INDIVIDUAL TEST 772

AMPA receptor 1 and 2 antibodies

IndicationSuspicion of paraneoplastic syndromes particularly in autoimmune encephalitis.MethodIndirect immunofluorescence (IIF) using transfected cells.AnswerThe result is reported as positive or negative. A positive result is followed by a titer (serum).InterpretationAntibodies against AMPA 1/2 receptors can be seen in paraneoplastic syndrome and in classical autoimmune encephalitis, but cases of isolated psychoses have also been described. There are relatively few cases published, of which 90% have been women. Age at diagnosis has been 38-87 years (median 60 years). The antibodies have been associated with tumors (lung, breast and thymoma) in 70% of the cases. Antibodies may occur together with other autoantibodies in around 60% of the cases (antibodies against ANA, GAD-65, cardiolipin, VGCC, SOX1, CV2/CRMP5 and TPO).

The AMPA receptors belong to the group of extracellular antigens. Generally, immunotherapy is more often effective in autoantibodies directed against extracellular antigens than in autoantibodies against intracellular antigens. Antibody titers can be used to monitor treatment effectiveness. Screening for cancer diagnosis is recommended.ReferencesLancaster, Martinez-Hernandez, Dalmau. Encephalitis and antibodies to synaptic and neuronal cell surface proteins. Neurology. 2011 Jul 12;77(2):179-89.  PMID: 21747075.

Titulaera et al. Screening for tumours in paraneoplastic syndromes: report of an EFNS Task Force. European Journal of Neurology. Epublished 2010. Eur J Neurol. 2011 January; 18(1): 19–23. PMID: 20880069.

Zuliani, Graus, Giometto, Bien, Vincent. Central nervous system neuronal surface antibody associated syndromes: review and guidelines for recognition. J Neurol Neurosurg Psychiatry 2012;83:638-645. PMID: 22448032.

Lai, Hughes, Peng, et al. AMPA receptor antibodies in limbic encephalitis alter synaptic receptor location. Ann Neurol 2009;65:424-434. PMID 19338055.

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Neurology

ENSKILD ANALYS 772

AMPA receptor 1 & 2 antikroppar

IndikationMisstanke om paraneoplastiska syndrom och framför allt vid autoimmun encefalit.MetodIndirekt immunofluorescens (IIF) med transfekterade celler.SvarResultatet anges som positiv eller negativ. Positivt resultat titreras (serum).TolkningAntikroppar mot AMPA 1/2 receptorer kan ses vid paraneoplastiska syndrom och framför allt vid klassisk autoimmun encefalit men fall av isolerade psykoser har också beskrivits. Det finns ett fåtal fall publicerade varav 90% har varit kvinnor. Ålder vid diagnos har varit 38-87 år (median 60 år). Antikropparna har varit associerade med tumörer (lung, bröst och tymom) i 70% av fallen. Antikropparna kan förekomma tillsammans med andra autoantikroppar i runt 60% av fallen (antikroppar mot ANA, GAD-65, kardiolipin, VGCC, SOX1, CV2/CRMP5 och TPO).

AMPA receptorerna räknas till gruppen extracellulära antigen. Generellt sett är behandling med immunoterapi oftare effektiv vid autoantikroppar riktade mot extracellulära antigen än vid autoantikroppar mot intracellulära antigen. Antikroppstitrar kan användas för att följa behandlingseffektivitet. Uppföljning med cancerdiagnostik rekommenderas.

ReferenserLancaster, Martinez-Hernandez, Dalmau. Encephalitis and antibodies to synaptic and neuronal cell surface proteins. Neurology. 2011 Jul 12;77(2):179-89.  PMID: 21747075.

Titulaera et al. Screening for tumours in paraneoplastic syndromes: report of an EFNS Task Force. European Journal of Neurology. Epublished 2010. Eur J Neurol. 2011 January; 18(1): 19–23. PMID: 20880069.

Zuliani, Graus, Giometto, Bien, Vincent. Central nervous system neuronal surface antibody associated syndromes: review and guidelines for recognition. J Neurol Neurosurg Psychiatry 2012;83:638-645. PMID: 22448032.

Lai, Hughes, Peng, et al. AMPA receptor antibodies in limbic encephalitis alter synaptic receptor location. Ann Neurol 2009;65:424-434. PMID 19338055.

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