INDIVIDUAL TEST 835
CASPR2 Antibodies (IgG)
Indication
Suspicion of autoimmune encephalitis and Morvan’s Syndrome
Sample material
Serum
- Minim. volume: 0.5 mL
CSF
- Minim. volume: 0.5 mL
Transport
Within Sweden
- room temperature
International
- cold
Method
Cell-based assay (CBA), Indirect immunofluorescence (IIF)
Reference interval
Serum
- <1:10 negative
CSF
- negative
Result
Serum
- results are reported as negative, borderline, or positive. A positive result is followed by a titer.
CSF
- results are reported as negative or positive.
Interpretation
CASPR2 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 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.
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 a 2 or 0 point PNS score, depending on the clinical phenotype, according to Updated Diagnostic Criteria for Paraneoplastic Neurologic Syndromes.
References
- Leypoldt F et al. Ann N Y Acad Sci. 2015. Autoimmune encephalopathies. PMID: 25315420
- Dalmau J et al. Physiol Rev. 2017. Autoantibodies to Synaptic Receptors and Neuronal Cell Surface Proteins in Autoimmune Diseases of the Central Nervous System. PMID: 28298428
- Dalmau J et al. Lancet Neurol. 2019. An update on anti-NMDA receptor encephalitis for neurologists and psychiatrists: mechanisms and models. PMID: 31326280
- Graus F et al. Neurol Neuroimmunol Neuroinflamm. 2021. Updated Diagnostic Criteria for Paraneoplastic Neurologic Syndromes. PMID: 34006622
- Flanagan EP et al. JAMA Neurol. 2023. Autoimmune Encephalitis Misdiagnosis in Adults. PMID: 36441519
Last updated: 2024-08-12
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ENSKILD ANALYS 835
CASPR2-antikroppar (IgG)
Indikation
Misstanke om autoimmun encefalit och Morvan-Syndrom
Provmaterial
Serum
- Minim. volym: 0,5 mL
Likvor
- Minim. volym: 0,5 mL
Transport
Inom Sverige
- rumstemperatur
Internationellt
- kylt
Metod
Cell-based assay (CBA), indirekt immunofluorescens (IIF)
Referensintervall
Serum
- <1:10 negativt
Likvor
- negativt
Resultat
Serum
- resultat anges som negativt, gränsvärde eller positivt. Positivt resultat titreras.
Likvor
- resultat anges som negativt eller positivt.
Tolkning
CASPR2 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.
Antikroppar riktade mot CASPR2 bedöms vara Intermediate- eller Lower risk-antibodies med en förekomst på 50% eller respektive <30% 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
- Leypoldt F et al. Ann N Y Acad Sci. 2015. Autoimmune encephalopathies. PMID: 25315420
- Dalmau J et al. Physiol Rev. 2017. Autoantibodies to Synaptic Receptors and Neuronal Cell Surface Proteins in Autoimmune Diseases of the Central Nervous System. PMID: 28298428
- Dalmau J et al. Lancet Neurol. 2019. An update on anti-NMDA receptor encephalitis for neurologists and psychiatrists: mechanisms and models. PMID: 31326280
- Graus F et al. Neurol Neuroimmunol Neuroinflamm. 2021. Updated Diagnostic Criteria for Paraneoplastic Neurologic Syndromes. PMID: 34006622
- Flanagan EP et al. JAMA Neurol. 2023. Autoimmune Encephalitis Misdiagnosis in Adults. PMID: 36441519
Senast uppdaterat: 2024-08-12