Anti–CASPR2 Antibodies
Test details
Paraneoplastic neurological syndromes (PNS) are immune-mediated neurological disorders that can affect any part of the nervous system, occur in association with cancer and are often supported by the presence of specific neuronal autoantibodies.
Neuronal autoantibodies are classified as high-, intermediate-, or low-risk for paraneoplastic aetiology. Cancer association is 30–70% for intermediate-risk antibodies and <30% for low-risk ones. In general, intermediate- and low-risk antibodies are pathogenic and patients often respond well to immunosuppressive therapy.
Autoantibodies against contactin-associated protein 2 (CASPR2) are markers of autoimmune neuronal disease. They are considered intermediate risk in patients with Morvan syndrome, where association with malignant thymoma is about 50%. In patients with limbic encephalitis or Isaacs syndrome, anti-CASPR2 antibodies are considered low risk, with thymoma association <30%.
For neuronal autoantibodies with intermediate or low-risk phenotypes, testing should be performed in parallel on serum and cerebrospinal fluid.
Sample type
Serum, EDTA plasma, heparin plasma, citrate plasma, CSF
Method
IFA cells
Preparation
Fasting for at least 8-12 hours before sampling
Storage conditions
Refer to the Health Service Charter to check storage conditions
Shipping
+2/+8°C
References
Graus F, Vogrig A, Muñiz-Castrillo S, Antoine JG, Desestret V, Dubey D, Giometto B, Irani SR, Joubert B, Leypoldt F, McKeon A, Prüss H, Psimaras D, Thomas L, Titulaer MJ, Vedeler CA, Verschuuren JJ, Dalmau J, Honnorat J. Updated Diagnostic Criteria for Paraneoplastic Neurologic Syndromes. Neurol Neuroimmunol Neuroinflamm. 2021 May 18;8(4):e1014. doi: 10.1212/NXI.0000000000001014. PMID: 34006622; PMCID: PMC8237398.
Irani SR, Pettingill P, Kleopa KA, Schiza N, Waters P, Mazia C, Zuliani L, Watanabe O, Lang B, Buckley C, Vincent A. Morvan syndrome: clinical and serological observations in 29 cases. Ann Neurol. 2012 Aug;72(2):241-55. doi: 10.1002/ana.23577. Epub 2012 Apr 4. PMID: 22473710.
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