{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,18]],"date-time":"2026-09-18T06:08:47Z","timestamp":1789711727748,"version":"4.0.1"},"reference-count":21,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2026,9,18]],"date-time":"2026-09-18T00:00:00Z","timestamp":1789689600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["frontiersin.org"],"crossmark-restriction":true},"short-container-title":["Front. Immunol."],"abstract":"<jats:p>Anti-dipeptidyl-peptidase-like protein 6 (DPPX) antibody encephalitis is an ultra-rare subtype of autoimmune encephalitis (AE), with no more than 115 cases reported in the literature to date. Anti-Yo antibody-associated encephalitis is also uncommon. No previous case reports have described anti-DPPX antibody-associated encephalitis with anti-Yo antibody first detected during the one-year follow-up. This article presents the first such case, admitted to the Department of Neurology, the Affiliated Traditional Chinese Medicine Hospital, Guangzhou Medical University. The patient presented with acute onset manifested by increased muscle tone, neuronal hyperexcitability, autonomic dysfunction and transient myocardial injury. Anti-DPPX antibodies were detected in both serum and cerebrospinal fluid (CSF) upon initial admission, while anti-Yo antibodies were first detected during one-year follow-up. The patient achieved remarkable clinical remission after receiving immunotherapy with intravenous immunoglobulin (IVIG) combined with corticosteroids. We comprehensively summarize the clinical manifestations, diagnostic workup, therapeutic regimen and long-term follow-up outcomes of this case, and conduct a review of the available literature to investigate the pathophysiological mechanisms underlying these two antibodies. This report aims to raise clinicians\u2019 awareness of rare subtypes of AE, provide effective treatment strategies, and advance the understanding of the heterogeneous spectrum of AE.<\/jats:p>","DOI":"10.3389\/fimmu.2026.1792230","type":"journal-article","created":{"date-parts":[[2026,9,18]],"date-time":"2026-09-18T05:36:35Z","timestamp":1789709795000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":0,"title":["Anti-DPPX antibody-associated encephalitis with the first detected anti-Yo antibody during one-year follow-up: a case report"],"prefix":"10.3389","volume":"17","author":[{"given":"Ling","family":"Yang","sequence":"first","affiliation":[{"name":"Department of Neurology, The Affiliated Guangzhou Hospital of Traditional Chinese Medicine (TCM) of Guangzhou University of Chinese Medicine","place":["Guangzhou, China"]},{"name":"Department of Neurology, The Affiliated Traditional Chinese Medicine Hospital, Guangzhou Medical University","place":["Guangzhou, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lingling","family":"Qiu","sequence":"additional","affiliation":[{"name":"Department of Neurology, The Affiliated Guangzhou Hospital of Traditional Chinese Medicine (TCM) of Guangzhou University of Chinese Medicine","place":["Guangzhou, China"]},{"name":"Department of Neurology, The Affiliated Traditional Chinese Medicine Hospital, Guangzhou Medical University","place":["Guangzhou, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Min","family":"Zhang","sequence":"additional","affiliation":[{"name":"Department of Neurology, The Affiliated Guangzhou Hospital of Traditional Chinese Medicine (TCM) of Guangzhou University of Chinese Medicine","place":["Guangzhou, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ting","family":"Zeng","sequence":"additional","affiliation":[{"name":"Department of Neurology, The Affiliated Traditional Chinese Medicine Hospital, Guangzhou Medical University","place":["Guangzhou, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Chengyin","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Neurology, The Affiliated Traditional Chinese Medicine Hospital, Guangzhou Medical University","place":["Guangzhou, China"]}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2026,9,18]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","DOI":"10.1212\/NXI.0000000000200350","article-title":"Clinical and paraclinical characterizations, management, and prognosis in DPPX antibody-associated encephalitis: A systematic review","volume":"12","author":"Li","year":"2025","journal-title":"Neurol Neuroimmunol Neuroinflamm"},{"key":"B2","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2025.1594823","article-title":"Case report: Treatment of delayed tremor episodes in a patient with DPPX antibody encephalitis","volume":"16","author":"Yu","year":"2025","journal-title":"Front Immunol"},{"key":"B3","doi-asserted-by":"publisher","DOI":"10.1038\/s41572-025-00650-1","article-title":"Autoimmune encephalitis","volume":"11","author":"de Bruijn","year":"2025","journal-title":"Nat Rev Dis Primers"},{"key":"B4","doi-asserted-by":"publisher","DOI":"10.1002\/ana.23756","article-title":"Encephalitis and antibodies to dipeptidyl-peptidase-like protein-6, a subunit of Kv4.2 potassium channels","volume":"73","author":"Boronat","year":"2013","journal-title":"Ann Neurol"},{"key":"B5","doi-asserted-by":"publisher","DOI":"10.3760\/cma.j.cn113694-20220219-00118","article-title":"Chinese expert consensus on diagnosis and treatment of autoimmune encephalitis","volume":"55","year":"2022","journal-title":"Zhonghua Shenjingke Zazhi (Chin J Neurol)"},{"key":"B6","doi-asserted-by":"publisher","DOI":"10.3389\/fneur.2021.817896","article-title":"Clinical features and prognosis in Chinese patients with dipeptidyl-peptidase-like protein 6 antibody-associated encephalitis","volume":"12","author":"Miao","year":"2022","journal-title":"Front Neurol"},{"key":"B7","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2024.1338714","article-title":"Case report: Successful treatment of an anti-D2R and DPPX antibody-associated autoimmune encephalitis patient with high-dose methylprednisolone and intravenous immunoglobulin","volume":"15","author":"Lin","year":"2024","journal-title":"Front Immunol"},{"key":"B8","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2020.00991","article-title":"Immunological bases of paraneoplastic cerebellar degeneration and therapeutic implications","volume":"11","author":"Yshii","year":"2020","journal-title":"Front Immunol"},{"key":"B9","doi-asserted-by":"publisher","DOI":"10.3389\/fimmu.2024.1476397","article-title":"Case report: Paraneoplastic cerebellar degeneration associated with anti-Yo antibody successfully treated with ofatumumab","volume":"15","author":"Dou","year":"2024","journal-title":"Front Immunol"},{"key":"B10","doi-asserted-by":"publisher","DOI":"10.1007\/s00262-017-2041-8","article-title":"Cerebellar degeneration-related proteins 2 and 2-like are present in ovarian cancer in patients with and without Yo antibodies","volume":"66","author":"Raspotnig","year":"2017","journal-title":"Cancer Immunol Immunother"},{"key":"B11","doi-asserted-by":"publisher","DOI":"10.1177\/17562864251377559","article-title":"Clinical and tumor features of patients with immune checkpoint inhibitor-related neurological disorders and anti-Yo antibodies","volume":"18","author":"Farina","year":"2025","journal-title":"Ther Adv Neurol Disord"},{"key":"B12","doi-asserted-by":"publisher","DOI":"10.1177\/03000605241248050","article-title":"Mimickers of autoimmune encephalitis: A literature review","volume":"52","author":"Alshutaihi","year":"2024","journal-title":"J Int Med Res"},{"key":"B13","doi-asserted-by":"publisher","DOI":"10.1007\/s12016-011-8294-7","article-title":"Molecular mimicry as a mechanism of autoimmune disease","volume":"42","author":"Cusick","year":"2012","journal-title":"Clin Rev Allergy Immunol"},{"key":"B14","doi-asserted-by":"publisher","DOI":"10.3969\/j.issn.1006-2963.2024.02.013","article-title":"Two cases of anti-dipeptidyl peptidase-like protein 6 antibody-associated encephalitis","volume":"31","author":"Lu","year":"2024","journal-title":"Zhongguo Shenjing Mianyixue He Shenjingbingxue Zazhi (Chin J Neuroimmunol Neurol)"},{"key":"B15","doi-asserted-by":"publisher","DOI":"10.1002\/ejhf.1494","article-title":"Heart Failure Association of the European Society of Cardiology practical guidance on the use of natriuretic peptide concentrations","volume":"21","author":"Mueller","year":"2019","journal-title":"Eur J Heart Fail"},{"key":"B16","doi-asserted-by":"publisher","DOI":"10.1007\/s00115-022-01411-1","article-title":"Autoimmunenzephalitis \u2013 ein update [Autoimmune encephalitis-an update","volume":"94","author":"Heine","year":"2023","journal-title":"Nervenarzt"},{"key":"B17","doi-asserted-by":"publisher","DOI":"10.1002\/mdc3.13058","article-title":"Oro-bucco-lingual dyskinesia, weight loss, and cognitive decline in anti-DPPX antibody-mediated encephalitis","volume":"7","author":"Deuel","year":"2020","journal-title":"Mov Disord Clin Pract"},{"key":"B18","doi-asserted-by":"publisher","DOI":"10.1016\/j.clineuro.2020.106093","article-title":"Co-occurrence of antibodies against dipeptidyl-peptidase-like protein-6 and aquaporin-4 during a case of paraneoplastic encephalitis","volume":"197","author":"Bien","year":"2020","journal-title":"Clin Neurol Neurosurg"},{"key":"B19","doi-asserted-by":"publisher","DOI":"10.12182\/20220160206","article-title":"Disease characteristics, treatment, and prognosis of Chinese patients with autoimmune encephalitis: A retrospective study","volume":"53","author":"Yang","year":"2022","journal-title":"Sichuan Da Xue Xue Bao Yi Xue Ban"},{"key":"B20","doi-asserted-by":"publisher","DOI":"10.3760\/cma.j.cn113694-20240305-00133","article-title":"Diagnosis and treatment of autoimmune cerebellar ataxia","volume":"57","author":"Guan","year":"2024","journal-title":"Chin J Neurol"},{"key":"B21","doi-asserted-by":"publisher","DOI":"10.1136\/ijgc-2021-003077","article-title":"Paraneoplastic cerebellar degeneration associated with anti-Yo antibodies in uterine papillary serous carcinoma","volume":"32","author":"Deleuze","year":"2022","journal-title":"Int J Gynecol Cancer"}],"container-title":["Frontiers in Immunology"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fimmu.2026.1792230\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,9,18]],"date-time":"2026-09-18T05:36:46Z","timestamp":1789709806000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fimmu.2026.1792230\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,9,18]]},"references-count":21,"alternative-id":["10.3389\/fimmu.2026.1792230"],"URL":"https:\/\/doi.org\/10.3389\/fimmu.2026.1792230","relation":{},"ISSN":["1664-3224"],"issn-type":[{"value":"1664-3224","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,9,18]]},"article-number":"1792230"}}