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Pure autonomic failure* (PAF) * is a neurodegenerative disease of the autonomic nervous system, which regulates involuntary body processes such as blood pressure and breathing rate. PAF affects only the peripheral autonomic nervous system, which means it does not involve the brain and spinal cord (the central nervous system). It usually begins in midlife, although it can begin earlier. The main symptom of PAF is orthostatic hypotension, a sudden drop in blood pressure upon standing. This causes a person to feel dizzy and lightheaded, requiring them to sit or lie down to prevent fainting. People with PAF also have an abnormal blood pressure response to other common daily activities or exposures, as well as additional symptoms affecting various body systems.
The reason that PAF develops is not known. It is not known to be inherited. The symptoms are caused by abnormal accumulations of protein, called Lewy bodies, in the cells of autonomic nerves. The Lewy bodies restrict the production and release of norepinephrine from nerve cells, which in turn causes hypotension. Diagnosing PAF may involve tilt table testing, 24-hour blood pressure monitoring, hyperventilation testing, and a norepinephrine blood test. Treatment options do not stop disease progression but help to control symptoms. Options may include avoiding triggers of symptoms, increasing fluid and salt intake, using compressive garments, and/or various medications. PAF usually has a slowly progressive course and may result in a person being restricted to a reclined position in their home.
Source: GARD Last updated on 05-01-20
The primary symptom of pure autonomic failure (PAF) is orthostatic hypotension, which is a sudden drop in blood pressure that occurs when a person stands up. This causes a person to feel dizzy and lightheaded, and causes the need to sit, squat, or lie down in order to prevent fainting. Interestingly, when people with PAF lie down with their face upward, they often have blood pressure readings consistent with high blood pressure (hypertension). People with PAF also have dramatic changes in blood pressure in response to common activities such as eating, drinking, or exercising, as well as to exposure to heat or certain medications. For example, blood pressure drops after eating (especially carbohydrate-rich foods) and exercising. However, drinking fluids causes blood pressure to rise. The symptoms of PAF are typically worse in the morning and in hot weather.
Other symptoms of PAF may include an inability or decreased ability to sweat; dry mouth; diarrhea or constipation; neck pain that is often relieved when lying down; changes in urinary habits; impaired sense of smell; and sexual dysfunction (such as erectile dysfunction in men). In general, PAF does not cause cognitive impairment. However, some people with PAF may have deficits of attention or executive functioning.
Last updated on 05-01-20
MedlinePlus, a Web site designed by the National Library of Medicine Web site to help you research your health questions, provides more information about autonomic nervous system disorders in general. Click on the link to view this information.
Last updated on 04-27-20
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