RT Journal Article
SR Electronic
T1 Epidemiology of paraneoplastic neurologic syndromes and autoimmune encephalitides in France
JF Neurology - Neuroimmunology Neuroinflammation
JO Neurol Neuroimmunol Neuroinflamm
FD Lippincott Williams & Wilkins
SP e883
DO 10.1212/NXI.0000000000000883
VO 7
IS 6
A1 Julien Hébert
A1 Benjamin Riche
A1 Alberto Vogrig
A1 Sergio Muñiz-Castrillo
A1 Bastien Joubert
A1 Géraldine Picard
A1 Véronique Rogemond
A1 Dimitri Psimaras
A1 Agusti Alentorn
A1 Giulia Berzero
A1 Virginie Desestret
A1 Muriel Rabilloud
A1 Jérôme Honnorat
YR 2020
UL http://nn.neurology.org/content/7/6/e883.abstract
AB Objective To determine the observed and expected incidence rates of paraneoplastic neurologic syndromes (PNSs) and autoimmune encephalitides (AEs) diagnosed in France between 2016 and 2018, we conducted a population-based epidemiologic study.Methods Observed incidence rates were stratified by sex, age groups, region of care, year of diagnosis, and disease subgroups. National expected incidence rates were calculated based on rates obtained in the area directly adjacent to the Reference Center using a mixed Poisson model and compared with observed incidence rates.Results Six hundred thirty-two patients with definite PNS or AE met the inclusion criteria. The observed incidence rate of definite PNS and AE in France was 3.2 per million person-years (CI95%: 2.9–3.4) compared with an expected incidence rate of 7.1 per million person-years (CI95%: 3.9–11.4). The national observed incidence rate for the antibody-positive AE subgroup increased from 1.4 per million person-years (CI95%: 1.2–1.7) in 2016 to 2.1 per million person-years (CI95%: 1.7–2.4) in 2018, thus surpassing the incidence rate of classical PNS (1.2 per million person-years [CI95%: 1.0–1.5]) of 2018.Conclusions There was a significant widespread year-to-year increase in the incidence of diagnoses registered with the Reference Center for all subgroups of PNS and AE studied. The national observed incidence rate is likely underestimated due to underdiagnosis and underreporting.AE=autoimmune encephalitides; CRC=colorectal cancer; LEMS=Lambert-Eaton myasthenic syndrome; PNS=paraneoplastic neurologic syndrome; SCLC=small-cell lung cancer