U.S. flag

An official website of the United States government

Format

Send to:

Choose Destination

Pure autonomic failure

MedGen UID:
98293
•Concept ID:
C0393911
•
Disease or Syndrome
Synonyms: Bradbury-Eggleston syndrome; Idiopathic orthostatic hypotension (a symptom); Orthostatic hypotension (a symptom)
SNOMED CT: Bradbury-Eggleston syndrome (84438001); Pure autonomic failure (84438001); Autonomic failure (84438001)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
•Concept ID:
CN307044
•
Finding
Source: Orphanet
clinical entity without genetic inheritance.
See: This record
Not genetically inherited (Orphanet)
 
Monarch Initiative: MONDO:0018608
OMIM®: 146500
Orphanet: ORPHA441

Definition

Pure autonomic failure (PAF) is a neurodegenerative disease that affects the sympathetic branch of the autonomous nervous system and that manifests with orthostatic hypotension. [from ORDO]

Professional guidelines

PubMed

Pharmacologic treatment of orthostatic hypotension.
Park JW, Okamoto LE, Shibao CA, Biaggioni I
Auton Neurosci 2020 Dec;229:102721. Epub 2020 Aug 28 doi: 10.1016/j.autneu.2020.102721. PMID: 32979782Free PMC Article
Autonomic dysfunction in Parkinson's disease: Implications for pathophysiology, diagnosis, and treatment.
Chen Z, Li G, Liu J
Neurobiol Dis 2020 Feb;134:104700. Epub 2019 Dec 3 doi: 10.1016/j.nbd.2019.104700. PMID: 31809788
Autonomic dysfunction: Diagnosis and management.
Rafanelli M, Walsh K, Hamdan MH, Buyan-Dent L
Handb Clin Neurol 2019;167:123-137. doi: 10.1016/B978-0-12-804766-8.00008-X. PMID: 31753129

Recent clinical studies

Etiology

Synucleinopathies.
Lamotte G, Singer W
Handb Clin Neurol 2023;196:175-202. doi: 10.1016/B978-0-323-98817-9.00032-6. PMID: 37620069
Seed amplification assay for the detection of pathologic alpha-synuclein aggregates in cerebrospinal fluid.
Concha-Marambio L, Pritzkow S, Shahnawaz M, Farris CM, Soto C
Nat Protoc 2023 Apr;18(4):1179-1196. Epub 2023 Jan 18 doi: 10.1038/s41596-022-00787-3. PMID: 36653527Free PMC Article
Synuclein in neurodegeneration.
Bougea A
Adv Clin Chem 2021;103:97-134. Epub 2020 Oct 20 doi: 10.1016/bs.acc.2020.08.007. PMID: 34229853
Dysautonomia: A Forgotten Condition - Part 1.
Rocha EA, Mehta N, Távora-Mehta MZP, Roncari CF, Cidrão AAL, Elias Neto J
Arq Bras Cardiol 2021 Apr;116(4):814-835. doi: 10.36660/abc.20200420. PMID: 33886735Free PMC Article
Synucleinopathies.
Coon EA, Singer W
Continuum (Minneap Minn) 2020 Feb;26(1):72-92. doi: 10.1212/CON.0000000000000819. PMID: 31996623Free PMC Article

Diagnosis

Seed amplification assay for the detection of pathologic alpha-synuclein aggregates in cerebrospinal fluid.
Concha-Marambio L, Pritzkow S, Shahnawaz M, Farris CM, Soto C
Nat Protoc 2023 Apr;18(4):1179-1196. Epub 2023 Jan 18 doi: 10.1038/s41596-022-00787-3. PMID: 36653527Free PMC Article
Pharmacologic treatment of orthostatic hypotension.
Park JW, Okamoto LE, Shibao CA, Biaggioni I
Auton Neurosci 2020 Dec;229:102721. Epub 2020 Aug 28 doi: 10.1016/j.autneu.2020.102721. PMID: 32979782Free PMC Article
Autonomic dysfunction in Parkinson's disease: Implications for pathophysiology, diagnosis, and treatment.
Chen Z, Li G, Liu J
Neurobiol Dis 2020 Feb;134:104700. Epub 2019 Dec 3 doi: 10.1016/j.nbd.2019.104700. PMID: 31809788
Autonomic dysfunction: Diagnosis and management.
Rafanelli M, Walsh K, Hamdan MH, Buyan-Dent L
Handb Clin Neurol 2019;167:123-137. doi: 10.1016/B978-0-12-804766-8.00008-X. PMID: 31753129
Orthostatic Hypotension: JACC State-of-the-Art Review.
Freeman R, Abuzinadah AR, Gibbons C, Jones P, Miglis MG, Sinn DI
J Am Coll Cardiol 2018 Sep 11;72(11):1294-1309. doi: 10.1016/j.jacc.2018.05.079. PMID: 30190008

Therapy

Autonomic dysfunction in Parkinson's disease: Implications for pathophysiology, diagnosis, and treatment.
Chen Z, Li G, Liu J
Neurobiol Dis 2020 Feb;134:104700. Epub 2019 Dec 3 doi: 10.1016/j.nbd.2019.104700. PMID: 31809788
REM Sleep Behavior Disorder in Parkinson's Disease and Other Synucleinopathies.
St Louis EK, Boeve AR, Boeve BF
Mov Disord 2017 May;32(5):645-658. doi: 10.1002/mds.27018. PMID: 28513079
Cardiovascular complications in patients with autonomic failure.
Milazzo V, Di Stefano C, Milan A, Ravera A, Sobrero G, Sabia L, Veglio F, Maule S
Clin Auton Res 2015 Jun;25(3):133-40. Epub 2015 Mar 20 doi: 10.1007/s10286-015-0275-0. PMID: 25791260
Vagolysis.
Hutchinson CD, Jardine DL, Hurrell M
Age Ageing 2008 Sep;37(5):602-4. Epub 2008 Jun 12 doi: 10.1093/ageing/afn105. PMID: 18556705
Treatment of orthostatic hypotension.
Freeman R
Semin Neurol 2003 Dec;23(4):435-42. doi: 10.1055/s-2004-817727. PMID: 15088264

Prognosis

Dysautonomia: A Forgotten Condition - Part 1.
Rocha EA, Mehta N, Távora-Mehta MZP, Roncari CF, Cidrão AAL, Elias Neto J
Arq Bras Cardiol 2021 Apr;116(4):814-835. doi: 10.36660/abc.20200420. PMID: 33886735Free PMC Article
Autonomic dysfunction in Parkinson's disease: Implications for pathophysiology, diagnosis, and treatment.
Chen Z, Li G, Liu J
Neurobiol Dis 2020 Feb;134:104700. Epub 2019 Dec 3 doi: 10.1016/j.nbd.2019.104700. PMID: 31809788
Pure Autonomic Failure.
Coon EA, Singer W, Low PA
Mayo Clin Proc 2019 Oct;94(10):2087-2098. Epub 2019 Sep 9 doi: 10.1016/j.mayocp.2019.03.009. PMID: 31515103Free PMC Article
Pure Autonomic Failure.
Thaisetthawatkul P
Curr Neurol Neurosci Rep 2016 Aug;16(8):74. doi: 10.1007/s11910-016-0673-2. PMID: 27338613
Pure autonomic failure.
Garland EM, Hooper WB, Robertson D
Handb Clin Neurol 2013;117:243-57. doi: 10.1016/B978-0-444-53491-0.00020-1. PMID: 24095130

Clinical prediction guides

An overview on pure autonomic failure.
Pavy-Le Traon A, Foubert-Samier A, Fabbri M
Rev Neurol (Paris) 2024 Jan-Feb;180(1-2):94-100. Epub 2023 Dec 20 doi: 10.1016/j.neurol.2023.11.003. PMID: 38129276
Pure autonomic failure and the differential diagnosis of autonomic peripheral neuropathies.
González-Duarte A, Varma-Doyle A, Freeman R
Curr Opin Neurol 2021 Oct 1;34(5):675-682. doi: 10.1097/WCO.0000000000000985. PMID: 34392300
Predicting phenoconversion in pure autonomic failure.
Coon EA, Mandrekar JN, Berini SE, Benarroch EE, Sandroni P, Low PA, Singer W
Neurology 2020 Aug 18;95(7):e889-e897. Epub 2020 Jun 16 doi: 10.1212/WNL.0000000000010002. PMID: 32546656Free PMC Article
Autonomic dysfunction in Parkinson's disease: Implications for pathophysiology, diagnosis, and treatment.
Chen Z, Li G, Liu J
Neurobiol Dis 2020 Feb;134:104700. Epub 2019 Dec 3 doi: 10.1016/j.nbd.2019.104700. PMID: 31809788
Autonomic dysfunction in genetic forms of synucleinopathies.
Chelban V, Vichayanrat E, Schottlaende L, Iodice V, Houlden H
Mov Disord 2018 Mar;33(3):359-371. doi: 10.1002/mds.27343. PMID: 29508456

Recent systematic reviews

Hemodynamic Effects of the Osmopressor Response: A Systematic Review and Meta-Analysis.
Oyewunmi OA, Lei LY, Laurin JKH, Morillo CA, Sheldon RS, Raj SR
J Am Heart Assoc 2023 Nov 7;12(21):e029645. Epub 2023 Nov 6 doi: 10.1161/JAHA.122.029645. PMID: 37929748Free PMC Article
The potential prognostic role of cardiovascular autonomic failure in α-synucleinopathies.
Fanciulli A, Strano S, Colosimo C, Caltagirone C, Spalletta G, Pontieri FE
Eur J Neurol 2013 Feb;20(2):231-5. Epub 2012 Jul 27 doi: 10.1111/j.1468-1331.2012.03819.x. PMID: 22834919

Supplemental Content

Table of contents

    Clinical resources

    Practice guidelines

    • PubMed
      See practice and clinical guidelines in PubMed. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.
    • Bookshelf
      See practice and clinical guidelines in NCBI Bookshelf. The search results may include broader topics and may not capture all published guidelines. See the FAQ for details.

    Reviews

    Related information

    • MeSH
      Related Medical Subject Headings
    • OMIM
      Related records in OMIM
    • PMC Articles
      Related information in PubMed Central Links
    • PubMed
      Related literature resources in PubMed
    • PubMed (OMIM)
      Related literature resources in PubMed

    Recent activity

    Clear Turn Off Turn On

    Your browsing activity is empty.

    Activity recording is turned off.

    Turn recording back on

    See more...