A health condition that occurs quite rarely and causes a sudden drop in blood pressure upon standing, and autonomic nervous system problems, is called pure autonomic failure (PAF). PAF often worsens over time, but there are some therapies available to manage the symptoms.
In other words, pure autonomic failure is a neurodegenerative disorder that negatively affects the autonomic nervous system. One of the most common features of PAF is orthostatic hypotension, but it may also cause other health problems.
Normally, the autonomic nervous system is responsible for the autonomic functions of the body. For example, blood pressure, heart rate, digestion, and breathing. However, if you develop PAF, you may experience dizziness and other symptoms (such as loss of bladder control or blurred vision), especially when standing from a chair.
While it may occur at any age, it is usually diagnosed in adults between the ages of 40 and 60. Sometimes, this condition is called idiopathic orthostatic hypotension or Bradbury-Eggleston syndrome.
Symptoms
The primary symptom of this condition is orthostatic hypotension, which can make you feel dizzy or cause fainting when you stand. However, PAF may also affect other structures and organs in the body. Check below for other symptoms of PAF:
- Anhidrosis (sweating less than usual)
- Hyperhidrosis (excessive sweating)
- Blurred vision caused by problems with the pupils
- Urinary retention (it occurs when the bladder is not emptying completely)
- Fecal incontinence (constipation or loss of bowel control)
- Sexual dysfunction (including erectile dysfunction, anorgasmia, and others)
- Loss of bladder control (also known as urinary incontinence)
Moreover, approximately 50% of people with pure autonomic failure develop a mild form of anemia. Do not hesitate to see a doctor if any of the previous symptoms occur.
Causes
This condition often occurs due to a buildup of an abnormal protein (alpha-synuclein) in the autonomic nerves. Thus, the affected nerves are in clusters (known as autonomic ganglia) on either side of the spinal cord and nearby organs. When these nerve cells decrease, they cause autonomic malfunctions. It is important to know that PAF does not affect any other nerves, and the brain and spinal cord do not experience damage. Nowadays, healthcare providers are studying the possible causes of alpha-synuclein buildup. In addition, when this protein builds up in the brain, it may cause Parkinson’s disease, multiple system atrophy, or Lewy body dementia. In some cases, people with PAF may develop one of the previous conditions.
What Are The Potential Complications of Pure Autonomic Failure?
People with this condition may also experience some complications, especially if they do not get treatment or develop a severe form of PAF. Check below some examples:
- Orthostatic hypotension (a sudden drop in blood pressure that may lead to blurred vision, fainting, or lightheadedness)
- Supine hypertension
- An increased risk of stroke and heart failure
- Bladder dysfunction
- Digestive problems (including swallowing problems, bloating, nausea, and others)
- Postprandial hypotension
- Sleep disorders (such as REM sleep behavior disorder)
- Vision changes (such as dry eyes, blurred vision, and others)
- Exercise intolerance
This document does not contain all possible complications of this condition. However, you can talk with your doctor about ways to reduce the risk or even avoid them. Additionally, there is no way to prevent this condition because doctors do not fully understand what exactly causes it.
Diagnosis
Usually, it is difficult to diagnose this condition because the symptoms it causes may resemble other health conditions. In most cases, doctors perform the following tests to rule out other disorders that may also cause orthostatic hypotension. Check below some examples:
- Tilt table test – This test is used to see how your blood pressure responds to standing up after lying down.
- QSART (quantitative sudomotor axon reflex test) – During this test, doctors will examine the nerves responsible for sweating. In general, QSART is often used to diagnose autonomic nervous system conditions, such as peripheral neuropathies and some types of pain disorders.
- Imaging tests – The following tests are used to get detailed images of the brain and heart muscle. They often perform them to rule out other disorders. For example, cardiac functional imaging and brain MRI (magnetic resonance imaging) scans.
- Laboratory tests – Reduced levels of Norepinephrine in the blood and urine typically support a PAF diagnosis.
- Thermoregulatory sweat test – It is used to measure how much you sweat in a room that has a controlled temperature, humidity, and airflow capabilities. However, this test is used less commonly during PAF diagnosis.
While it is overwhelming to go through previous tests, the right diagnosis helps make the best treatment plan for you.
Treatment
This condition cannot be cured. That’s why the treatment focuses on relieving the symptoms, preventing serious complications, and improving your quality of life. Check some treatment options below, according to the problems the condition causes:
Orthostatic Hypotension
Usually, physicians recommend the following strategies to manage this symptom. Check below some non-medical options:
- Slowly transitioning from lying or sitting position to standing
- Drink plenty of water to prevent dehydration
- Use electrolyte-replacement beverages to increase your salt intake
- Raise your head of the bed 4-6 inches
- Wear compression garments
In more severe cases, doctors may prescribe some medicines to manage orthostatic hypotension.
Bowel Problems
Usually, doctors recommend foods rich in fiber and stool softeners if you have constipation. When these options do not help, your doctor may recommend enemas. Check below some options often recommended by doctors for people with a loss of bowel control:
- Dietary changes
- Medicines (such as Loperamide and Diphenoxylate)
- Surgery (including sacral nerve stimulation or colostomy)
- Bowel training
Urinary Issues
The following medicines are often used to ease urinary problems. For example:
- Oxybutynin
- Mirabegron
- Tolterodine
- Bethanechol
When medicines do not work, doctors may recommend a Foley catheter.
Sexual Dysfunction
Doctors often prescribe erectile dysfunction (ED) medicines, such as Sildenafil, Tadalafil, and others, for people who have problems with erectile function. Moreover, previous medications may also help alleviate orthostatic hypotension.
Frequently Asked Questions
What is the cause of pure autonomic failure?
This serious health condition occurs due to abnormal buildup of the protein called alpha-synuclein in the nerve cells of the autonomic nervous system. Doctors do not fully understand why this process occurs.
Can pure autonomic failure go away?
Unfortunately, there is no way to cure this condition, but doctors often prescribe treatments to ease the symptoms, prevent serious complications, and improve your quality of life.
What is the life expectancy with PAF?
In most cases, people survive 20 years or more after diagnosis. Furthermore, some people with this condition may live into their 80s. If you have additional questions, ask your healthcare provider.
