Epidemiology of paraneoplastic neurological syndromes: a population-based study



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.

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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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Author information

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.

Correspondence to Alberto Vogrig.

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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) doi:10.1007/s00415-019-09544-1

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