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Epidemiology of paraneoplastic neurological syndromes: a population-based study

Journal of Neurology volume 267, pages 26–35 (2020)Cite this article

Abstract

Background

The epidemiology of paraneoplastic neurological syndromes (PNS) remains to be defined. We present here the first population-based incidence study and report the clinical spectrum and antibody profile of PNS in a large area in Northeastern Italy.

Methods

We performed a 9-year (2009–2017) population-based epidemiological study of PNS in the provinces of Udine, Pordenone and Gorizia, in the Friuli-Venezia Giulia region (983,190 people as of January 1, 2017). PNS diagnosis and subgroups were defined by the 2004 diagnostic criteria. Age- and sex-adjusted incidence rates were calculated.

Results

We identified 89 patients with a diagnosis of definite PNS. Median age was 68 years (range 26–90), 52% were female. The incidence of PNS was 0.89/100,000 person-years. PNS incidence rates increased over time from 0.62/100,000 person-years (2009–2011), 0.81/100,000 person-years (2012–2014) to 1.22/100,000 person-years (2015–2017). The prevalence of PNS was 4.37 per 100,000. Most common PNS were limbic encephalitis (31%), cerebellar degeneration (28%) and encephalomyelitis (20%). Among antibody (Ab)-positive cases, most frequent specificities included: Yo (30%), Hu (26%), and Ma2 (22%), while the most frequent associated tumors were lung (17%) and breast cancer (16%), followed by lymphoma (12%). PNS developed in 1 in every 334 cancers in our region. Statistically significant associations were observed between cancer type and Ab-specificity (P < 0.001), and between neurological syndrome and Ab-specificity (P < 0.001).

Conclusions

This first population-based study found an incidence of PNS that approximates 1/100,000 person-years and a prevalence of 4/100,000. Moreover, the incidence of PNS is increasing over time, probably due to increased awareness and improved detection techniques.

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Data access, responsibility, and analysis

The corresponding author had full access to all the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis.

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Funding

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Author information

Authors and Affiliations

  1. Clinical Neurology Unit, Santa Maria della Misericordia University Hospital, Piazzale Santa Maria della Misericordia, 15, 33010, Udine, Italy

    Alberto Vogrig, Gian Luigi Gigli & Mariarosaria Valente

  2. French Reference Center of Paraneoplastic Neurological Syndrome, Hospices Civils de Lyon, Hôpital Neurologique, Lyon, France

    Alberto Vogrig & Jérôme Honnorat

  3. SynatAc Team, NeuroMyoGene Institute, INSERM U1217/CNRS UMR5310, Lyon, France

    Alberto Vogrig & Jérôme Honnorat

  4. University Claude Bernard Lyon 1, Université de Lyon, Lyon, France

    Alberto Vogrig & Jérôme Honnorat

  5. Department of Medicine (DAME), University of Udine Medical School, Udine, Italy

    Gian Luigi Gigli, Samantha Segatti, Elisa Corazza, Alessandro Marini, Andrea Bernardini, Francesco Curcio & Mariarosaria Valente

  6. Department of Mathematics, Informatics and Physics (DMIF), University of Udine, Udine, Italy

    Gian Luigi Gigli

  7. Institute of Hygiene and Clinical Epidemiology, Santa Maria della Misericordia University Hospital, Udine, Italy

    Francesca Valent

  8. Department of Laboratory Medicine, Institute of Clinical Pathology, Santa Maria della Misericordia University Hospital, Udine, Italy

    Martina Fabris & Francesco Curcio

  9. Department of Neuroscience, Biomedicine and Movement Sciences, University of Verona, Verona, Italy

    Francesco Brigo

  10. Department of Neurology, Hospital Franz Tappeiner, Merano, Italy

    Francesco Brigo

  11. Department of Oncology, Santa Maria della Misericordia University Hospital, Udine, Italy

    Donatella Iacono

  12. Neurology Unit, Hospital of Pordenone, Pordenone, Italy

    Paolo Passadore

  13. Neurology Unit, Hospital of Gorizia, Gorizia, Italy

    Michele Rana

Authors
  1. Alberto Vogrig
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  2. Gian Luigi Gigli
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  7. Francesca Valent
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  9. Francesco Curcio
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  14. Jérôme Honnorat
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  15. Mariarosaria Valente
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Contributions

Study concept and design: AV. Acquisition of data: AV, SS, EC, AM, AB, MF, PP and MR. Analysis and interpretation of data: AV, GLG, SS, EC, AM, AB, FV, MF, FC, FB, DI, PP, MR, JH and MV. Drafting of the manuscript: AV. Critical revision of the manuscript for important intellectual content: AV, GLG, SS, EC, AM, AB, FV, MF, FC, FB, DI, PP, MR, JH and MV. Study supervision: GLG and MV. All authors read and approved the final manuscript.

Corresponding author

Correspondence to Alberto Vogrig.

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The authors declare that they have no conflict of interest.

Ethical standards

All procedures were performed in accordance with the institutional ethics committee and the Declaration of Helsinki.

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Vogrig, A., Gigli, G.L., Segatti, S. et al. Epidemiology of paraneoplastic neurological syndromes: a population-based study. J Neurol 267, 26–35 (2020). https://doi.org/10.1007/s00415-019-09544-1

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  • DOI: https://doi.org/10.1007/s00415-019-09544-1

Keywords

  • Paraneoplastic syndromes
  • Epidemiology
  • Incidence
  • Prevalence
  • Encephalitis
  • Autoimmune