Alberto Vogrig1,2,3,4, Gian Luigi Gigli5,6,7, Samantha Segatti6, Elisa Corazza6, Alessandro Marini6, Andrea Bernardini6, Francesca Valent8, Martina Fabris9, Francesco Curcio6,9, Francesco Brigo10,11, Donatella Iacono12, Paolo Passadore13, Michele Rana14, Jérôme Honnorat15,16,17, Mariarosaria Valente5,6. 1. Clinical Neurology Unit, Santa Maria della Misericordia University Hospital, Piazzale Santa Maria della Misericordia, 15, 33010, Udine, Italy. alberto.vogrig@gmail.com. 2. French Reference Center of Paraneoplastic Neurological Syndrome, Hospices Civils de Lyon, Hôpital Neurologique, Lyon, France. alberto.vogrig@gmail.com. 3. SynatAc Team, NeuroMyoGene Institute, INSERM U1217/CNRS UMR5310, Lyon, France. alberto.vogrig@gmail.com. 4. University Claude Bernard Lyon 1, Université de Lyon, Lyon, France. alberto.vogrig@gmail.com. 5. Clinical Neurology Unit, Santa Maria della Misericordia University Hospital, Piazzale Santa Maria della Misericordia, 15, 33010, Udine, Italy. 6. Department of Medicine (DAME), University of Udine Medical School, Udine, Italy. 7. Department of Mathematics, Informatics and Physics (DMIF), University of Udine, Udine, Italy. 8. Institute of Hygiene and Clinical Epidemiology, Santa Maria della Misericordia University Hospital, Udine, Italy. 9. Department of Laboratory Medicine, Institute of Clinical Pathology, Santa Maria della Misericordia University Hospital, Udine, Italy. 10. Department of Neuroscience, Biomedicine and Movement Sciences, University of Verona, Verona, Italy. 11. Department of Neurology, Hospital Franz Tappeiner, Merano, Italy. 12. Department of Oncology, Santa Maria della Misericordia University Hospital, Udine, Italy. 13. Neurology Unit, Hospital of Pordenone, Pordenone, Italy. 14. Neurology Unit, Hospital of Gorizia, Gorizia, Italy. 15. French Reference Center of Paraneoplastic Neurological Syndrome, Hospices Civils de Lyon, Hôpital Neurologique, Lyon, France. 16. SynatAc Team, NeuroMyoGene Institute, INSERM U1217/CNRS UMR5310, Lyon, France. 17. University Claude Bernard Lyon 1, Université de Lyon, Lyon, France.
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.
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.
Authors: T H Popperud; M I Boldingh; C Brunborg; K W Faiz; A T Heldal; A H Maniaol; K I Müller; M Rasmussen; K Øymar; E Kerty Journal: Eur J Paediatr Neurol Date: 2016-09-10 Impact factor: 3.140
Authors: Francesc Graus; Alberto Vogrig; Sergio Muñiz-Castrillo; Jean-Christophe G Antoine; Virginie Desestret; Divyanshu Dubey; Bruno Giometto; Sarosh R Irani; Bastien Joubert; Frank Leypoldt; Andrew McKeon; Harald Prüss; Dimitri Psimaras; Laure Thomas; Maarten J Titulaer; Christian A Vedeler; Jan J Verschuuren; Josep Dalmau; Jerome Honnorat Journal: Neurol Neuroimmunol Neuroinflamm Date: 2021-05-18