{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,19]],"date-time":"2026-10-19T02:13:09Z","timestamp":1792375989466,"version":"4.4.0"},"reference-count":12,"publisher":"Springer Science and Business Media LLC","issue":"3","license":[{"start":{"date-parts":[[2023,8,9]],"date-time":"2023-08-09T00:00:00Z","timestamp":1691539200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2023,8,9]],"date-time":"2023-08-09T00:00:00Z","timestamp":1691539200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Cerebellum"],"abstract":"<jats:title>Abstract<\/jats:title><jats:p>We describe a male patient presenting with cerebellar ataxia and behavioural frontotemporal dementia in whom imaging showed cerebellar atrophy. He had significantly low N-acetyl aspartate to creatine (NAA\/Cr) area ratio on MR spectroscopy of the cerebellum, primarily affecting the vermis. CT body scan showed extensive abnormal tissue within the mesentery, the retroperitoneum and perinephric areas. PET-CT showed increased tracer uptake within the wall of the aorta suggestive of an aortitis and within the perinephric tissue bilaterally. Biopsy of the perinephric tissue confirmed IgG4 disease. Treatment with steroids and mycophenolate improved his clinical state, but he developed symptoms attributed to pericardiac effusion that necessitated treatment initially with drainage and subsequently with pericardial window. After a course of rituximab, he had an episode of sepsis that did not respond to appropriate treatment and died as a result. Both the imaging findings and neurological presentation with cerebellar ataxia and behavioural frontotemporal dementia are novel in the context of IgG4 disease.<\/jats:p>","DOI":"10.1007\/s12311-023-01592-8","type":"journal-article","created":{"date-parts":[[2023,8,9]],"date-time":"2023-08-09T17:01:45Z","timestamp":1691600505000},"page":"1231-1234","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":7,"title":["IgG4 Disease-Related Ataxia"],"prefix":"10.1007","volume":"23","author":[{"given":"Marios","family":"Hadjivassiliou","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Daniel","family":"Blackburn","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ronan","family":"O\u2019Malley","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Nigel","family":"Hoggard","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2023,8,9]]},"reference":[{"issue":"10","key":"1592_CR1","doi-asserted-by":"publisher","first-page":"982","DOI":"10.1007\/s00535-003-1175-y","volume":"38","author":"T Kamisawa","year":"2003","unstructured":"Kamisawa T, Funata N, Hayashi Y, Eishi Y, Koike M, Tsuruta K, et al. A new clinicopathological entity of IgG4-related autoimmune disease. J Gastroenterol. 2003;38(10):982\u20134. https:\/\/doi.org\/10.1007\/s00535-003-1175-y.","journal-title":"J Gastroenterol."},{"key":"1592_CR2","doi-asserted-by":"publisher","first-page":"1403","DOI":"10.1016\/S0140-6736(02)08359-9","volume":"359","author":"H Hamano","year":"2002","unstructured":"Hamano H, Kawa S, Ochi Y, Unno H, Shiba N, Wajiki M, et al. Hydronephrosis associated with retroperitoneal fibrosis and sclerosing pancreatitis. Lancet. 2002;359:1403\u20134.","journal-title":"Lancet"},{"key":"1592_CR3","doi-asserted-by":"publisher","first-page":"732","DOI":"10.1056\/NEJM200103083441005","volume":"344","author":"H Hamano","year":"2001","unstructured":"Hamano H, Kawa S, Horiuchi A, Unno H, Furuya N, Akamatsu T, et al. High serum IgG4 concentrations in patients with sclerosing pancreatitis. N Engl J Med. 2001;344:732\u20138.","journal-title":"N Engl J Med"},{"key":"1592_CR4","doi-asserted-by":"publisher","first-page":"335","DOI":"10.3109\/s10165-006-0518-Y","volume":"16","author":"M Yamamoto","year":"2006","unstructured":"Yamamoto M, Takahashi H, Ohara M, Suzuki C, Naishiro Y, Yamamoto H, et al. A new conceptualization of Mikulicz\u2019s disease as an IgG4-related plasmacytic disease. Mod Rheumatol. 2006;16:335\u201340.","journal-title":"Mod Rheumatol"},{"key":"1592_CR5","doi-asserted-by":"publisher","first-page":"e3344","DOI":"10.1097\/MD.0000000000003344","volume":"95","author":"CA Perugino","year":"2016","unstructured":"Perugino CA, Wallace ZS, Meyersohn N, et al. Large vessel involvement by IgG4-related disease. Medicine. 2016;95:e3344.","journal-title":"Medicine"},{"key":"1592_CR6","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1002\/art.37715","volume":"65","author":"JC Jennette","year":"2013","unstructured":"Jennette JC, Falk RJ, Bacon PA, et al. 2012 Revised International Chapel Hill Consensus Conference nomenclature of vasculitides. Artritis Rheum. 2013;65:1\u201311.","journal-title":"Artritis Rheum"},{"issue":"1","key":"1592_CR7","doi-asserted-by":"publisher","first-page":"108","DOI":"10.1097\/RLU.0b013e31823933c1","volume":"37","author":"T Norikane","year":"2012","unstructured":"Norikane T, Yamamoto Y, Okada M, Maeda Y, Aga F, Kawai N, et al. Hypertrophic cranial pachymeningitis with IgG4-positive plasma cells detected by C-11 methionine PET. Clin Nucl Med. 2012;37(1):108\u20139. https:\/\/doi.org\/10.1097\/RLU.0b013e31823933c1.","journal-title":"Clin Nucl Med"},{"key":"1592_CR8","doi-asserted-by":"publisher","unstructured":"Tse GH, Hadjivassiliou M, Devgire V, Hobson E, Connolly DJA, Hoggard N. IgG4-related disease presenting with ataxia. Res Square. 2021. https:\/\/doi.org\/10.21203\/rs.3.rs-139926\/V1.","DOI":"10.21203\/rs.3.rs-139926\/V1"},{"key":"1592_CR9","doi-asserted-by":"publisher","unstructured":"Hadjivassiliou M, Graus F, Honnorat J, Jarius S, Titulaer M, Manto M, et al. Diagnostic criteria for primary autoimmune cerebellar ataxia-guidelines from an International Task Force on immune-mediated cerebellar ataxias. 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