Journal of Neurology

, Volume 267, Issue 1, pp 26–35 | Cite as

Epidemiology of paraneoplastic neurological syndromes: a population-based study

  • Alberto VogrigEmail author
  • Gian Luigi Gigli
  • Samantha Segatti
  • Elisa Corazza
  • Alessandro Marini
  • Andrea Bernardini
  • Francesca Valent
  • Martina Fabris
  • Francesco Curcio
  • Francesco Brigo
  • Donatella Iacono
  • Paolo Passadore
  • Michele Rana
  • Jérôme Honnorat
  • Mariarosaria Valente
Original Communication



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.


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.


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).


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.


Paraneoplastic syndromes Epidemiology Incidence Prevalence Encephalitis Autoimmune 


Author 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.


No targeted funding reported.

Compliance with ethical standards

Conflicts of interest

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.

Supplementary material

415_2019_9544_MOESM1_ESM.docx (19 kb)
Supplementary file1 (DOCX 18 kb)


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Copyright information

© Springer-Verlag GmbH Germany, part of Springer Nature 2019

Authors and Affiliations

  • Alberto Vogrig
    • 1
    • 2
    • 3
    • 4
    Email author
  • Gian Luigi Gigli
    • 1
    • 5
    • 6
  • Samantha Segatti
    • 5
  • Elisa Corazza
    • 5
  • Alessandro Marini
    • 5
  • Andrea Bernardini
    • 5
  • Francesca Valent
    • 7
  • Martina Fabris
    • 8
  • Francesco Curcio
    • 5
    • 8
  • Francesco Brigo
    • 9
    • 10
  • Donatella Iacono
    • 11
  • Paolo Passadore
    • 12
  • Michele Rana
    • 13
  • Jérôme Honnorat
    • 2
    • 3
    • 4
  • Mariarosaria Valente
    • 1
    • 5
  1. 1.Clinical Neurology UnitSanta Maria della Misericordia University HospitalUdineItaly
  2. 2.French Reference Center of Paraneoplastic Neurological SyndromeHospices Civils de Lyon, Hôpital NeurologiqueLyonFrance
  3. 3.SynatAc TeamNeuroMyoGene Institute, INSERM U1217/CNRS UMR5310LyonFrance
  4. 4.University Claude Bernard Lyon 1, Université de LyonLyonFrance
  5. 5.Department of Medicine (DAME)University of Udine Medical SchoolUdineItaly
  6. 6.Department of Mathematics, Informatics and Physics (DMIF)University of UdineUdineItaly
  7. 7.Institute of Hygiene and Clinical EpidemiologySanta Maria della Misericordia University HospitalUdineItaly
  8. 8.Department of Laboratory Medicine, Institute of Clinical PathologySanta Maria della Misericordia University HospitalUdineItaly
  9. 9.Department of Neuroscience, Biomedicine and Movement SciencesUniversity of VeronaVeronaItaly
  10. 10. Department of NeurologyHospital Franz TappeinerMeranoItaly
  11. 11.Department of OncologySanta Maria della Misericordia University HospitalUdineItaly
  12. 12.Neurology UnitHospital of PordenonePordenoneItaly
  13. 13.Neurology UnitHospital of GoriziaGoriziaItaly

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