Evidence map›Paper›PMID 39321395›Full record

ArticleNeurology(R) neuroimmunology & neuroinflammation2024

Comprehensive Analysis of Paraneoplastic Neurologic Syndrome and PNS-CARE Diagnostic Criteria in Clinical Practice.

Hannah Zhao-Fleming, Mohamed Rezk, Shailee Shah, Pranjal Gupta, Anastasia Zekeridou, Eoin P Flanagan, Sean J Pittock, Andrew McKeon, Divyanshu Dubey

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In one paragraph

Article in Neurology(R) neuroimmunology & neuroinflammation, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 9 papers, 1 of them a synthesis that pooled it.

0numbers the graph read from it
0cells of the map it votes in
9citing papers in PubMed, 1 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.

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

9 citing papers in PubMed, 1 synthesis or guideline pooled it.

  1. Pooled it
  2. Article
  3. Review
  4. Observational
  5. Review
  6. Article
  7. Article
  8. Article
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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.

Hannah Zhao-FlemingFrom the Departments of Neurology (H.Z.-F., M.R., P.G., A.Z., E.P.F., S.J.P., A.M., D.D.) and Laboratory Medicine and Pathology (M.R., P.G., A.Z., E.P.F., S.J.P., A.M., D.D.), Mayo Clinic, Rochester, MN; and Department of Neurology (S.S.), Vanderbilt University, Nashville, TN.ORCID 0009-0000-8695-4152
Mohamed RezkFrom the Departments of Neurology (H.Z.-F., M.R., P.G., A.Z., E.P.F., S.J.P., A.M., D.D.) and Laboratory Medicine and Pathology (M.R., P.G., A.Z., E.P.F., S.J.P., A.M., D.D.), Mayo Clinic, Rochester, MN; and Department of Neurology (S.S.), Vanderbilt University, Nashville, TN.
Shailee ShahFrom the Departments of Neurology (H.Z.-F., M.R., P.G., A.Z., E.P.F., S.J.P., A.M., D.D.) and Laboratory Medicine and Pathology (M.R., P.G., A.Z., E.P.F., S.J.P., A.M., D.D.), Mayo Clinic, Rochester, MN; and Department of Neurology (S.S.), Vanderbilt University, Nashville, TN.ORCID 0000-0002-3451-0303
Pranjal GuptaFrom the Departments of Neurology (H.Z.-F., M.R., P.G., A.Z., E.P.F., S.J.P., A.M., D.D.) and Laboratory Medicine and Pathology (M.R., P.G., A.Z., E.P.F., S.J.P., A.M., D.D.), Mayo Clinic, Rochester, MN; and Department of Neurology (S.S.), Vanderbilt University, Nashville, TN.ORCID 0009-0000-8482-8259
Anastasia ZekeridouFrom the Departments of Neurology (H.Z.-F., M.R., P.G., A.Z., E.P.F., S.J.P., A.M., D.D.) and Laboratory Medicine and Pathology (M.R., P.G., A.Z., E.P.F., S.J.P., A.M., D.D.), Mayo Clinic, Rochester, MN; and Department of Neurology (S.S.), Vanderbilt University, Nashville, TN.ORCID 0000-0002-1960-9065
Eoin P FlanaganFrom the Departments of Neurology (H.Z.-F., M.R., P.G., A.Z., E.P.F., S.J.P., A.M., D.D.) and Laboratory Medicine and Pathology (M.R., P.G., A.Z., E.P.F., S.J.P., A.M., D.D.), Mayo Clinic, Rochester, MN; and Department of Neurology (S.S.), Vanderbilt University, Nashville, TN.ORCID 0000-0002-6661-2910
Sean J PittockFrom the Departments of Neurology (H.Z.-F., M.R., P.G., A.Z., E.P.F., S.J.P., A.M., D.D.) and Laboratory Medicine and Pathology (M.R., P.G., A.Z., E.P.F., S.J.P., A.M., D.D.), Mayo Clinic, Rochester, MN; and Department of Neurology (S.S.), Vanderbilt University, Nashville, TN.ORCID 0000-0002-6140-5584
Andrew McKeonFrom the Departments of Neurology (H.Z.-F., M.R., P.G., A.Z., E.P.F., S.J.P., A.M., D.D.) and Laboratory Medicine and Pathology (M.R., P.G., A.Z., E.P.F., S.J.P., A.M., D.D.), Mayo Clinic, Rochester, MN; and Department of Neurology (S.S.), Vanderbilt University, Nashville, TN.ORCID 0000-0001-6856-8143
Divyanshu DubeyFrom the Departments of Neurology (H.Z.-F., M.R., P.G., A.Z., E.P.F., S.J.P., A.M., D.D.) and Laboratory Medicine and Pathology (M.R., P.G., A.Z., E.P.F., S.J.P., A.M., D.D.), Mayo Clinic, Rochester, MN; and Department of Neurology (S.S.), Vanderbilt University, Nashville, TN.ORCID 0000-0001-6865-9045

Funding

A Phase-2b, Double-Blind, Randomized Controlled Trial to Evaluate the Activity and Safety of Inebilizumab in Anti-N-methyl-D-aspartate receptor (NMDAR) Encephalitis and Assess Markers of DiseaseU01NS120901 · NINDS · UTAH STATE HIGHER EDUCATION SYSTEM--UNIVERSITY OF UTAH · PI Stacey Lynn Clardy · 2021 to 2026
$19.6M
Utilizing epidemiology, imaging and fluid biomarkers in MOG antibody-associated disease to inform prognosis, pathogenesis and therapeuticsR01NS113828 · NINDS · MAYO CLINIC ROCHESTER · PI Eoin P Flanagan · 2020 to 2026
$2.4M
NIH HHS R01NS113828NIH HHS RO1NS126227NIH HHS U01NS120901
6 · The paper itself

Abstract

BACKGROUND AND

objectivesParaneoplastic neurologic syndrome (PNS) diagnostic criteria were first proposed in 2004 and updated in 2021. The PNS-CARE score, derived from the updated criteria, is a composite model for assigning likelihood for patients with suspected PNS. In this study, we evaluated the utility and applicability of the 2021 PNS-CARE score and present our PNS cohort.

methodsThis is a retrospective study. We identified Mayo Clinic patients suspected to have PNS (1/2005-12/2020) and collected relevant information including demographics, PNS presentation, and clinical outcomes. Inclusion criteria were the following: (1) patients with a syndrome consistent with PNS and (2) patients with sufficient information available in charts. Exclusion criteria were the following: (1) evaluation only before 2005, (2) patients not evaluated by neurology, (3) presentation after immune checkpoint inhibitors, and (4) syndromes not included in 2021 criteria. All patients were evaluated for the 2021 and 2004 PNS criteria.

resultsWe identified 484 patients suspected to have PNS at initial presentation, of whom 212 (44%) were considered to have PNS after completion of evaluation. Among these 212 patients, the most common autoantibodies were PCA1 (Yo)-IgG (17%), KLHL11-IgG (16%), and CRMP5-IgG (14%) and the most common phenotypes were rapidly progressive cerebellar syndrome (29%), brainstem encephalitis (14%), and limbic encephalitis (8%). The 2021 PNS criteria definite/probable categorization (PNS-CARE score ≥ 6) had a sensitivity and specificity of 93% and 100%, respectively, while the 2004 PNS criteria definite categorization had a sensitivity and specificity of 67% and 99%, respectively. We found 15 patients with a PNS-CARE score ≤5 who likely had PNS on our review. The most common presentation among these patients was KLHL11-IgG brainstem encephalitis (7/15, 47%) with likely burned-out testicular tumor. DISCUSSION: Our study validates the PNS-CARE score. A clearer understanding of typical PNS presentation and common underlying malignancies and autoantibodies can aid in earlier and more accurate diagnosis, which is crucial for downstream clinical decisions. Some patients with an intermediate-risk phenotype do not meet probable/definite criteria despite the presence of high-risk antibodies and/or underlying malignancy.

Indexed as

Paraneoplastic Syndromes, Nervous SystemAdultAgedAged, 80 and overAutoantibodiesFemaleHumansMaleMiddle AgedRetrospective StudiesAutoantibodies

Identifiers

PMID39321395
PMCPMC11443324

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