Evidence map›Paper›PMID 40053259›Full record

ArticleClinical autonomic research : official journal of the Clinical Autonomic Research Society2025

The phenotype of "pure" autonomic failure.

Elizabeth A Coon, Negin Badihian, Stuart J McCarter, David M Sletten, Sarah E Berini, Eduardo E Benarroch, Paola Sandroni, Phillip A Low, Wolfgang Singer

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In one paragraph

Article in Clinical autonomic research : official journal of the Clinical Autonomic Research Society, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 2 papers.

0numbers the graph read from it
0cells of the map it votes in
2citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.

2 · The registry

The trial behind it

Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

2 citing papers in PubMed.

  1. Article
  2. Review
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

9 authors.

Elizabeth A CoonDepartment of Neurology, Mayo Clinic, 200 First Street SW, Rochester, MN, 55905, USA. Coon.elizabeth@mayo.edu.ORCID http://orcid.org/0000-0003-1206-0222
Negin BadihianDepartment of Neurology, Cleveland Clinic, Cleveland, OH, USA.
Stuart J McCarterDepartment of Neurology, Mayo Clinic, 200 First Street SW, Rochester, MN, 55905, USA.
David M SlettenDepartment of Neurology, Mayo Clinic, 200 First Street SW, Rochester, MN, 55905, USA.
Sarah E BeriniDepartment of Neurology, Mayo Clinic, 200 First Street SW, Rochester, MN, 55905, USA.
Eduardo E BenarrochDepartment of Neurology, Mayo Clinic, 200 First Street SW, Rochester, MN, 55905, USA.
Paola SandroniDepartment of Neurology, Mayo Clinic, 200 First Street SW, Rochester, MN, 55905, USA.
Phillip A LowDepartment of Neurology, Mayo Clinic, 200 First Street SW, Rochester, MN, 55905, USA.
Wolfgang SingerDepartment of Neurology, Mayo Clinic, 200 First Street SW, Rochester, MN, 55905, USA.

Funding

Mayo Clinic Center for Translational Science ActivitiesUL1TR000135 · NCATS · MAYO CLINIC ROCHESTER · PI KHOSLA, SUNDEEP · 2012 to 2015
$41.2M
Studies on Diagnosis, Pathophysiology, and Treatment of Autonomic Failure in MSAP01NS044233 · NINDS · UNIVERSITY OF CALIFORNIA, SAN DIEGO · PI MASLIAH, ELIEZER · 2003 to 2014
$12.4M
Training CoreU54NS065736 · NINDS · VANDERBILT UNIVERSITY MEDICAL CENTER · PI KAUFMANN, HORACIO · 2009 to 2018
$11.6M
Synucleinopathies – Novel Targets in Early Diagnosis, Pathophysiology, and Therapeutic ApproachR01NS092625 · NINDS · MAYO CLINIC ROCHESTER · PI SINGER, WOLFGANG, VEMURI, PRASHANTHI · 2015 to 2025
$6.6M
Differential Approach to the Postural Tachycardia SyndromeK23NS075141 · NINDS · MAYO CLINIC ROCHESTER · PI SINGER, WOLFGANG · 2012 to 2016
$872k
Phase 1 Study of Autologous Mesenchymal Stem Cell in Multiple System AtrophyR01FD004789 · FDA · MAYO CLINIC ROCHESTER · PI LOW, PHILLIP A · 2014 to 2016
$600k
Center for Clinical and Translational Science, Mayo Clinic UL1TR000135FDA HHS R01 FD004789NCATS NIH HHS UL1 TR000135NIH HHS K23NS075141NIH HHS NS 65736 FP00101673NIH HHS P01NS44233NIH HHS R01 FD004789NIH HHS R01 NS092625NIH HHS U54NS065736NINDS NIH HHS K23 NS075141NINDS NIH HHS P01 NS044233NINDS NIH HHS R01 NS092625NINDS NIH HHS U54 NS065736
6 · The paper itself

Abstract

purposeIdentifying features of patients who remain pure autonomic failure has implications on disease definition and offers insights into synucleinopathy progression. We sought to determine symptom timeline and autonomic features in patients who retain the pure autonomic failure phenotype with prolonged follow-up.

methodsWe reviewed all patients diagnosed with pure autonomic failure from 2001 to 2011 evaluated at Mayo Clinic, Rochester, with autonomic reflex screen and over 1 year of in-person follow-up. Clinical evaluations and patient telephone calls were used to assess timeline of symptoms.

resultsOf 202 patients, 133 remained pure autonomic failure with median follow-up time of 9.05 years (interquartile range (IQR) 4.2-13.1). Additional autonomic symptoms included constipation (N = 60; 45%), bladder symptoms (N = 78; 59%), which were severe in 50 patients (37.6%) with incontinence or requiring catheterization, sexual dysfunction (N = 53; 40%) and thermoregulatory dysfunction (N = 51; 38%). Assessment of dream enactment behavior was completed in 86 patients and endorsed in 45 patients (52%). Median time to dream enactment behavior onset from orthostatic hypotension was 7.00 years (1.55-13.50). Other autonomic symptoms tended to occur near orthostatic hypotension. Autonomic testing showed moderate to severe autonomic failure with median composite autonomic score of 6 (IQR 4-8; N = 133) and median percentage anhidrosis of 51% (IQR 3-93%; N = 105).

conclusionsPatients with pure autonomic failure typically have symptom onset near development of orthostatic hypotension while dream enactment behavior may occur later. Our findings underscore that not all patients with pure autonomic failure will develop motor or cognitive symptoms, even with prolonged follow-up.

Indexed as

Pure Autonomic FailureAdultAgedFemaleFollow-Up StudiesHumansMaleMiddle AgedPhenotypeDementia with Lewy bodiesMultiple system atrophyOrthostatic hypotensionParkinson diseasePure autonomic failure

Identifiers

PMID40053259
PMCPMC13524171

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Registered trials

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.