Evidence map›Paper›PMID 26138942›Full record

ArticleNeurology2015

When DLB, PD, and PSP masquerade as MSA: an autopsy study of 134 patients.

Shunsuke Koga, Naoya Aoki, Ryan J Uitti, Jay A van Gerpen, William P Cheshire, Keith A Josephs, Zbigniew K Wszolek, J William Langston, Dennis W Dickson

Registry-linked trialAbstract read
In one paragraph

Article in Neurology, 2015. The graph could read no effect estimate from its abstract, so it casts no vote on the map. It is linked to trial NCT04496830 (Cerebrospinal Fluid-biomarkers-based Diagnostic and Prognostic Models for Multiple Sclerosis), which is not on this map. Cited by 205 papers, 4 of them syntheses that pooled it.

0numbers the graph read from it
0cells of the map it votes in
205citing papers in PubMed, 4 pooled it
–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.

NCT04496830 completednot on this mapstarted 2020, after this paper: background citation

Cerebrospinal Fluid-biomarkers-based Diagnostic and Prognostic Models for Multiple Sclerosis

TypeobservationalSponsorWashington University School of MedicineRan2020 to 2023Enrolled161ConditionsMultiple Sclerosis
3 · Its place in the literature

Who cites it

205 citing papers in PubMed, 4 syntheses or guidelines pooled it.

  1. Pooled it
  2. The Movement Disorder Society Criteria for the Diagnosis of Multiple System Atrophy.Movement disorders : official journal of the Movement Disorder Society · 2022
    Pooled it
  3. Pooled it
  4. Pooled it
  5. Article
  6. Article
  7. Multiple system atrophy: cure and care.Journal of neural transmission (Vienna, Austria : 1996) · 2026
    Review
  8. Article
  9. Article
  10. Article
  11. Serotonin Transporter Distribution in the Brainstem of Multiple-System Atrophy-Parkinsonian Type: Insights from Pathology andJournal of nuclear medicine : official publication, Society of Nuclear Medicine · 2026
    Article
  12. Article
  13. Article
  14. Article
  15. Locus coeruleus degeneration is associated with orthostatic hypotension in Parkinson's disease and multiple system atrophy.Clinical autonomic research : official journal of the Clinical Autonomic Research Society · 2026
    Article
  16. How to treat cardiovascular autonomic failure in Parkinson's disease.Journal of neural transmission (Vienna, Austria : 1996) · 2026
    Review
  17. Article
  18. Review
  19. Review
  20. Article

145 more citing papers are in PubMed but not listed here.

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.

Shunsuke KogaFrom the Departments of Neuroscience (S.K., N.A., D.W.D.) and Neurology (R.J.U., J.A.v.G., W.P.C., Z.K.W.), Mayo Clinic, Jacksonville, FL; the Department of Neurology (Behavioural Neurology) (K.A.J.), Mayo Clinic, Rochester, MN; and The Parkinson's Institute and Clinical Center (J.W.L.), Sunnyvale, CA.
Naoya AokiFrom the Departments of Neuroscience (S.K., N.A., D.W.D.) and Neurology (R.J.U., J.A.v.G., W.P.C., Z.K.W.), Mayo Clinic, Jacksonville, FL; the Department of Neurology (Behavioural Neurology) (K.A.J.), Mayo Clinic, Rochester, MN; and The Parkinson's Institute and Clinical Center (J.W.L.), Sunnyvale, CA.
Ryan J UittiFrom the Departments of Neuroscience (S.K., N.A., D.W.D.) and Neurology (R.J.U., J.A.v.G., W.P.C., Z.K.W.), Mayo Clinic, Jacksonville, FL; the Department of Neurology (Behavioural Neurology) (K.A.J.), Mayo Clinic, Rochester, MN; and The Parkinson's Institute and Clinical Center (J.W.L.), Sunnyvale, CA.
Jay A van GerpenFrom the Departments of Neuroscience (S.K., N.A., D.W.D.) and Neurology (R.J.U., J.A.v.G., W.P.C., Z.K.W.), Mayo Clinic, Jacksonville, FL; the Department of Neurology (Behavioural Neurology) (K.A.J.), Mayo Clinic, Rochester, MN; and The Parkinson's Institute and Clinical Center (J.W.L.), Sunnyvale, CA.
William P CheshireFrom the Departments of Neuroscience (S.K., N.A., D.W.D.) and Neurology (R.J.U., J.A.v.G., W.P.C., Z.K.W.), Mayo Clinic, Jacksonville, FL; the Department of Neurology (Behavioural Neurology) (K.A.J.), Mayo Clinic, Rochester, MN; and The Parkinson's Institute and Clinical Center (J.W.L.), Sunnyvale, CA.
Keith A JosephsFrom the Departments of Neuroscience (S.K., N.A., D.W.D.) and Neurology (R.J.U., J.A.v.G., W.P.C., Z.K.W.), Mayo Clinic, Jacksonville, FL; the Department of Neurology (Behavioural Neurology) (K.A.J.), Mayo Clinic, Rochester, MN; and The Parkinson's Institute and Clinical Center (J.W.L.), Sunnyvale, CA.
Zbigniew K WszolekFrom the Departments of Neuroscience (S.K., N.A., D.W.D.) and Neurology (R.J.U., J.A.v.G., W.P.C., Z.K.W.), Mayo Clinic, Jacksonville, FL; the Department of Neurology (Behavioural Neurology) (K.A.J.), Mayo Clinic, Rochester, MN; and The Parkinson's Institute and Clinical Center (J.W.L.), Sunnyvale, CA.
J William LangstonFrom the Departments of Neuroscience (S.K., N.A., D.W.D.) and Neurology (R.J.U., J.A.v.G., W.P.C., Z.K.W.), Mayo Clinic, Jacksonville, FL; the Department of Neurology (Behavioural Neurology) (K.A.J.), Mayo Clinic, Rochester, MN; and The Parkinson's Institute and Clinical Center (J.W.L.), Sunnyvale, CA.
Dennis W DicksonFrom the Departments of Neuroscience (S.K., N.A., D.W.D.) and Neurology (R.J.U., J.A.v.G., W.P.C., Z.K.W.), Mayo Clinic, Jacksonville, FL; the Department of Neurology (Behavioural Neurology) (K.A.J.), Mayo Clinic, Rochester, MN; and The Parkinson's Institute and Clinical Center (J.W.L.), Sunnyvale, CA. dickson.dennis@mayo.edu.

Funding

Vascular Structure and Function in Cognitive AgingP01AG003949 · NIA · YESHIVA UNIVERSITY · PI CAROL A. DERBY · 1985 to 2026
$73.9M
THE PGRN/TDP-43 AXIS IN ALZHEIMER?S DISEASE AND NEURODEGENERATIONP50AG016574 · NIA · MAYO CLINIC ROCHESTER · PI VEMURI, PRASHANTHI · 1999 to 2018
$36.9M
Neuropathology CoreP50NS072187 · NINDS · MAYO CLINIC JACKSONVILLE · PI DICKSON, DENNIS WILLIAM · 2010 to 2017
$8.7M
Toxicant-induced alpha-synuclein pathology in primatesU54ES012077 · NIEHS · PARKINSON'S INSTITUTE · PI LANGSTON, JAMES WILLIAM · 2002 to 2007
$7.4M
Longitudinal multi-modality imaging in progressive apraxia of speech (Diversity Supplement)R01DC012519 · NIDCD · MAYO CLINIC ROCHESTER · PI Jennifer Louise Whitwell · 2013 to 2026
$7.2M
Understanding the role of TDP-43 in Alzheimers disease and FTLDR01AG037491 · NIA · MAYO CLINIC ROCHESTER · PI JOSEPHS, KEITH A · 2010 to 2024
$6.5M
Biomarkers for Preclinical Parkinson's diseaseR01NS057567 · NINDS · UNIVERSITY OF WASHINGTON · PI ZHANG, JING · 2008 to 2012
$3.1M
PIB PET Scanning in Speech and Language Based DementiasR01DC010367 · NIDCD · MAYO CLINIC ROCHESTER · PI JOSEPHS, KEITH A · 2010 to 2014
$1.7M
NIA NIH HHS P01-AG003949NIA NIH HHS P50-AG016574NIA NIH HHS R01-AG037491NIDCD NIH HHS R01-DC010367NIDCD NIH HHS R01-DC012519NIEHS NIH HHS U54-ES012077NINDS NIH HHS P50-NS072187NINDS NIH HHS R01-NS057567
6 · The paper itself

Abstract

objectiveTo determine ways to improve diagnostic accuracy of multiple system atrophy (MSA), we assessed the diagnostic process in patients who came to autopsy with antemortem diagnosis of MSA by comparing clinical and pathologic features between those who proved to have MSA and those who did not. We focus on likely explanations for misdiagnosis.

methodsThis is a retrospective review of 134 consecutive patients with an antemortem clinical diagnosis of MSA who came to autopsy with neuropathologic evaluation of the brain. Of the 134 patients, 125 had adequate medical records for review. Clinical and pathologic features were compared between patients with autopsy-confirmed MSA and those with other pathologic diagnoses, including dementia with Lewy bodies (DLB), Parkinson disease (PD), and progressive supranuclear palsy (PSP).

resultsOf the 134 patients with clinically diagnosed MSA, 83 (62%) had the correct diagnosis at autopsy. Pathologically confirmed DLB was the most common misdiagnosis, followed by PSP and PD. Despite meeting pathologic criteria for intermediate to high likelihood of DLB, several patients with DLB did not have dementia and none had significant Alzheimer-type pathology. Autonomic failure was the leading cause of misdiagnosis in DLB and PD, and cerebellar ataxia was the leading cause of misdiagnosis in PSP.

conclusionsThe diagnostic accuracy for MSA was suboptimal in this autopsy study. Pathologically confirmed DLB, PD, and PSP were the most common diseases to masquerade as MSA. This has significant implications not only for patient care, but also for research studies in MSA cases that do not have pathologic confirmation.

Indexed as

AgedAged, 80 and overAutopsyBrainDiagnosis, DifferentialFemaleHumansLewy Body DiseaseMaleMiddle AgedMultiple System AtrophyParkinson DiseaseRetrospective StudiesSupranuclear Palsy, Progressive

Identifiers

PMID26138942
PMCPMC4534078

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