INDIVIDUAL TEST 833

LGI-1 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).InterpretationLGI-1 is a subunit in the potassium channel complex (VGKC). Antibodies against LGI-1 have been reported in classical autoimmune encephalitis and epilepsy. Antibodies against LGI-1 are the second most common in autoimmune encephalitis following antibodies against NMDAR. Antibodies against LG-1 are detected more often in men, age when diagnosed has varied between 30-80 years (median 60 years). LGI-1 antibodies are associated with tumors (lung, thymoma but also other tumor forms) in 25-31% of the cases.

The LGI-1 belongs 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.

References: Lancaster, 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, Huijbers, Lancaster, et al. Investigation of LGI1 as the antigen in limbic encephalitis previously attributed to potassium channels: a case series. Lancet Neurol 2010;9:776-785. PMID 20580615.

Irani SR, Alexander S, Waters P, et al. Antibodies to Kv1 potassium channelcomplex proteins leucine-rich, glioma inactivated 1 protein and contactin-associated protein-2 in limbic encephalitis, Morvan’s syndrome and acquired neuromyotonia. Brain 2010;133:2734-2748. PMID 20663977.

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Neurology

ENSKILD ANALYS 833

LGI-1 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).TolkningLGI-1 är en subenhet i kaliumkanalskomplexet (VGKC). Antikroppar mot LGI-1 har rapporterats förekomma vid klassisk autoimmun encefalit och epilepsi. Antikroppar mot LGI-1 anses vara de näst vanligast förekommande vid autoimmun encefalit efter antikroppar mot NMDAR. Autoantikroppar mot LG-1 påvisas något oftare hos män, ålder vid diagnos har varierat mellan 30-80 år (median 60 år). LGI-1 antikroppar är associerade med tumörer (lung, tymom men även andra tumörformer) i 25-31% av fallen.

LGI-1 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, Huijbers, Lancaster, et al. Investigation of LGI1 as the antigen in limbic encephalitis previously attributed to potassium channels: a case series. Lancet Neurol 2010;9:776-785. PMID 20580615.

Irani SR, Alexander S, Waters P, et al. Antibodies to Kv1 potassium channelcomplex proteins leucine-rich, glioma inactivated 1 protein and contactin-associated protein-2 in limbic encephalitis, Morvan’s syndrome and acquired neuromyotonia. Brain 2010;133:2734-2748. PMID 20663977.

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