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Later, ARHGAP26-IgG\/anti-Ca was reported in patients with limbic encephalitis\/cognitive decline or peripheral neuropathy. In several of the reported cases, the syndrome was associated with cancer. ARHGAP10\/GRAF2, which is expressed throughout the central nervous system, shares significant sequence homology with ARHGAP26\/GRAF. Mutations in the ARHGAP10 gene have been linked to cognitive and psychiatric symptoms and schizophrenia.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Objective<\/jats:title>\n                <jats:p>To assess whether ARHGAP26-IgG\/anti-Ca co-reacts with ARHGAP10.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Methods<\/jats:title>\n                <jats:p>Serological testing for ARHGAP10\/GRAF2 autoantibodies by recombinant cell-based assays and isotype and IgG subclass analyses.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Results<\/jats:title>\n                <jats:p>26\/31 serum samples (84%) from 9\/12 (75%) ARHGAP26-IgG\/anti-Ca-positive patients and 4\/6 ARHGAP26-IgG\/anti-Ca-positive CSF samples from four patients were positive also for ARHGAP10-IgG. ARHGAP10-IgG (termed anti-Ca2) remained detectable in the long-term (up to 109\u00a0months) and belonged mainly to the complement-activating IgG1 subclass. Median ARHGAP26-IgG\/anti-Ca and median ARHGAP10-IgG\/anti-Ca2 serum titres were 1:3200 and 1:1000, respectively, with extraordinarily high titres in some samples (ARHGAP26-IgG\/anti-Ca: up to 1:1000,000; ARHGAP10-IgG: up to 1:32,000). ARHGAP26\/anti-Ca serum titres exceeded those of ARHGAP10-IgG in all samples but one. A subset of patients was positive also for ARHGAP10-IgM and ARHGAP10-IgA. CSF\/serum ratios and antibody index calculation suggested intrathecal production of ARHGAP26-IgG\/anti-Ca and anti-ARHGAP10. Of 101 control samples, 100 were completely negative for ARHGAP10-IgG; a single control sample bound weakly (1:10) to the ARHGAP10-transfected cells.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Conclusions<\/jats:title>\n                <jats:p>We demonstrate that a substantial proportion of patients with ARHGAP26-IgG\/anti-Ca-positive autoimmune encephalitis co-react with ARHGAP10. Further studies on the clinical and diagnostic implications of ARHGAP10-IgG\/anti-Ca2 seropositivity in patients with autoimmune encephalitis are warranted.<\/jats:p>\n              <\/jats:sec>","DOI":"10.1007\/s00415-022-11178-9","type":"journal-article","created":{"date-parts":[[2022,5,27]],"date-time":"2022-05-27T17:02:57Z","timestamp":1653670977000},"page":"5420-5430","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":6,"title":["Rho GTPase-activating protein 10 (ARHGAP10\/GRAF2) is a novel autoantibody target in patients with autoimmune encephalitis"],"prefix":"10.1007","volume":"269","author":[{"given":"Sven","family":"Jarius","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lars","family":"Komorowski","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jens U.","family":"Regula","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"J\u00fcrgen","family":"Haas","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stefanie","family":"Brakopp","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Brigitte","family":"Wildemann","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2022,5,27]]},"reference":[{"key":"11178_CR1","doi-asserted-by":"publisher","first-page":"656","DOI":"10.3389\/fneur.2018.00656","volume":"9","author":"F Bartels","year":"2018","unstructured":"Bartels F, Pruss H, Finke C (2018) Anti-ARHGAP26 autoantibodies are associated with isolated cognitive impairment. 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