INDIVIDUAL TEST 835
CASPR2 Antibodies (IgG)
Indication Suspicion of autoimmune encephalitis and Morvans Syndrome.Method Indirect immunofluorescence (IIF) using transfected cells.
Answer Serum: The result is reported as positive, borderline or negative. A positive result is followed by a titer.
CSF: The result is reported as positive or negative. CSF samples are not titrated.
Interpretation CASPR2 (Contactin Associated Protein 2) is a subunit of the potassium channel complex (VGKC) and is included in the group of extracellular antigens. Antibodies against CASPR2 may occur in neuromyotonia, autoimmune encephalitis and Morvan Syndrome. There are a relatively few cases published, where 85% have been men and the age of diagnosis has been 46-77 years (median 60 years). There is an association with malignancy, mainly thymoma. Antibody titers can be used to monitor treatment effectiveness. Generally, treatment with immunotherapy is more effective at autoantibodies directed against extracellular antigens than autoantibodies against intracellular antigens. Follow up with cancer diagnosis is recommended.
Antibodies against CASPR2 are graded as Intermediate- or Lower risk-antibodies with a frequency of respectively 50% or <30% of underlying cancer. A positive result yields 2 or 0 points PNS score, depending on the clinical phenotype, according to Updated Diagnostic Criteria for Paraneoplastic Neurologic Syndromes.
References
- Dalmau J, Geis C, Graus F. Autoantibodies to Synaptic Receptors and Neuronal Cell Surface Proteins in Autoimmune Diseases of the Central Nervous System. Physiol Rev. 2017 Apr;97(2):839-887. PMID: 28298428;
- Graus F, et al. Updated Diagnostic Criteria for Paraneoplastic Neurologic Syndromes. Neurol Neuroimmunol Neuroinflamm. 2021 May 18;8(4):e1014. PMID: 34006622
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ENSKILD ANALYS 835
CASPR2-antikroppar (IgG)
Indikation Misstanke om autoimmun encefalit och Morvan-Syndrom.Metod Indirekt immunofluorescens (IIF) med transfekterade celler.
Svar Serum: Resultatet anges som positiv, gränsvärde eller negativ. Positivt resultat titreras.
Likvor: Resultatet anges som positiv eller negativ. Likvorprover titreras ej.
Tolkning CASPR2 (contactin associated protein 2) är en subenhet i kaliumkanalskomplexet (VGKC) och räknas till gruppen extracellulära antigen. Antikroppar mot CASPR2 kan förekomma vid neuromyotoni, autoimmun encefalit och Morvan-Syndrom. Det finns ett relativt fåtal fall publicerade där 85% har varit män och ålder vid diagnos har varit 46-77 år (median 60 år). Det finns en association till malignitet, främst tymom. Antikroppstitrar kan användas för att följa behandlingseffektivitet. Generellt sett är behandling med immunoterapi oftare effektfull vid autoantikroppar riktade mot extracellulära antigen än vid autoantikroppar mot intracellulära antigen. Uppföljning med cancerdiagnostik rekommenderas.
Antikropparna riktade mot CASPR2 bedöms vara Intermediate- eller Lower risk-antibodies med en förekomst på 50% eller <30% respektive för en underliggande cancer. Positivitet ger 2 alternativt 0 poäng beroende på klinisk bild, i PNS score enligt Updated Diagnostic Criteria for Paraneoplastic Neurologic Syndromes.
Referenser
- Dalmau J, Geis C, Graus F. Autoantibodies to Synaptic Receptors and Neuronal Cell Surface Proteins in Autoimmune Diseases of the Central Nervous System. Physiol Rev. 2017 Apr;97(2):839-887. PMID: 28298428;
- Graus F, et al. Updated Diagnostic Criteria for Paraneoplastic Neurologic Syndromes. Neurol Neuroimmunol Neuroinflamm. 2021 May 18;8(4):e1014. PMID: 34006622