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. 2020 Aug 26;7(6):e883.
doi: 10.1212/NXI.0000000000000883. Print 2020 Nov.

Epidemiology of paraneoplastic neurologic syndromes and autoimmune encephalitides in France

Julien Hébert  1 , Benjamin Riche  1 , Alberto Vogrig  1 , Sergio Muñiz-Castrillo  1 , Bastien Joubert  1 , Géraldine Picard  1 , Véronique Rogemond  1 , Dimitri Psimaras  1 , Agusti Alentorn  1 , Giulia Berzero  1 , Virginie Desestret  1 , Muriel Rabilloud  1 , Jérôme Honnorat  2
Affiliations

Epidemiology of paraneoplastic neurologic syndromes and autoimmune encephalitides in France

Julien Hébert et al. Neurol Neuroimmunol Neuroinflamm. .

Abstract

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.

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Figures

Figure 1
Figure 1. Sex-age pyramids by subgroup
(A) Definite paraneoplastic neurologic syndromes and autoimmune encephalitides. (B) Paraneoplastic neurologic syndromes. (C) NMDAr-positive patients. (D). LGI1-positive patients.
Figure 2
Figure 2. Proportion of females by antibody type
Figure 3
Figure 3. Maps of relative incidence rates of definite paraneoplastic neurologic syndromes and autoimmune encephalitides in France for 2016–2018
See this image and copyright information in PMC

References

    1. Graus F, Delattre JY, Antoine JC, et al. . Recommended diagnostic criteria for paraneoplastic neurological syndromes. J Neurol Neurosurg Psychiatry 2004;75:1135–1140. - PMC - PubMed
    1. Graus F, Titulaer M, Ramani B, et al. . A clinical approach to diagnosis of autoimmune encephalitis. Lancet Neurol 2016;15:391–404. - PMC - PubMed
    1. Dalmau J, Gleichman AJ, Hughes EG, et al. . Anti-NMDA-receptor encephalitis: case series and analysis of the effect of antibodies. Lancet Neurol 2008;7:1091–1098. - PMC - PubMed
    1. Finke C, Kopp U, Pruss H, Dalmau J, Wandinger K, Ploner C. Cognitive deficits following anti-NMDA receptor encephalitis. J Neurol Neurosurg Psychiatry 2012;83:195–198. - PMC - PubMed
    1. Hébert J, Day GS, Steriade C, Wennberg RA, Tang-Wai DF. Long-Term cognitive outcomes in patients with autoimmune encephalitis. Can J Neurol Sci 2018;45:540–544. - PubMed

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