{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,5]],"date-time":"2026-05-05T03:00:23Z","timestamp":1777950023999,"version":"3.51.4"},"reference-count":24,"publisher":"Frontiers Media SA","license":[{"start":{"date-parts":[[2026,5,4]],"date-time":"2026-05-04T00:00:00Z","timestamp":1777852800000},"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. Med."],"abstract":"<jats:p>Recurrent polychondritis (RP) is a rare systemic autoimmune disorder that rarely involves the central nervous system (CNS). This report describes a 68-year-old male RP patient who was admitted with bilateral auricular swelling and pain that had lasted for one month, accompanied by limb weakness and pain that had lasted for ten days. Neurological assessment revealed sluggish responses, dysarthria, decreased limb muscle strength and urinary\/bowel dysfunction, with a Montreal Cognitive Assessment (MoCA) score of 21\/30. Laboratory tests showed elevated inflammatory markers, and autoantibodies in the serum and cerebrospinal fluid (CSF) were negative. CSF analysis revealed increased intracranial pressure and elevated protein levels, as well as decreased immunoglobulin and complement levels. A brain MRI revealed bilateral periventricular, centrum semiovale, and medial temporal lobe fluid-attenuated inversion recovery (FLAIR) hyperintensities. The diagnosis of RP was confirmed based on the Damiani\u2013Levine criteria, alongside the diagnoses of autoimmune limbic encephalitis (LE) and secondary demyelinating encephalopathy (DE). Following treatment with methylprednisolone pulse therapy followed by cyclophosphamide, the patient\u2019s symptoms improved significantly. At the three-month follow-up, limb muscle strength had recovered, but urinary dysfunction persisted. This case demonstrates that RP can affect the CNS and the central-autonomic axis. Early recognition and initiation of immunotherapy can improve prognosis.<\/jats:p>","DOI":"10.3389\/fmed.2026.1825793","type":"journal-article","created":{"date-parts":[[2026,5,4]],"date-time":"2026-05-04T05:34:26Z","timestamp":1777872866000},"update-policy":"https:\/\/doi.org\/10.3389\/crossmark-policy","source":"Crossref","is-referenced-by-count":0,"title":["Recurrent polychondritis complicated with demyelinating encephalopathy and limbic encephalitis: a case report and literature review"],"prefix":"10.3389","volume":"13","author":[{"given":"Xinyue","family":"Wang","sequence":"first","affiliation":[{"name":"Department of Neurology, Xinhua Hospital Affiliated to Dalian University","place":["Dalian, Liaoning, China"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yue","family":"Wang","sequence":"additional","affiliation":[{"name":"Department of Medical Psychology, No. 967 Hospital, Joint Logistics Support Force, Chinese People's Liberation Army (Maolin Campus)","place":["Dalian, Liaoning, China"]}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ping","family":"Lin","sequence":"additional","affiliation":[{"name":"Department of Neurology, Xinhua Hospital Affiliated to Dalian University","place":["Dalian, Liaoning, China"]}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1965","published-online":{"date-parts":[[2026,5,4]]},"reference":[{"key":"ref1","doi-asserted-by":"publisher","first-page":"101867","DOI":"10.1016\/j.berh.2023.101867","article-title":"Relapsing polychondritis: best Practice & Clinical Rheumatology","volume":"37","author":"Mertz","year":"2023","journal-title":"Best Pract Res Clin Rheumatol"},{"key":"ref2","doi-asserted-by":"publisher","first-page":"1687704","DOI":"10.3389\/fimmu.2025.1687704","article-title":"Anti-gamma-aminobutyric acid B receptor antibody-associated limbic encephalitis in relapsing polychondritis: a rare case report and literature review","volume":"16","author":"Zhang","year":"2025","journal-title":"Front Immunol"},{"key":"ref3","doi-asserted-by":"publisher","first-page":"1846","DOI":"10.3346\/jkms.2016.31.11.1846","article-title":"Relapsing Polychondritis with central nervous system involvement: experience of three different cases in a single center","volume":"31","author":"Jeon","year":"2016","journal-title":"J Korean Med Sci"},{"key":"ref4","doi-asserted-by":"publisher","first-page":"618","DOI":"10.7861\/clinmed.2023-0430","article-title":"Relapsing polychondritis presenting with sero-negative limbic encephalitis","volume":"23","author":"Husein","year":"2023","journal-title":"Clin Med"},{"key":"ref5","doi-asserted-by":"publisher","first-page":"481","DOI":"10.2169\/internalmedicine.9411-22","article-title":"Relapsing Polychondritis and aseptic meningoencephalitis","volume":"62","author":"Yokota","year":"2023","journal-title":"Intern Med"},{"key":"ref6","doi-asserted-by":"publisher","first-page":"1397","DOI":"10.1007\/s13760-022-01970-9","article-title":"Relapsing polychondritis with multiple neurologic manifestations","volume":"122","author":"Lee","year":"2022","journal-title":"Acta Neurol Belg"},{"key":"ref7","doi-asserted-by":"publisher","first-page":"229","DOI":"10.31138\/mjr.34.2.229","article-title":"Limbic encephalitis as a late complication of relapsing Polychondritis: a case report and review of the literature","volume":"34","author":"Michalaki","year":"2023","journal-title":"Mediterr J Rheumatol"},{"key":"ref8","doi-asserted-by":"publisher","first-page":"20587384211000547","DOI":"10.1177\/20587384211000547","article-title":"Comparison of relapsing polychondritis patients with and without central nervous system involvement: a retrospective study of 181 patients","volume":"35","author":"Cao","year":"2021","journal-title":"Int J Immunopathol Pharmacol"},{"key":"ref9","doi-asserted-by":"publisher","first-page":"391","DOI":"10.1016\/S1474-4422(15)00401-9","article-title":"A clinical approach to diagnosis of autoimmune encephalitis","volume":"15","author":"Graus","year":"2016","journal-title":"Lancet Neurol"},{"key":"ref10","doi-asserted-by":"publisher","first-page":"318","DOI":"10.1016\/j.clineuro.2005.11.025","article-title":"CNS demyelination in autoimmune diseases","volume":"108","author":"Brinar","year":"2006","journal-title":"Clin Neurol Neurosurg"},{"key":"ref11","doi-asserted-by":"publisher","first-page":"929","DOI":"10.1288\/00005537-197906000-00009","article-title":"Relapsing polychondritis--report of ten cases","volume":"89","author":"Damiani","year":"1979","journal-title":"Laryngoscope"},{"key":"ref12","doi-asserted-by":"crossref","first-page":"1269","DOI":"10.1093\/brain\/113.5.1269","article-title":"A study of immunoglobulin G in the cerebrospinal fluid of 1007 patients with suspected neurological disease using isoelectric focusing and the log IgG-index. 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