{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,23]],"date-time":"2026-09-23T02:19:50Z","timestamp":1790129990229,"version":"4.0.1"},"reference-count":26,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2025,10,30]],"date-time":"2025-10-30T00:00:00Z","timestamp":1761782400000},"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>Progressive encephalomyelitis with rigidity and myoclonus (PERM) is a rare neurological disorder characterized by rigidity, painful spasms, hyperekplexia, brainstem involvement, and autonomic dysfunction. In China, limited awareness and delayed antibody testing often hinder early diagnosis. Here, we report four cases of PERM, three of which required intensive care unit (ICU) admission. The clinical and immunological features of these patients were systematically summarized. This case series highlights the characteristic clinical and immunological profiles of PERM and underscores the importance of early recognition and timely intervention, as most patients achieve substantial recovery with immunosuppressive therapy.<\/jats:p>","DOI":"10.3389\/fimmu.2025.1616207","type":"journal-article","created":{"date-parts":[[2025,10,30]],"date-time":"2025-10-30T06:22:50Z","timestamp":1761805370000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":2,"title":["Clinical features of progressive encephalomyelitis with rigidity and myoclonus: Case Report"],"prefix":"10.3389","volume":"16","author":[{"given":"Lixia","family":"Qin","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Qihua","family":"Chen","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1965","published-online":{"date-parts":[[2025,10,30]]},"reference":[{"key":"B1","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp-2014-309201","article-title":"Stiff-person syndrome: insights into a complex autoimmune disorder","volume":"86","author":"Baizabal-Carvallo","year":"2015","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"B2","doi-asserted-by":"publisher","first-page":"587","DOI":"10.1038\/s41582-024-01012-3","article-title":"Stiff-person syndrome and related disorders - diagnosis, mechanisms and therapies","volume":"20","author":"Dalakas","year":"2024","journal-title":"Nat Rev Neurol"},{"key":"B3","doi-asserted-by":"publisher","first-page":"27","DOI":"10.1093\/brain\/99.1.27","article-title":"Progressive encephalomyelitis with rigidity","volume":"99","author":"Whiteley","year":"1976","journal-title":"Brain"},{"key":"B4","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awu142","article-title":"Glycine receptor antibodies in perm and related syndromes: characteristics, clinical features and outcomes","volume":"137","author":"Carvajal-Gonz\u00e1lez","year":"2014","journal-title":"Brain"},{"key":"B5","doi-asserted-by":"publisher","DOI":"10.1002\/ana.25832","article-title":"Glycine receptor autoantibodies impair receptor function and induce motor dysfunction","volume":"88","author":"Rauschenberger","year":"2020","journal-title":"Ann Neurol"},{"key":"B6","doi-asserted-by":"publisher","DOI":"10.1002\/mds.10279","article-title":"Stiff man syndrome and related conditions","volume":"17","author":"Meinck","year":"2002","journal-title":"Mov Disord"},{"key":"B7","doi-asserted-by":"publisher","DOI":"10.1212\/01.wnl.0000327606.50322.f0","article-title":"Progressive encephalomyelitis, rigidity, and myoclonus: A novel glycine receptor antibody","volume":"71","author":"Hutchinson","year":"2008","journal-title":"Neurology"},{"key":"B8","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awu153","article-title":"Glycine receptor antibodies in perm: A new channelopathy","volume":"137","author":"Martinez-Martinez","year":"2014","journal-title":"Brain"},{"key":"B9","doi-asserted-by":"publisher","first-page":"e200473","DOI":"10.1212\/nxi.0000000000200473","article-title":"Progressive encephalomyelitis with rigidity and myoclonus with glycine receptor antibodies: clinical features and outcomes","volume":"12","author":"Guasp","year":"2025","journal-title":"Neurol Neuroimmunol Neuroinflamm"},{"key":"B10","doi-asserted-by":"publisher","DOI":"10.1007\/s00415-023-12123-0","article-title":"Expanding clinical profiles and prognostic markers in stiff person syndrome spectrum disorders","volume":"271","author":"Wang","year":"2024","journal-title":"J Neurol"},{"key":"B11","doi-asserted-by":"publisher","DOI":"10.1016\/j.jocn.2013.07.014","article-title":"Hypoventilation in glycine-receptor antibody related progressive encephalomyelitis, rigidity and myoclonus","volume":"21","author":"Bourke","year":"2014","journal-title":"J Clin Neurosci"},{"key":"B12","doi-asserted-by":"publisher","DOI":"10.1001\/jamaneurol.2013.4442","article-title":"Childhood onset of stiff-man syndrome","volume":"70","author":"Clardy","year":"2013","journal-title":"JAMA Neurol"},{"key":"B13","doi-asserted-by":"publisher","first-page":"498","DOI":"10.1001\/jamaneurol.2013.1872","article-title":"Progressive encephalomyelitis with rigidity and myoclonus: the first pediatric case with glycine receptor antibodies","volume":"70","author":"Dam\u00e1sio","year":"2013","journal-title":"JAMA Neurol"},{"key":"B14","doi-asserted-by":"publisher","DOI":"10.1001\/jamaneurol.2016.0133","article-title":"Clinical and immunologic investigations in patients with stiff-person spectrum disorder","volume":"73","author":"Martinez-Hernandez","year":"2016","journal-title":"JAMA Neurol"},{"key":"B15","doi-asserted-by":"publisher","first-page":"e200298","DOI":"10.1212\/nxi.0000000000200298","article-title":"Progressive encephalomyelitis with rigidity and myoclonus with glycine receptor and gad65 antibodies: case report and potential mechanisms","volume":"11","author":"Winklehner","year":"2024","journal-title":"Neurol Neuroimmunol Neuroinflamm"},{"key":"B16","doi-asserted-by":"publisher","first-page":"44","DOI":"10.1001\/jamaneurol.2013.574","article-title":"Glycine receptor autoimmune spectrum with stiff-man syndrome phenotype","volume":"70","author":"McKeon","year":"2013","journal-title":"JAMA Neurol"},{"key":"B17","doi-asserted-by":"publisher","DOI":"10.3389\/fneur.2023.1289460","article-title":"Prevalence of non-neurological autoantibodies and related comorbidities in stiff person spectrum disorders","volume":"14","author":"Balshi","year":"2023","journal-title":"Front Neurol"},{"key":"B18","doi-asserted-by":"publisher","first-page":"39","DOI":"10.1016\/j.jns.2017.08.010","article-title":"Myasthenia gravis with systemic and neurological polyautoimmunity","volume":"381","author":"Duarte","year":"2017","journal-title":"J Neurol Sci"},{"key":"B19","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra1602678","article-title":"Myasthenia gravis","volume":"375","author":"Gilhus","year":"2016","journal-title":"N Engl J Med"},{"key":"B20","doi-asserted-by":"publisher","DOI":"10.4103\/neuroIndia.NI_493_16","article-title":"Stiff-person syndrome after thymectomy in myasthenia gravis mimicking a post-thymectomy myasthenic crisis","volume":"65","author":"Lee","year":"2017","journal-title":"Neurol India"},{"key":"B21","doi-asserted-by":"publisher","DOI":"10.1002\/(SICI)1097-4598(199704)20:4<493::AID-MUS13>3.0.CO;2-#","article-title":"Stiff-persons\u2019 Syndrome associated with thymoma and subsequent myasthenia gravis","volume":"20","author":"Nicholas","year":"1997","journal-title":"Muscle Nerve"},{"key":"B22","doi-asserted-by":"publisher","DOI":"10.2169\/internalmedicine.56.7979","article-title":"Thymoma-associated progressive encephalomyelitis with rigidity and myoclonus (Perm) with myasthenia gravis","volume":"56","author":"Morise","year":"2017","journal-title":"Intern Med"},{"key":"B23","doi-asserted-by":"publisher","DOI":"10.1136\/jnnp.2004.036558","article-title":"Stiff person syndrome with eye movement abnormality, myasthenia gravis, and thymoma","volume":"76","author":"Thomas","year":"2005","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"B24","first-page":"98","article-title":"Stiff person syndrome and myasthenia gravis","volume":"50","author":"Saravanan","year":"2002","journal-title":"Neurol India"},{"key":"B25","doi-asserted-by":"publisher","first-page":"199","DOI":"10.1038\/cmi.2010.74","article-title":"Thymoma and autoimmunity","volume":"8","author":"Shelly","year":"2011","journal-title":"Cell Mol Immunol"},{"key":"B26","doi-asserted-by":"publisher","DOI":"10.1016\/j.bbcan.2022.188730","article-title":"Recent thymic emigrants as the bridge between thymoma and autoimmune diseases","volume":"1877","author":"Ao","year":"2022","journal-title":"Biochim Biophys Acta Rev Cancer"}],"container-title":["Frontiers in Immunology"],"original-title":[],"link":[{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fimmu.2025.1616207\/full","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,30]],"date-time":"2025-10-30T06:22:50Z","timestamp":1761805370000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.frontiersin.org\/articles\/10.3389\/fimmu.2025.1616207\/full"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,10,30]]},"references-count":26,"alternative-id":["10.3389\/fimmu.2025.1616207"],"URL":"https:\/\/doi.org\/10.3389\/fimmu.2025.1616207","relation":{},"ISSN":["1664-3224"],"issn-type":[{"value":"1664-3224","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,10,30]]},"article-number":"1616207"}}