Dysautonomia International’s 6th Annual Conference concluded on June 24th, 2018. We have gathered some of the key points that were discussed there, which we will share in a series of articles, so that you are up to date on all of the action.
Summary of Pure Autonomic Failure
Pure Autonomic Failure (PAF) is a peripheral degenerative disorder of the autonomic nervous system (ANS). PAF was formerly known as Bradbury-Eggleston Syndrome, after the two researchers who first described it in 1925. PAF is also referred to as idiopathic orthostatic hypotension by some physicians. PAF is one of three diseases classified as a primary autonomic disorder, the other two being Multiple System Atrophy and Parkinson’s Disease.
Symptoms
PAF usually affects more men than women, in middle to later life. However, it is occasionally seen in younger patients. PAF is characterized by orthostatic hypotension (low blood pressure upon standing), a low resting supine plasma noradrenaline concentration that does not increase significantly upon standing, a decreased ability to sweat, persistent neck pain that is often relieved when lying down, raised blood pressure while laying down, changes in urinary habits, and poor tolerance of high altitude. In men with PAF, impotence often occurs.
What Causes PAF?
Researchers are not certain of the underlying pathology of PAF yet, but at least in some cases, a loss of nerve cells in the spinal cord has been documented.
Treatment
Treatment often focuses on counteracting the effects of orthostatic hypotension, and supine hypertension, which can be difficult. Patients are often advised to increase fluid and salt intake, wear compression stockings, sleep with the head of their bed at an upright angle, and to use pharmacological options as directed by their physician.
Prognosis
The prognosis is good for PAF compared to other similar disorders, such as Multiple System Atrophy, with a slower progression and more lifestyle and pharmacological treatment options. Most PAF patients survive 20 years or more after diagnosis, many into their 80’s. The cause of death is often recurrent infection or a pulmonary embolism. PAF patients have a reduced risk of heart attacks and stroke.
Source: Dysautonomia International
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