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March 2022; 9 (2) ArticleOpen Access

Population-Based Epidemiology Study of Paraneoplastic Neurologic Syndromes

View ORCID ProfileShailee Shah, Eoin P. Flanagan, View ORCID ProfilePritikanta Paul, Carin Y. Smith, Sandra C. Bryant, Michelle F. Devine, Vanda A. Lennon, Andrew McKeon, Sean J. Pittock, Divyanshu Dubey
First published December 22, 2021, DOI: https://doi.org/10.1212/NXI.0000000000001124
Shailee Shah
From the Department of Neurology (S.S., E.P.F., P.P., M.F.D., V.A.L., A.M., S.J.P., D.D.), Mayo Clinic; Department of Laboratory Medicine and Pathology (E.P.F., V.A.L., A.M., S.J.P., D.D.), Mayo Clinic College of Medicine; Department of Quantitative Health Sciences (C.Y.S., S.C.B.), Mayo Clinic; Olmsted Medical Center (M.F.D.); and Department of Immunology (V.A.L.), Mayo Clinic, Rochester, MN.
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  • ORCID record for Shailee Shah
  • For correspondence: shah.shailee@mayo.edu
Eoin P. Flanagan
From the Department of Neurology (S.S., E.P.F., P.P., M.F.D., V.A.L., A.M., S.J.P., D.D.), Mayo Clinic; Department of Laboratory Medicine and Pathology (E.P.F., V.A.L., A.M., S.J.P., D.D.), Mayo Clinic College of Medicine; Department of Quantitative Health Sciences (C.Y.S., S.C.B.), Mayo Clinic; Olmsted Medical Center (M.F.D.); and Department of Immunology (V.A.L.), Mayo Clinic, Rochester, MN.
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  • For correspondence: flanagan.eoin@mayo.edu
Pritikanta Paul
From the Department of Neurology (S.S., E.P.F., P.P., M.F.D., V.A.L., A.M., S.J.P., D.D.), Mayo Clinic; Department of Laboratory Medicine and Pathology (E.P.F., V.A.L., A.M., S.J.P., D.D.), Mayo Clinic College of Medicine; Department of Quantitative Health Sciences (C.Y.S., S.C.B.), Mayo Clinic; Olmsted Medical Center (M.F.D.); and Department of Immunology (V.A.L.), Mayo Clinic, Rochester, MN.
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Carin Y. Smith
From the Department of Neurology (S.S., E.P.F., P.P., M.F.D., V.A.L., A.M., S.J.P., D.D.), Mayo Clinic; Department of Laboratory Medicine and Pathology (E.P.F., V.A.L., A.M., S.J.P., D.D.), Mayo Clinic College of Medicine; Department of Quantitative Health Sciences (C.Y.S., S.C.B.), Mayo Clinic; Olmsted Medical Center (M.F.D.); and Department of Immunology (V.A.L.), Mayo Clinic, Rochester, MN.
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  • For correspondence: smith.carin@mayo.edu
Sandra C. Bryant
From the Department of Neurology (S.S., E.P.F., P.P., M.F.D., V.A.L., A.M., S.J.P., D.D.), Mayo Clinic; Department of Laboratory Medicine and Pathology (E.P.F., V.A.L., A.M., S.J.P., D.D.), Mayo Clinic College of Medicine; Department of Quantitative Health Sciences (C.Y.S., S.C.B.), Mayo Clinic; Olmsted Medical Center (M.F.D.); and Department of Immunology (V.A.L.), Mayo Clinic, Rochester, MN.
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  • For correspondence: bryant@mayo.edu
Michelle F. Devine
From the Department of Neurology (S.S., E.P.F., P.P., M.F.D., V.A.L., A.M., S.J.P., D.D.), Mayo Clinic; Department of Laboratory Medicine and Pathology (E.P.F., V.A.L., A.M., S.J.P., D.D.), Mayo Clinic College of Medicine; Department of Quantitative Health Sciences (C.Y.S., S.C.B.), Mayo Clinic; Olmsted Medical Center (M.F.D.); and Department of Immunology (V.A.L.), Mayo Clinic, Rochester, MN.
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  • For correspondence: devine.michelle@mayo.edu
Vanda A. Lennon
From the Department of Neurology (S.S., E.P.F., P.P., M.F.D., V.A.L., A.M., S.J.P., D.D.), Mayo Clinic; Department of Laboratory Medicine and Pathology (E.P.F., V.A.L., A.M., S.J.P., D.D.), Mayo Clinic College of Medicine; Department of Quantitative Health Sciences (C.Y.S., S.C.B.), Mayo Clinic; Olmsted Medical Center (M.F.D.); and Department of Immunology (V.A.L.), Mayo Clinic, Rochester, MN.
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  • For correspondence: lennon.vanda@mayo.edu
Andrew McKeon
From the Department of Neurology (S.S., E.P.F., P.P., M.F.D., V.A.L., A.M., S.J.P., D.D.), Mayo Clinic; Department of Laboratory Medicine and Pathology (E.P.F., V.A.L., A.M., S.J.P., D.D.), Mayo Clinic College of Medicine; Department of Quantitative Health Sciences (C.Y.S., S.C.B.), Mayo Clinic; Olmsted Medical Center (M.F.D.); and Department of Immunology (V.A.L.), Mayo Clinic, Rochester, MN.
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  • For correspondence: mckeon.andrew@mayo.edu
Sean J. Pittock
From the Department of Neurology (S.S., E.P.F., P.P., M.F.D., V.A.L., A.M., S.J.P., D.D.), Mayo Clinic; Department of Laboratory Medicine and Pathology (E.P.F., V.A.L., A.M., S.J.P., D.D.), Mayo Clinic College of Medicine; Department of Quantitative Health Sciences (C.Y.S., S.C.B.), Mayo Clinic; Olmsted Medical Center (M.F.D.); and Department of Immunology (V.A.L.), Mayo Clinic, Rochester, MN.
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  • For correspondence: pittock.sean@mayo.edu
Divyanshu Dubey
From the Department of Neurology (S.S., E.P.F., P.P., M.F.D., V.A.L., A.M., S.J.P., D.D.), Mayo Clinic; Department of Laboratory Medicine and Pathology (E.P.F., V.A.L., A.M., S.J.P., D.D.), Mayo Clinic College of Medicine; Department of Quantitative Health Sciences (C.Y.S., S.C.B.), Mayo Clinic; Olmsted Medical Center (M.F.D.); and Department of Immunology (V.A.L.), Mayo Clinic, Rochester, MN.
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  • For correspondence: dubey.divyanshu@mayo.edu
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Citation
Population-Based Epidemiology Study of Paraneoplastic Neurologic Syndromes
Shailee Shah, Eoin P. Flanagan, Pritikanta Paul, Carin Y. Smith, Sandra C. Bryant, Michelle F. Devine, Vanda A. Lennon, Andrew McKeon, Sean J. Pittock, Divyanshu Dubey
Neurol Neuroimmunol Neuroinflamm Mar 2022, 9 (2) e1124; DOI: 10.1212/NXI.0000000000001124

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Abstract

Objectives Population-based epidemiologic data for paraneoplastic neurologic syndromes (PNSs) in the United States are lacking. Our objective was to evaluate the incidence, prevalence, and associated morbidity of PNS.

Methods We performed a population-based epidemiology study in Olmsted County, Minnesota, with patients identified between January 1, 1987, and December 31, 2018, using the medical records linkage system of the Rochester Epidemiology Project (REP) who met the definite/probable 2021 PNS criteria and 2004 PNS criteria. Patients with dermatomyositis and myasthenia gravis with underlying tumors were included. Age- and sex-specific population counts were obtained from REP resources for January 1, 2014 (prevalence denominator) and annually for 1987–2018 (incidence denominator). Morbidity was estimated using disability-adjusted life years (DALYs; years lived with disability [YLD] plus years of life lost [YLL]).

Results There were 28 patients with PNS identified (50% female) residing in Olmsted County, Minnesota, with median age at diagnosis of 54.5 (IQR 46.5–69.0) years. All patients had a cancer diagnosis, and 18 (64%) patients were neural autoantibody positive including antineuronal nuclear autoantibody type 1 (ANNA-1/anti-Hu; n = 1), ANNA-2/anti-Ri (n = 1), muscle-type acetylcholine receptor (AChR; n = 6), Purkinje cell cytoplasmic antibody type 1 (PCA-1/anti-Yo; n = 1), kelch-like protein 11 (KLH11; n = 3), collapsin response mediator protein 5 (CRMP-5/anti-CV2; n = 2), α-amino-3-hydroxy-5-methyl-4-isoxazole propionic acid receptor (n = 1), neurofilament light chain (n = 1), leucine zipper 4 (LUZP4; n = 1), and unclassified neural antibodies (n = 1). PNS incidence was 0.6/100,000 person-years and increased over time from 0.4/100,000 person-years (1987–2002) to 0.8/100,000 person-years (2003–2018) (p = 0.06). Prevalence was 5.4/100,000 people. The median follow-up period after PNS diagnosis was 3.1 years (IQR, 1.1–9.9 years). Total disability-adjusted life years (DALYs) for 28 patients with PNS were 472.7 years, based on total years of life lost (YLL) for patients dying between 1987 and 2018 (n = 15) of 445.3 years plus years lived with disability (YLD) 27.4 years.

Discussion PNSs are rare neurologic disorders but are associated with severe morbidity and mortality. The estimated number of prevalent PNS cases in the United States is 17,099, and predicted DALY for all US PNS cases is 292,393 years. Their apparent increasing rate of detection is attributable to increasing physician awareness and availability of serologic testing.

Glossary

DALY=
disability-adjusted life year;
GBD=
Global Burden of Disease;
LUZP4=
leucine zipper 4;
PNS=
paraneoplastic neurologic syndrome;
REP=
Rochester Epidemiology Project;
YLD=
years lived with disability;
YLL=
years of life lost

Footnotes

  • Go to Neurology.org/NN for full disclosures. Funding information is provided at the end of the article.

  • The Article Processing Charge was funded by the authors.

  • Received August 17, 2021.
  • Accepted in final form November 15, 2021.
  • Copyright © 2021 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.

This is an open access article distributed under the terms of the Creative Commons Attribution-NonCommercial-NoDerivatives License 4.0 (CC BY-NC-ND), which permits downloading and sharing the work provided it is properly cited. The work cannot be changed in any way or used commercially without permission from the journal.

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