Pure Autonomic Failure

A degenerative disease of the AUTONOMIC NERVOUS SYSTEM that is characterized by idiopathic ORTHOSTATIC HYPOTENSION and a greatly reduced level of CATECHOLAMINES. No other neurological deficits are present.
Also Known As:
Autonomic Failure, Pure; Bradbury Eggleston Syndrome; Syndrome, Bradbury-Eggleston; Bradbury-Eggleston Syndrome
Networked: 144 relevant articles (4 outcomes, 11 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Orthostatic Hypotension (Postural Hypotension)
2. Parkinson Disease (Parkinson's Disease)
3. Multiple System Atrophy
4. Shy-Drager Syndrome
5. Diabetic Neuropathies (Diabetic Neuropathy)


1. Goldstein, David S: 21 articles (11/2015 - 08/2002)
2. Holmes, Courtney: 15 articles (11/2015 - 08/2002)
3. Sharabi, Yehonatan: 9 articles (11/2015 - 08/2003)
4. Biaggioni, Italo: 7 articles (02/2015 - 03/2002)
5. Mathias, Christopher J: 7 articles (01/2015 - 05/2002)
6. Mathias, C J: 7 articles (10/2008 - 02/2001)
7. Robertson, David: 6 articles (01/2013 - 10/2002)
8. Pechnik, Sandra: 6 articles (02/2009 - 08/2003)
9. Kaufmann, Horacio: 5 articles (05/2015 - 08/2003)
10. Jordan, Jens: 5 articles (02/2015 - 03/2002)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Pure Autonomic Failure:
1. Idiopathic orthostatic hypotensionIBA
2. Dopamine beta-HydroxylaseIBA
07/15/2003 - "Variations in the dopamine beta-hydroxylase gene are not associated with the autonomic disorders, pure autonomic failure, or multiple system atrophy."
07/15/2003 - "Recent findings of an association between human NE deficiency and variants at the dopamine beta-hydroxylase (DBH) gene [Kim et al., 2002] prompted us to investigate these markers in patients with autonomic disorders; 38 with orthostatic intolerance (OI), 26 with pure autonomic failure (PAF), and 39 with multiple system atrophy (MSA). "
08/01/1991 - "The effect of a balanced liquid meal on supine and postural blood pressure (BP) responses was investigated in three groups of patients with chronic autonomic failure; 10 with associated neurological impairment (multiple system atrophy (MSA), Shy-Drager syndrome) and seven without (of which five had pure autonomic failure (PAF); and two had a deficiency of the enzyme dopamine beta hydroxylase, DBH-deficiency). "
01/01/2006 - "DOPS improved orthostatic hypotension in patients with familial amyloid polyneuropathy, congenital deficiency of dopamine beta hydroxylase, pure autonomic failure and multiple system atrophy. "
04/01/1995 - "The effect of supine exercise on blood pressure (BP), measured while recumbent and after head-up postural change, was investigated in three groups with marked postural hypotension due to chronic autonomic failure: 15 with associated neurological impairment (Shy-Drager syndrome, SDS, multiple system atrophy); 15 with pure autonomic failure (PAF) and two with a deficiency of the enzyme dopamine beta hydroxylase (DBH deficiency). "
3. Midodrine (Midon)FDA LinkGeneric
4. cevimeline (Evoxac)FDA Link
5. hydroxide ionIBA
6. DroxidopaIBA
7. 3-Iodobenzylguanidine (Iobenguane)IBA
8. Dopamine beta hydroxylase deficiencyIBA
04/01/1995 - "1. The cardiovascular and catecholamine responses to supine leg exercise were measured in fifteen normal subjects (controls) and in three groups with sympathetic dysfunction: fifteen with central failure (Shy-Drager syndrome; SDS), fifteen with peripheral failure (pure autonomic failure; PAF) and two with isolated dopamine beta-hydroxylase deficiency (DBH deficiency). "
01/01/1998 - "Four different forms of primary autonomic failure (multiple system atrophy, pure autonomic failure, Parkinson's disease and dopamine beta-hydroxylase deficiency) have been described. "
07/01/1994 - "We compared the three techniques under conditions of effective cardiac sympathetic denervation, pure autonomic failure (n = 4), dopamine beta-hydroxylase deficiency (n = 1), and after cardiac transplantation (n = 9) as well as in the context of sympathetic nervous activation in cardiac failure (n = 15) and with aging (n = 10). "
11/01/2015 - "Norepinephrine deficiency in NOH can arise peripherally, due to cardiovascular sympathetic denervation (as in pure autonomic failure, Parkinson's disease, and a variety of neuropathies), or centrally, due to a failure of viscerosensory signals to generate adequate sympathetic traffic to intact sympathetic nerve endings (as in multiple system atrophy). "
09/01/2006 - "This literature review confirms a high prevalence of sympathetic deficits and parasympathetic deficits in acute or subacute dysautonomia, diabetes, amyloidosis, pure autonomic failure, paraneoplastic syndromes, Sjögren syndrome, familial dysautonomia, and dopamine beta-hydroxylase deficiency. "
9. SynucleinsIBA
10. AntibodiesIBA
11/01/2015 - "This is because some of the patients with chronic progression mimicking neurodegenerative diseases such as pure autonomic failure were positive for these antibodies. "
06/01/2003 - "These observations expand the clinical spectrum of AAN to include chronic cases, some being indistinguishable from pure autonomic failure, and support the concept that ganglionic AChR antibodies are important diagnostically and pathophysiologically in acquired dysautonomia."
12/01/2006 - "In this study we evaluated by indirect immunohistochemistry the prevalence of cerebrospinal fluid (CSF) antibodies reacting with structures of rat pons/medulla in patients with multiple system atrophy (MSA) (n = 29), Parkinson disease with neurogenic orthostatic hypotension (n = 13), or pure autonomic failure (n = 11) and in control subjects without autonomic failure (n = 33). "
04/01/2013 - "Based on the detection of these antibodies in various dysautonomic conditions, AAG is considered a broad-spectrum disease entity that includes acute pandysautonomia as well as secondary autonomic neuropathy, restricted forms of dysautonomia (postural tachycardia syndrome and chronic intestinal pseudoobstruction), and chronic dysautonomia, mimicking pure autonomic failure. "
05/01/2014 - "Recently, autoimmune autonomic ganglionopathy associated with anti-ganglionic acetylcholine receptor antibodies has attracted attention as a differential diagnosis of pure autonomic failure, which is characterized by Lewy body pathology."

Therapies and Procedures

1. Denervation
2. Sympathectomy (Sympathectomies)
3. Bed Rest (Bedrest)
4. Autonomic Denervation
5. Catheters