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01-01-2020 | Paraneoplastic Syndromes | Original Communication

Epidemiology of paraneoplastic neurological syndromes: a population-based study

Authors: Alberto Vogrig, 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

Published in: Journal of Neurology | Issue 1/2020

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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Title: Epidemiology of paraneoplastic neurological syndromes: a population-based study
Authors: Alberto Vogrig
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
Publication date: 01-01-2020
Publisher: Springer Berlin Heidelberg
Keywords: Paraneoplastic Syndromes
Autoimmune Encephalitis
Encephalitis
Published in: Journal of Neurology / Issue 1/2020
Print ISSN: 0340-5354
Electronic ISSN: 1432-1459
DOI: https://doi.org/10.1007/s00415-019-09544-1

At a glance: The ONWARDS insulin icodec trials

Springer Medicine

Epidemiology of paraneoplastic neurological syndromes: a population-based study

Abstract

Background

Methods

Results

Conclusions

At a glance: The ONWARDS insulin icodec trials

Springer Medicine

Abstract

Background

Methods

Results

Conclusions

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