Journal Article

Epidemiology of paraneoplastic neurological syndromes: a population-based study

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

2020

Overview

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.

• Publisher: Springer Berlin Heidelberg,Springer Nature B.V
• Subject: Adult / Aged / Aged, 80 and over / Autoimmune diseases / Breast cancer / Cerebellum / Degeneration / Diagnosis / Encephalitis / Encephalomyelitis / Encephalomyelitis - epidemiology / Encephalomyelitis - immunology / Epidemiology / Female / Health risk assessment / Humans / Incidence / Italy - epidemiology / Limbic Encephalitis - epidemiology / Limbic Encephalitis - immunology / Lung cancer / Lymphoma / Male / Medicine / Medicine & Public Health / Middle Aged / Neoplasms - epidemiology / Neurology / Neuroradiology / Neurosciences / Original Communication / Paraneoplastic Cerebellar Degeneration - epidemiology / Paraneoplastic Cerebellar Degeneration - immunology / Paraneoplastic Syndromes, Nervous System - epidemiology / Paraneoplastic Syndromes, Nervous System - immunology / Population / Population studies / Population-based studies / Prevalence / Statistical analysis / Tumors