy is a progressive, fatal disorder that makes muscles stiff (rigid) and causes problems with movement, loss of coordination, and malfunction of internal body processes... read more or dementia with Lewy bodies.
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 may occur in people with disorders that involve accumulation of synuclein, including pure autonomic failure.
Symptoms of Pure Autonomic Failure
The most common symptom of pure autonomic failure is
An excessive decrease in blood pressure when a person stands ( 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 )
People may sweat less and become intolerant of heat.
The pupils may not widen (dilate) and narrow (constrict) normally. Vision may be blurred.
People may have difficulty emptying the bladder ( urine 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 ). They may be constipated 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 or lose control of bowel movements ( fecal incontinence Fecal Incontinence Fecal incontinence is the loss of control over bowel movements. Fecal incontinence can occur briefly during bouts of diarrhea or when hard stool becomes lodged in the rectum ( fecal impaction)... read more ). 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 ).
Diagnosis of Pure Autonomic Failure
A doctor's evaluation
Tests to rule out other possible causes
Doctors check for signs of autonomic dysfunction during the physical examination and with tests 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 .
If people have REM sleep behavior disorder and orthostatic hypotension due to autonomic dysfunction, they probably have pure autonomic failure.
Doctors may do a blood test to measure levels of norepinephrine. Norepinephrine is one of the chemical messengers (neurotransmitters) used by nerve cells to communicate with each other. This blood test may distinguish pure autonomic failure from other autonomic nervous system disorders that cause similar symptoms. Low levels of norepinephrine suggest pure autonomic failure.
Treatment of Pure Autonomic Failure
There is no specific treatment, so the focus is on relieving symptoms:
Orthostatic hypotension: Measures are taken to stabilize the sudden changes in blood pressure. Consuming more salt and water may increase the volume of blood and thus help increase blood pressure. Standing up slowly may help prevent blood pressure from decreasing too much or too fast when a person stands, as may wearing an abdominal binder or compression stockings. These garments help maintain blood pressure by promoting blood flow from the legs to the heart and thus prevent too much blood from staying (pooling) in the legs. Raising the head of the bed by about 4 inches (10 centimeters) can help prevent blood pressure from increasing too much when the person lies down. Fludrocortisone may be taken by mouth. It helps the body retain salt and water and thus may increase blood pressure as needed when a person stands. Other drugs, such as midodrine or droxidopa, taken by mouth, may also help.
Constipation Treatment 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 : A high-fiber diet and stool softeners are recommended. If constipation persists, enemas may be necessary.
Sweating abnormalities: If sweating is reduced or absent, people should avoid warm environments to avoid overheating the body.
Urinary retention Treatment 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 : If needed, people can learn to insert a catheter (a thin rubber tube) into the bladder themselves. They insert it several times a day. It is inserted through the urethra, allowing urine in the bladder to drain out. People remove the catheter after the bladder is empty. This measure helps prevent the bladder from stretching and urinary tract infections from developing. Washing the hands, cleansing the area around the urethra, and using a sterile or clean catheter also help prevent infections. Inserting a catheter becomes more difficult as coordination deteriorates. Sometimes drugs such as bethanechol are used to stimulate contractions of the bladder and thus help the bladder empty.
Urinary incontinence Treatment 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 : Oxybutynin, mirabegron, tamsulosin, or tolterodine, taken by mouth, may be used to relax the muscles of an overactive bladder. If incontinence persists, using a catheter inserted into the bladder may help. People may learn to insert it themselves.
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