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Real-world application of the updated diagnostic criteria for paraneoplastic neurological syndromes

Published:September 21, 2022DOI:https://doi.org/10.1016/j.jneuroim.2022.577972

      Abstract

      Following recent discoveries, diagnostic criteria for paraneoplastic neurological syndromes (PNS) have been recently updated. However, how the criteria impact PNS diagnosis is still unclear. We retrospectively applied the previously existing 2004 criteria (2004-c) and the updated 2021 diagnostic criteria (2021-c) to 74 patients with suspect PNS. The 2021 criteria were highly sensitive (88%) and specific (80%). There was good concordance between the definite PNS group (2004-c) and the definite plus probable PNS group (2021-c). The inter-rater reliability for the 2021-c was excellent. The application of the 2021 criteria improves the diagnosis of patients with PNS.

      Graphical abstract

      Keywords

      Abbreviations:

      IgG (Immunoglobulin G), NMDAR (N-methyl-d-aspartate receptor), LGI1 (Leucine-rich glioma-inactivated protein 1), CASPR2 (Contactin-associated protein 2), AMPAR (α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid receptor), GABAAR and GABABR (Gamma-aminobutyric acid receptor type A and B), mGluR1 and mGluR5 (Metabotropic glutamate receptor 1 and 5), GFAP (Glial fibrillary acidic protein), GAD65 (Glutamic acid decarboxylase 65), SOX1 (Sry-like high mobility group box 1), VGCC (P/Q-type voltage-gated calcium channels), PCA2/MAP1B (Purkinje cell antibody type-2/microtubule-associated protein 1B), Tr/DNER (Tr/ Delta/Notch-like epidermal growth factor-related receptor)
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