{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,3]],"date-time":"2026-05-03T10:33:43Z","timestamp":1777804423355,"version":"3.51.4"},"reference-count":22,"publisher":"SAGE Publications","issue":"11","license":[{"start":{"date-parts":[[2021,5,31]],"date-time":"2021-05-31T00:00:00Z","timestamp":1622419200000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["J Child Neurol"],"published-print":{"date-parts":[[2021,10]]},"abstract":"<jats:p>CLIPPERS (chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids) is an extremely rare neurologic inflammatory condition. Fewer than 10 pediatric cases have been described.<\/jats:p>\n                  <jats:p>Debate persists as to whether it is a distinct disease or a clinical, radiologic, and histologic phenotype evolving into another disorder. We propose that CLIPPERS may be a clinical manifestation of an underlying state of immune-dysregulation.<\/jats:p>\n                  <jats:p>We describe the case of the youngest known report of CLIPPERS, an 18-month-old infant from Melbourne, Australia. Reviewing the literature for all reported pediatric cases, we identified that robust investigation and whole exome sequencing was underutilized and proposed diagnostic criteria were frequently unmet. Particular focus should be paid to genes known to cause familial hemophagocytic lymphohistiocytosis (HLH), with the CLIPPERS phenotype manifesting as a form of isolated central nervous system (CNS)-HLH in some patients. Curative treatment options such as hematopoietic stem cell transplantation may be appropriate for some patients and should be considered early.<\/jats:p>","DOI":"10.1177\/08830738211009654","type":"journal-article","created":{"date-parts":[[2021,5,31]],"date-time":"2021-05-31T06:25:29Z","timestamp":1622442329000},"page":"935-942","update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":15,"title":["Exploring the Intersection of Isolated-CNS Hemophagocytic Lymphohistiocytosis and Pediatric Chronic Lymphocytic Inflammation With Pontine Perivascular Enhancement Responsive to Steroids"],"prefix":"10.1177","volume":"36","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3002-5108","authenticated-orcid":false,"given":"Carly","family":"Debinski","sequence":"first","affiliation":[{"name":"Monash Children\u2019s Hospital, Melbourne, Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Stacy","family":"Goergen","sequence":"additional","affiliation":[{"name":"Monash Children\u2019s Hospital, Melbourne, Australia"},{"name":"Monash University, Melbourne, Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Catriona","family":"McLean","sequence":"additional","affiliation":[{"name":"Anatomical Pathology Department, Alfred Hospital, Melbourne, Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michael E.","family":"Buckland","sequence":"additional","affiliation":[{"name":"Neuropathology Department, Royal Prince Alfred, Sydney, Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Beena","family":"Kumar","sequence":"additional","affiliation":[{"name":"Monash Children\u2019s Hospital, Melbourne, Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Georgina","family":"Tiller","sequence":"additional","affiliation":[{"name":"Monash Children\u2019s Hospital, Melbourne, Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Theresa","family":"Cole","sequence":"additional","affiliation":[{"name":"Royal Children\u2019s Hospital, Melbourne, Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Samar","family":"Ojaimi","sequence":"additional","affiliation":[{"name":"Monash Children\u2019s Hospital, Melbourne, Australia"},{"name":"Monash University, Melbourne, Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Michael","family":"Fahey","sequence":"additional","affiliation":[{"name":"Monash Children\u2019s Hospital, Melbourne, Australia"},{"name":"Monash University, Melbourne, Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"179","published-online":{"date-parts":[[2021,5,31]]},"reference":[{"key":"e_1_3_2_2_2","doi-asserted-by":"publisher","DOI":"10.1182\/bloodadvances.2020001605"},{"key":"e_1_3_2_3_2","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.24955"},{"key":"e_1_3_2_4_2","doi-asserted-by":"publisher","DOI":"10.1001\/archneurol.2012.122"},{"key":"e_1_3_2_5_2","doi-asserted-by":"publisher","DOI":"10.1212\/NXI.0000000000000560"},{"key":"e_1_3_2_6_2","doi-asserted-by":"publisher","DOI":"10.1111\/dmcn.13997"},{"key":"e_1_3_2_7_2","doi-asserted-by":"publisher","DOI":"10.1177\/2329048X19831096"},{"key":"e_1_3_2_8_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.msard.2017.07.012"},{"issue":"14","key":"e_1_3_2_9_2","first-page":"P6","article-title":"A pediatric case of CLIPPERS (chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids)","volume":"84","author":"Mathias S","year":"2015","unstructured":"Mathias S, Hickman D, Lightner D, Smith C, Baumann R. A pediatric case of CLIPPERS (chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids). Neurology. 2015;84(suppl 14):P6.265.","journal-title":"Neurology"},{"key":"e_1_3_2_10_2","first-page":"26","article-title":"Clippers (chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids)\u2014case report with neuroimaging","volume":"9","author":"Salam A","year":"2014","unstructured":"Salam A, Sana F, Nazir R, Saeed MA, Iqbal Y, Ahmad A. Clippers (chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids)\u2014case report with neuroimaging. Pak J Neurol Sci. 2014;9:26\u201328.","journal-title":"Pak J Neurol Sci"},{"key":"e_1_3_2_11_2","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awx200"},{"key":"e_1_3_2_12_2","doi-asserted-by":"publisher","DOI":"10.1111\/dmcn.14043"},{"key":"e_1_3_2_13_2","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awx298"},{"key":"e_1_3_2_14_2","doi-asserted-by":"crossref","unstructured":"Zalewski NL Tobin WO. CLIPPERS. 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