{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,5,14]],"date-time":"2025-05-14T01:03:39Z","timestamp":1747184619623,"version":"3.40.5"},"reference-count":10,"publisher":"Wiley","issue":"5","license":[{"start":{"date-parts":[[2020,12,23]],"date-time":"2020-12-23T00:00:00Z","timestamp":1608681600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100000738","name":"U.S. Department of Veterans Affairs","doi-asserted-by":"publisher","id":[{"id":"10.13039\/100000738","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Euro J of Neurology"],"published-print":{"date-parts":[[2021,5]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background and purpose<\/jats:title><jats:p>Primary autoimmune cerebellar ataxia (PACA) in the absence of another triggering disease represents an emerging category of neurological illness. We report such a case whose ataxia was markedly responsive to plasma exchange. We analyzed patient serum for the presence of IgM or IgG anticerebellar neuronal antibodies.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Case presentation: rat cerebellar slice cultures incubated with patient sera were studied for IgG and IgM antibody uptake, intracellular binding, and neuronal death. Patient serum was evaluated for anti\u2010myelin associated glycoprotein (anti\u2010MAG) and associated anti\u2010glycolipid antibodies.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Antibodies were taken up by viable cerebellar neurons and bound to intracellular antigens. Uptake and predominantly nuclear binding of IgG were seen in granule cells whereas cytoplasmic binding of IgM was observed predominantly in Purkinje cells. Intracellular antibody accumulation was not accompanied by neuronal death, consistent with the patient\u2019s excellent clinical response to plasma exchange. Anti\u2010MAG or other associated anti\u2010glycolipid antibodies were not detected.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>PACA may be associated with both IgG and IgM antibodies reactive with cerebellar neuronal antigens. Our patient\u2019s response to plasma exchange supports a role for antineuronal antibodies in disease pathogenesis and emphasizes the need for rapid diagnosis and treatment.<\/jats:p><\/jats:sec>","DOI":"10.1111\/ene.14659","type":"journal-article","created":{"date-parts":[[2020,11,30]],"date-time":"2020-11-30T20:00:24Z","timestamp":1606766424000},"page":"1771-1773","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":2,"title":["Treatment\u2010responsive primary autoimmune cerebellar ataxia in a patient with IgG and IgM anticerebellar antibodies"],"prefix":"10.1111","volume":"28","author":[{"given":"Stephen L.","family":"Jaffe","sequence":"first","affiliation":[{"name":"Hunter Holmes McGuire VA Medical Center Richmond VA USA"},{"name":"Department of Neurology Virginia Commonwealth University School of Medicine Richmond VA USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-9283-4007","authenticated-orcid":false,"given":"Noel G.","family":"Carlson","sequence":"additional","affiliation":[{"name":"GRECC VASLCHCS Salt Lake City UT USA"},{"name":"Department of Neurobiology University of Utah Salt Lake City UT USA"},{"name":"Department of Neurology University of Utah Health Science Center Salt Lake City UT USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8702-1311","authenticated-orcid":false,"given":"Lisa K.","family":"Peterson","sequence":"additional","affiliation":[{"name":"Department of Pathology University of Utah Salt Lake City UT USA"},{"name":"ARUP Institute for Clinical and Experimental Pathology Salt Lake City UT USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-7788-6178","authenticated-orcid":false,"given":"John E.","family":"Greenlee","sequence":"additional","affiliation":[{"name":"Department of Neurology University of Utah Health Science Center Salt Lake City UT USA"},{"name":"Neurology Service VASLCHCS Salt Lake City UT USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2020,12,23]]},"reference":[{"doi-asserted-by":"publisher","key":"e_1_2_8_2_1","DOI":"10.1007\/s12311-015-0664-x"},{"doi-asserted-by":"publisher","key":"e_1_2_8_3_1","DOI":"10.1186\/s40673-017-0073-7"},{"doi-asserted-by":"publisher","key":"e_1_2_8_4_1","DOI":"10.1097\/NEN.0b013e3181f0c82b"},{"doi-asserted-by":"publisher","key":"e_1_2_8_5_1","DOI":"10.1186\/1742-2094-6-31"},{"key":"e_1_2_8_6_1","first-page":"483","article-title":"Cerebellar atrophy and monoclonal gammopathy of undetermined significance: a possible correlation?","volume":"24","author":"Galie E","year":"2005","journal-title":"J Exp Clin Cancer Res"},{"doi-asserted-by":"publisher","key":"e_1_2_8_7_1","DOI":"10.1007\/s00415-017-8675-9"},{"doi-asserted-by":"publisher","key":"e_1_2_8_8_1","DOI":"10.1016\/0006-8993(84)90983-1"},{"doi-asserted-by":"publisher","key":"e_1_2_8_9_1","DOI":"10.1186\/s12974-014-0160-0"},{"doi-asserted-by":"publisher","key":"e_1_2_8_10_1","DOI":"10.1001\/jamaneurol.2015.2378"},{"doi-asserted-by":"publisher","key":"e_1_2_8_11_1","DOI":"10.1186\/s40673-015-0034-y"}],"container-title":["European Journal of Neurology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/ene.14659","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1111\/ene.14659","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/ene.14659","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,8,29]],"date-time":"2023-08-29T19:22:47Z","timestamp":1693336967000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/ene.14659"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2020,12,23]]},"references-count":10,"journal-issue":{"issue":"5","published-print":{"date-parts":[[2021,5]]}},"alternative-id":["10.1111\/ene.14659"],"URL":"https:\/\/doi.org\/10.1111\/ene.14659","archive":["Portico"],"relation":{},"ISSN":["1351-5101","1468-1331"],"issn-type":[{"type":"print","value":"1351-5101"},{"type":"electronic","value":"1468-1331"}],"subject":[],"published":{"date-parts":[[2020,12,23]]},"assertion":[{"value":"2020-08-04","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2020-11-25","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2020-12-23","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}