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000268498 0247_ $$2doi$$a10.1016/j.bbi.2024.01.220
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000268498 1001_ $$0P:(DE-2719)9002282$$aArlt, Friederike A$$b0$$eFirst author$$udzne
000268498 245__ $$aKCNA2 IgG autoimmunity in neuropsychiatric diseases.
000268498 260__ $$aOrlando, Fla.$$bAcademic Press$$c2024
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000268498 520__ $$aAutoantibodies against the potassium voltage-gated channel subfamily A member 2 (KCNA2) have been described in a few cases of neuropsychiatric disorders, but their diagnostic and pathophysiological role is currently unknown, imposing challenges to medical practice.We retrospectively collected comprehensive clinical and paraclinical data of 35 patients with KCNA2 IgG autoantibodies detected in cell-based and tissue-based assays. Patients' sera and cerebrospinal fluid (CSF) were used for characterization of the antigen, clinical-serological correlations, and determination of IgG subclasses.KCNA2 autoantibody-positive patients (n = 35, median age at disease onset of 65 years, range of 16-83 years, 74 % male) mostly presented with cognitive impairment and/or epileptic seizures but also ataxia, gait disorder and personality changes. Serum autoantibodies belonged to IgG3 and IgG1 subclasses and titers ranged from 1:32 to 1:10,000. KCNA2 IgG was found in the CSF of 8/21 (38 %) patients and in the serum of 4/96 (4.2 %) healthy blood donors. KCNA2 autoantibodies bound to characteristic anatomical areas in the cerebellum and hippocampus of mammalian brain and juxtaparanodal regions of peripheral nerves but reacted exclusively with intracellular epitopes. A subset of four KCNA2 autoantibody-positive patients responded markedly to immunotherapy alongside with conversion to seronegativity, in particular those presenting an autoimmune encephalitis phenotype and receiving early immunotherapy. An available brain biopsy showed strong immune cell invasion. KCNA2 autoantibodies occurred in less than 10 % in association with an underlying tumor.Our data suggest that KCNA2 autoimmunity is clinically heterogeneous. Future studies should determine whether KCNA2 autoantibodies are directly pathogenic or develop secondarily. Early immunotherapy should be considered, in particular if autoantibodies occur in CSF or if clinical or diagnostic findings suggest ongoing inflammation. Suspicious clinical phenotypes include autoimmune encephalitis, atypical dementia, new-onset epilepsy and unexplained epileptic seizures.
000268498 536__ $$0G:(DE-HGF)POF4-353$$a353 - Clinical and Health Care Research (POF4-353)$$cPOF4-353$$fPOF IV$$x0
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000268498 650_7 $$2Other$$aAutoantibody
000268498 650_7 $$2Other$$aAutoimmune dementia
000268498 650_7 $$2Other$$aAutoimmune encephalitis
000268498 650_7 $$2Other$$aEpilepsy
000268498 650_7 $$2Other$$aImmunotherapy
000268498 650_7 $$2Other$$aKCNA2
000268498 650_7 $$2Other$$aKv1.2
000268498 650_7 $$2NLM Chemicals$$aAutoantibodies
000268498 650_7 $$2NLM Chemicals$$aKCNA2 protein, human
000268498 650_7 $$2NLM Chemicals$$aKv1.2 Potassium Channel
000268498 650_2 $$2MeSH$$aAnimals
000268498 650_2 $$2MeSH$$aHumans
000268498 650_2 $$2MeSH$$aMale
000268498 650_2 $$2MeSH$$aAdolescent
000268498 650_2 $$2MeSH$$aYoung Adult
000268498 650_2 $$2MeSH$$aAdult
000268498 650_2 $$2MeSH$$aMiddle Aged
000268498 650_2 $$2MeSH$$aAged
000268498 650_2 $$2MeSH$$aAged, 80 and over
000268498 650_2 $$2MeSH$$aFemale
000268498 650_2 $$2MeSH$$aAutoimmunity
000268498 650_2 $$2MeSH$$aRetrospective Studies
000268498 650_2 $$2MeSH$$aAutoantibodies
000268498 650_2 $$2MeSH$$aSeizures
000268498 650_2 $$2MeSH$$aAutoimmune Diseases of the Nervous System
000268498 650_2 $$2MeSH$$aMammals
000268498 650_2 $$2MeSH$$aKv1.2 Potassium Channel
000268498 650_2 $$2MeSH$$aEncephalitis
000268498 650_2 $$2MeSH$$aHashimoto Disease
000268498 7001_ $$aMiske, Ramona$$b1
000268498 7001_ $$0P:(DE-2719)2812697$$aMachule, Marie-Luise$$b2
000268498 7001_ $$aBroegger Christensen, Peter$$b3
000268498 7001_ $$aMindorf, Swantje$$b4
000268498 7001_ $$aTeegen, Bianca$$b5
000268498 7001_ $$aBorowski, Kathrin$$b6
000268498 7001_ $$0P:(DE-2719)9001872$$aButhut, Maria$$b7$$udzne
000268498 7001_ $$0P:(DE-2719)9001490$$aRößling, Rosa$$b8$$udzne
000268498 7001_ $$0P:(DE-2719)2812653$$aSánchez-Sendín, Elisa$$b9$$udzne
000268498 7001_ $$0P:(DE-2719)2812820$$avan Hoof, Scott$$b10$$udzne
000268498 7001_ $$0P:(DE-2719)9001947$$aCordero-Gómez, César$$b11$$udzne
000268498 7001_ $$0P:(DE-2719)9002228$$aBünger, Isabel$$b12
000268498 7001_ $$aRadbruch, Helena$$b13
000268498 7001_ $$aKraft, Andrea$$b14
000268498 7001_ $$aAyzenberg, Ilya$$b15
000268498 7001_ $$aKlausewitz, Jaqueline$$b16
000268498 7001_ $$aHansen, Niels$$b17
000268498 7001_ $$aTimäus, Charles$$b18
000268498 7001_ $$0P:(DE-2719)2812030$$aKörtvelyessy, Peter$$b19$$udzne
000268498 7001_ $$aPostert, Thomas$$b20
000268498 7001_ $$aBaur-Seack, Kirsten$$b21
000268498 7001_ $$aRost, Constanze$$b22
000268498 7001_ $$aBrunkhorst, Robert$$b23
000268498 7001_ $$aDoppler, Kathrin$$b24
000268498 7001_ $$aHaigis, Niklas$$b25
000268498 7001_ $$aHamann, Gerhard$$b26
000268498 7001_ $$aKunze, Albrecht$$b27
000268498 7001_ $$aStützer, Alexandra$$b28
000268498 7001_ $$aMaschke, Matthias$$b29
000268498 7001_ $$aMelzer, Nico$$b30
000268498 7001_ $$aRosenow, Felix$$b31
000268498 7001_ $$aSiebenbrodt, Kai$$b32
000268498 7001_ $$aStenør, Christian$$b33
000268498 7001_ $$0P:(DE-2719)2000030$$aDichgans, Martin$$b34$$udzne
000268498 7001_ $$aGeorgakis, Marios K$$b35
000268498 7001_ $$aFang, Rong$$b36
000268498 7001_ $$0P:(DE-2719)2810273$$aPetzold, Gabor C$$b37$$udzne
000268498 7001_ $$0P:(DE-2719)2811292$$aGörtler, Michael$$b38
000268498 7001_ $$0P:(DE-2719)2000058$$aZerr, Inga$$b39$$udzne
000268498 7001_ $$aWunderlich, Silke$$b40
000268498 7001_ $$aMihaljevic, Ivan$$b41
000268498 7001_ $$aTurko, Paul$$b42
000268498 7001_ $$aSchmidt Ettrup, Marianne$$b43
000268498 7001_ $$0P:(DE-HGF)0$$aBuchholz, Emilie$$b44
000268498 7001_ $$0P:(DE-2719)2814341$$aFoverskov Rasmussen, Helle$$b45$$udzne
000268498 7001_ $$0P:(DE-HGF)0$$aNasouti, Mahoor$$b46
000268498 7001_ $$aTalucci, Ivan$$b47
000268498 7001_ $$aMaric, Hans M$$b48
000268498 7001_ $$aHeinemann, Stefan H$$b49
000268498 7001_ $$0P:(DE-2719)2811033$$aEndres, Matthias$$b50$$udzne
000268498 7001_ $$agroup, DEMDAS study$$b51$$eCollaboration Author
000268498 7001_ $$aKomorowski, Lars$$b52
000268498 7001_ $$0P:(DE-2719)2810931$$aPrüss, Harald$$b53$$eLast author$$udzne
000268498 773__ $$0PERI:(DE-600)1462491-6$$a10.1016/j.bbi.2024.01.220$$gVol. 117, p. 399 - 411$$p399 - 411$$tBrain, behavior and immunity$$v117$$x0889-1591$$y2024
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