Pure Autonomic Failure (PAF)
What is pure autonomic failure?
Pure autonomic failure (PAF) is a rare, neurodegenerative disorder that impacts the autonomic nervous system (ANS). In the past, it has also been referred to as Bradbury-Eggleston syndrome.
What are the symptoms of pure autonomic failure?
Symptoms of this condition typically manifest when a patient is middle-aged. These effects include:
- Orthostatic hypotension
- Blood pressure drops suddenly when a patient stands up
- Weakness
- Syncope
- Fainting due to a fall in blood pressure
- Dizziness
- Lightheadedness
- Blurry vision
- Bladder issues
- Sleep disorders
- Abnormal sweating
- Constipation
What causes pure autonomic failure?
While medical professionals are aware that Lewy bodies accumulate in autonomic nerve cells, they do not know why this happens. These proteins affect norepinephrine’s production and release from the nerve cells, which leads to the characteristic symptoms of PAF.
How is pure autonomic failure diagnosed?
Doctors will first perform a physical examination and look for the characteristic symptoms of PAF. A neurological examination is next, which must be thorough and will most likely be followed by tilt table testing, hyperventilation testing, a norepinephrine blood test, and 24-hour blood pressure monitoring.
What are the treatments for pure autonomic failure?
There is no cure for this condition; treatment is based on symptom management. Doctors will recommend lifestyle changes and medications. Lifestyle changes include drinking more fluids, increasing one’s salt intake, and maintaining a healthy exercise regimen. Medications include midodrine, droxidopa, fludrocortisone, and pyridostigmine.
