Pure Autonomic Failure (PAF)
Pure Autonomic Failure (PAF), or Bradbury-Eggleston Syndrome, is a peripheral degenerative disorder of the autonomic nervous system (ANS). This means that the sympathetic and parasympathetic parts of the nervous system are breaking down. It usually affects more men than women in the middle to late life age range.
PAF symptoms include orthostatic hypotension (often with high blood pressure once laying down), dizziness, sweating abnormalities, neck pain, impotence in men, urinary changes, and various other autonomic signs without more widespread neurological involvement, along with a low resting supine plasma noradrenaline concentration.
The prognosis is good for PAF when compared to other similar disorders, such as Multiple System Atrophy, with a slower progression and more lifestyle and pharmacological treatment options. Increasing salt and fluid, compression stockings, and various medications to treat orthostatic hypotension are a few of the ways patients can cope with this condition.
For more detailed information and resources about PAF:
- The Autonomic Disorders Consortium
- The Vanderbilt University Autonomic Disorders Consortium
- Genetic and Rare Diseases Information Center (GARD)
Other PAF Resources
- Current Clinical Trials (PDF with links)
- PAF Journal Articles
- PAF in the News
Sources:
1. Cognitive Functioning in Orthostatic Hypotension Due to Pure Autonomic Failure, Hannah C.Heims, Hugo D. Critchley, Naomi H.Martin, H.Rolf Jäger, Christopher J.Mathias and Lisa Cipolotti, Clin Auton Res (2006) 16 : 113-120.
2. National Institutes of Health Autonomic Disorders Consortium
3. Vanderbilt University Autonomic Dysfunction Center
4. Progression and prognosis in pure autonomic failure (PAF): comparison with multiple system atrophy. Mabuchi N et al., J Neurol Neurosurg Psychiatry. 2005 Jul;76(7):947-52.
5. Consensus committee of the American Autonomic Society and the American Academy of Neurology on the definition of orthostatic hypotension, pure autonomic failure, and multiple system atrophy. Neurology 1996;46:1470.
6. Renal impairment of pure autonomic failure. Garland EM et al., Hypertension. 2009 Nov;54(5):1057-61. Epub 2009 Sep 8.
7. Orthostatic hypotension and supine hypertension in primary autonomic failure. Pathophysiology, diagnosis and treatment. Tykocki T et al., Kardiol Pol. 2010 Sep;68(9):1057-63.
8. Progression and prognosis in pure autonomic failure (PAF): comparison with multiple system atrophy. N Mabuchi, M Hirayama, Y Koike, H Watanabe, H Ito, R Kobayashi, K Hamada, G SobueJ Neurol Neurosurg Psychiatry 2005;76:947–952.
Originally written by Claire Martin, President of Dysautonomia SOS