{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,8,19]],"date-time":"2026-08-19T18:15:39Z","timestamp":1787163339203,"version":"build-2736575974"},"reference-count":30,"publisher":"MDPI AG","issue":"1","license":[{"start":{"date-parts":[[2022,12,22]],"date-time":"2022-12-22T00:00:00Z","timestamp":1671667200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100017592","name":"Science and Technology Program of Shaanxi Province","doi-asserted-by":"publisher","award":["2021SF-333"],"award-info":[{"award-number":["2021SF-333"]}],"id":[{"id":"10.13039\/501100017592","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/501100017592","name":"Science and Technology Program of Shaanxi Province","doi-asserted-by":"publisher","award":["2022C005"],"award-info":[{"award-number":["2022C005"]}],"id":[{"id":"10.13039\/501100017592","id-type":"DOI","asserted-by":"publisher"}]},{"name":"Scientific Research Project of Shaanxi Health Commission","award":["2021SF-333"],"award-info":[{"award-number":["2021SF-333"]}]},{"name":"Scientific Research Project of Shaanxi Health Commission","award":["2022C005"],"award-info":[{"award-number":["2022C005"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Brain Sciences"],"abstract":"<jats:p>A nine-year-old boy manifested with headache, progressive mild cognitive decline and hemiparesis, but without clinical epileptic seizures (with abnormal EEG waves). Brain magnetic resonance imaging (MRI) showed bilateral cortical lesions mainly on the right hemisphere, and new lesions developed in frontal, parietal, occipital and temporal lobes around the old lesions presenting as a lace-like or ring-like enhancement in T1 with contrast over a disease course of five years. A suspected diagnosis of primary angiitis of the central nervous system was initially considered. Treated with high-dose corticosteroids, intravenous immunoglobulins and monthly pulse cyclophosphamide, his symptoms worsened with the intracranial lesion progression. Brain biopsy of the right frontal lobe was performed nearly five years after onset; prominent neuronal loss, a microglial nodule, as well as parenchymal and perivascular lymphocytic infiltrate within the cortex were found, which coincided with RE pathology changes. Encouragingly, after a regimen of rituximab, lesions on the follow-up brain MRI tended to be stable. Apparently, it was immune-mediated, but did not strictly fit any known disease entity, although it was similar to RE. We summarize this unique case, including clinical characteristics, imaging and pathology findings. We also discuss the diagnosis and treatment, focusing on comparison to RE as well as other possible neurological diseases.<\/jats:p>","DOI":"10.3390\/brainsci13010017","type":"journal-article","created":{"date-parts":[[2022,12,22]],"date-time":"2022-12-22T02:06:14Z","timestamp":1671674774000},"page":"17","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["A Special Case of Relapsing\u2013Remitting Bilateral Encephalitis: Without Epilepsy, but Responding to Rituximab and with a Brain Biopsy Coinciding with Rasmussen Encephalitis"],"prefix":"10.3390","volume":"13","author":[{"given":"Pei","family":"Liu","sequence":"first","affiliation":[{"name":"Department of Neurology, The First Hospital of Xi\u2019an (The First Affiliated Hospital of Northwest University), Xi\u2019an 710002, China"},{"name":"Xi\u2019an Key Laboratory for Innovation and Translation of Neuroimmunological Diseases, Xi\u2019an 710002, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Xuemei","family":"Lin","sequence":"additional","affiliation":[{"name":"Department of Neurology, The First Hospital of Xi\u2019an (The First Affiliated Hospital of Northwest University), Xi\u2019an 710002, China"},{"name":"Xi\u2019an Key Laboratory for Innovation and Translation of Neuroimmunological Diseases, Xi\u2019an 710002, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Shenghua","family":"Zong","sequence":"additional","affiliation":[{"name":"Department of Psychiatry and Neuropsychology, Division Translational Neuroscience, School for Mental Health and Neuroscience, Maastricht University, 6229 HX Maastricht, The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Yan","family":"Yan","sequence":"additional","affiliation":[{"name":"Department of Pathology, The First Hospital of Xi\u2019an (The First Affiliated Hospital of Northwest University), Xi\u2019an 710002, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Zhongzhong","family":"Liu","sequence":"additional","affiliation":[{"name":"Department of Neurology, The First Hospital of Xi\u2019an (The First Affiliated Hospital of Northwest University), Xi\u2019an 710002, China"},{"name":"Xi\u2019an Key Laboratory for Innovation and Translation of Neuroimmunological Diseases, Xi\u2019an 710002, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Qingli","family":"Lu","sequence":"additional","affiliation":[{"name":"Department of Neurology, The First Hospital of Xi\u2019an (The First Affiliated Hospital of Northwest University), Xi\u2019an 710002, China"},{"name":"Xi\u2019an Key Laboratory for Innovation and Translation of Neuroimmunological Diseases, Xi\u2019an 710002, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Qiaoqiao","family":"Chang","sequence":"additional","affiliation":[{"name":"Department of Neurology, The First Hospital of Xi\u2019an (The First Affiliated Hospital of Northwest University), Xi\u2019an 710002, China"},{"name":"Xi\u2019an Key Laboratory for Innovation and Translation of Neuroimmunological Diseases, Xi\u2019an 710002, China"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-1186-3322","authenticated-orcid":false,"given":"Songdi","family":"Wu","sequence":"additional","affiliation":[{"name":"Department of Neurology, The First Hospital of Xi\u2019an (The First Affiliated Hospital of Northwest University), Xi\u2019an 710002, China"},{"name":"Xi\u2019an Key Laboratory for Innovation and Translation of Neuroimmunological Diseases, Xi\u2019an 710002, China"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2022,12,22]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"454","DOI":"10.1093\/brain\/awh415","article-title":"Pathogenesis, diagnosis and treatment of Rasmussen encephalitis: A European consensus statement","volume":"128","author":"Bien","year":"2005","journal-title":"Brain"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"195","DOI":"10.1016\/S1474-4422(13)70260-6","article-title":"Rasmussen\u2019s encephalitis: Clinical features, pathobiology, and treatment advances","volume":"13","author":"Varadkar","year":"2014","journal-title":"Lancet Neurol."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"543","DOI":"10.1111\/epi.12042","article-title":"Rasmussen encephalitis: Incidence and course under randomized therapy with tacrolimus or intravenous immunoglobulins","volume":"54","author":"Bien","year":"2013","journal-title":"Epilepsia"},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"1539","DOI":"10.1111\/j.1528-1167.2012.03565.x","article-title":"Evolution of the EEG in children with Rasmussen\u2019s 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Scand."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"142","DOI":"10.1159\/000028709","article-title":"Rasmussen\u2019s encephalitis: Neuroimaging findings in 21 patients with a closer look at the basal ganglia","volume":"29","author":"Bhatjiwale","year":"1998","journal-title":"Pediatr. Neurosurg."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"678","DOI":"10.1212\/WNL.56.5.678","article-title":"Chronic encephalitis and temporal lobe epilepsy: A variant of Rasmussen\u2019s syndrome?","volume":"56","author":"Hennessy","year":"2001","journal-title":"Neurology"},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"1751","DOI":"10.1093\/brain\/awf176","article-title":"The natural history of Rasmussen\u2019s encephalitis","volume":"125","author":"Bien","year":"2002","journal-title":"Brain"},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"1325","DOI":"10.1136\/jnnp.57.11.1325","article-title":"Reappraisal of Rasmussen\u2019s syndrome with special emphasis on treatment with high doses of steroids","volume":"57","author":"Chinchilla","year":"1994","journal-title":"J. Neurol. Neurosurg. Psychiatry"},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"127","DOI":"10.1046\/j.1528-1157.2003.36602.x","article-title":"Bilateral Rasmussen encephalitis: Postmortem documentation in a five-year-old","volume":"44","author":"Tobias","year":"2003","journal-title":"Epilepsia"},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"984","DOI":"10.1212\/01.WNL.0000115393.67403.53","article-title":"Rasmussen encephalitis with active inflammation and delayed seizures onset","volume":"62","author":"Bien","year":"2004","journal-title":"Neurology"},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"387","DOI":"10.1111\/j.1468-1331.2007.01684.x","article-title":"Slowly progressive hemiparesis in childhood as a consequence of Rasmussen encephalitis without or with delayed-onset seizures","volume":"14","author":"Bien","year":"2007","journal-title":"Eur. J. Neurol."},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"269","DOI":"10.1001\/archneur.1993.00540030035011","article-title":"Rasmussen\u2019s chronic encephalitis in adults","volume":"50","author":"McLachlan","year":"1993","journal-title":"Arch. Neurol."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"352","DOI":"10.1111\/j.1528-1157.1994.tb02443.x","article-title":"Partially successful treatment of Rasmussen\u2019s encephalitis with zidovudine: Symptomatic improvement followed by involvement of the contralateral hemisphere","volume":"35","author":"DeToledo","year":"1994","journal-title":"Epilepsia"},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"418","DOI":"10.1212\/WNL.48.2.418","article-title":"Chronic encephalitis and epilepsy in adults and adolescents: A variant of Rasmussen\u2019s syndrome?","volume":"48","author":"Hart","year":"1997","journal-title":"Neurology"},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"345","DOI":"10.1111\/j.1600-0404.1997.tb00296.x","article-title":"Epilepsia partialis continua of childhood involving bilateral brain hemispheres","volume":"96","author":"Takahashi","year":"1997","journal-title":"Acta Neurol. Scand."},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"733","DOI":"10.1001\/archneur.55.5.733","article-title":"Familial alternating epilepsia partialis continua with chronic encephalitis: Another variant of Rasmussen syndrome?","volume":"55","author":"Silver","year":"1998","journal-title":"Arch. Neurol."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"398","DOI":"10.1016\/j.yebeh.2010.12.004","article-title":"Bilateral Rasmussen encephalitis","volume":"20","author":"Guan","year":"2011","journal-title":"Epilepsy Behav."},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"66","DOI":"10.1016\/j.ebcr.2013.03.004","article-title":"Bilateral Rasmussen\u2019s encephalitis associated with type II focal cortical dysplasia: Dormant \u2018second\u2019 epileptogenic zone in contralateral disease","volume":"1","author":"Frigeri","year":"2013","journal-title":"Epilepsy Behav. Case Rep."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"324","DOI":"10.1684\/epd.2013.0594","article-title":"Presentation, diagnosis and treatment of bilateral Rasmussen\u2019s encephalitis in a 12-year-old female","volume":"15","author":"Peariso","year":"2013","journal-title":"Epileptic Disord."},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"1753","DOI":"10.1111\/epi.12334","article-title":"Clinical application and evaluation of the Bien diagnostic criteria for Rasmussen encephalitis","volume":"54","author":"Olson","year":"2013","journal-title":"Epilepsia"},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"756","DOI":"10.1007\/s00247-009-1225-6","article-title":"MRI appearance of Rasmussen encephalitis","volume":"39","author":"Faingold","year":"2009","journal-title":"Pediatr. Radiol."},{"key":"ref_23","doi-asserted-by":"crossref","first-page":"250","DOI":"10.1212\/WNL.58.2.250","article-title":"Diagnosis and staging of Rasmussen\u2019s encephalitis by serial MRI and histopathology","volume":"58","author":"Bien","year":"2002","journal-title":"Neurology"},{"key":"ref_24","first-page":"179","article-title":"Rasmussen\u2019s encephalitis: Proton MR spectroscopy and diffusion MR findings","volume":"27","author":"Sener","year":"2000","journal-title":"J. Neuroradiol."},{"key":"ref_25","first-page":"1291","article-title":"(18)F-fluorodeoxyglucose positron emission tomography and MR imaging findings in Rasmussen encephalitis","volume":"22","author":"Fiorella","year":"2001","journal-title":"AJNR Am. J. Neuroradiol."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"757","DOI":"10.1136\/jnnp-2020-325300","article-title":"Autoimmune encephalitis: Proposed best practice recommendations for diagnosis and acute management","volume":"92","author":"Abboud","year":"2021","journal-title":"J. Neurol. Neurosurg. Psychiatry"},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"327","DOI":"10.1212\/WNL.58.2.327","article-title":"Unihemispheric cerebral vasculitis mimicking Rasmussen\u2019s encephalitis","volume":"58","author":"Derry","year":"2002","journal-title":"Neurology"},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"744429","DOI":"10.3389\/fnins.2021.744429","article-title":"Genetic Factors in Rasmussen\u2019s Encephalitis Characterized by Whole-Exome Sequencing","volume":"15","author":"Ai","year":"2021","journal-title":"Front. Neurosci."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"458","DOI":"10.1038\/nrneurol.2009.98","article-title":"A case of Rasmussen encephalitis treated with rituximab","volume":"5","author":"Thilo","year":"2009","journal-title":"Nat. Rev. Neurol."},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"100540","DOI":"10.1016\/j.ebr.2022.100540","article-title":"Rituximab in Rasmussen\u2019s encephalitis: A single center experience and review of the literature","volume":"19","author":"Jagtap","year":"2022","journal-title":"Epilepsy Behav. 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