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Epidemiology of paraneoplastic neurologic syndromes and autoimmune encephalitides in France.

ABSTRACT: 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. 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. 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. 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.

SUBMITTER: Hebert J

PROVIDER: S-EPMC7455315 | biostudies-literature | 2020 Nov

REPOSITORIES: biostudies-literature

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Epidemiology of paraneoplastic neurologic syndromes and autoimmune encephalitides in France.

Hébert Julien J Riche Benjamin B Vogrig Alberto A Muñiz-Castrillo Sergio S Joubert Bastien B Picard Géraldine G Rogemond Véronique V Psimaras Dimitri D Alentorn Agusti A Berzero Giulia G Desestret Virginie V Rabilloud Muriel M Honnorat Jérôme J

Neurology(R) neuroimmunology & neuroinflammation 20200826 6

<h4>Objective</h4>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.<h4>Methods</h4>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 Refe ...[more]

PMID: 32847939

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Project description:Background and objectiveParaneoplastic neurological syndromes (PNS) are a group of rare syndromes associated with immunopathological process and tumors. Paraneoplastic autoantibodies are important for the diagnosis of PNS and for searching for underlying tumors. With the development of detection methods and discovery of new autoantibodies, the 2004 guidelines on PNS have recently been updated by a worldwide PNS-Care expert group. For clinicians, proper testing methods and testing results explanation are important for the diagnosis and treatment of PNS. This review aims to review the detection of paraneoplastic autoantibodies and the significance of testing results.MethodsWe summarize the studies on detection methods, association of autoantibodies and PNS or tumors, particularly the guidelines of PNS.Key content and findingsAntibodies are divided into 3 groups in the context of PNS according to the frequency of cancer association regardless of their eventual pathogenic effect. Instead of well-characterized antibodies and partially-characterized antibodies, high-risk antibodies, intermediate risk antibodies and lower risk antibodies were applied. According to the location of recognized antigens, these autoantibodies are divided as anti-intracellular antigen antibodies and neuronal surface antibodies (NSAbs). Tissue-based assays is recommended as screening method for paraneoplastic antibodies. Moreover, this method is helpful to discover new autoantibodies. A combination of a screening method [tissue-based assays (TBA)] and a confirmatory test [immunoblot and cell-based assay (CBA)] can improve sensitivity and specificity of the tests. Many PNSs are associated with specific antineuronal antibodies, but there is considerable diversity. Some autoantibodies are markers of specific neurological syndromes. Paraneoplastic antibodies are often specific for the PNS-associated tumor rather than for a particular neurological syndrome.ConclusionsDiagnosis of PNS depends on integrated analysis of clinical manifestations and auxiliary examinations. During diagnosis, selection of candidate antibodies for testing is challenging due to the varying clinical phenotypes and tumors associated with a given antibody. Broad antibody panels are more likely to capture causative antibodies and should be considered. According to different subtypes of autoantibodies, specific tumors or PNS should be considered. However, antibody titers, including cerebrospinal fluid (CSF) titers, should not be the primary driver of treatment decisions.

Dataset Information

Epidemiology of paraneoplastic neurologic syndromes and autoimmune encephalitides in France.

Publications

Epidemiology of paraneoplastic neurologic syndromes and autoimmune encephalitides in France.

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