{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T01:29:13Z","timestamp":1791250153445,"version":"4.1.0"},"reference-count":24,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2009,11,11]],"date-time":"2009-11-11T00:00:00Z","timestamp":1257897600000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Pediatric Blood &amp; Cancer"],"published-print":{"date-parts":[[2010,3]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background<\/jats:title><jats:p>Central nervous system (CNS) complications of Langerhans cell histiocytosis (LCH) include mass lesions and a neurodegenerative (ND) syndrome with ataxia, dysarthria, dysmetria, learning and behavior difficulties and\/or characteristic changes on brain MRIs. Hydrocephalus has rarely been reported in LCH. LCH lesions of the orbit, mastoid and temporal bones (\u201cCNS\u2010Risk\u201d lesions) and diabetes insipidus predispose patients to ND\u2010CNS\u2010LCH. Treatment options have been limited and only a case series using trans\u2010retinoic acid (ATRA) and intravenous immunoglobulin (IVIG) have been published.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>We have used cytosine arabinoside (ARA\u2010C) with or without vincristine to treat eight patients with ND\u2010CNS LCH. Patients: Seven male children and one young adult male with clinical and radiologic ND\u2010CNS\u2010LCH were treated with a regimen of vincristine 1.5\u2009mg\/m<jats:sup>2<\/jats:sup> on day 1 and ARA\u2010C 100\u2009mg\/m<jats:sup>2<\/jats:sup> daily for 5 days or ARA\u2010C alone monthly for 4\u201319 months. Seven patients were evaluated with an ataxia rating scale (ARS) and all with serial MRIs of the brain.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Five of seven patients had decreases in their ARS scores and\/or decreased T2 hyperintense lesions on MRI images. Grade 2 neutropenia was the most frequent adverse event. Vincristine\u2010associated neuropathy occurred in two patients. Hydrocephalus caused symptoms and signs that confounded the diagnosis and management of ND\u2010CNS\u2010LCH in all four patients affected with both.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>Subtle changes in neurologic function may be complicated by hydrocephalus. Vcr\/ARA\u2010C or ARA\u2010C were an effective therapies for some ND\u2010CNS LCH patients. A clinical trial using this and possibly other modalities such as IVIG or ATRA should be done. Pediatr Blood Cancer 2010;54:416\u2013423. \u00a9 2009 Wiley\u2010Liss, Inc.<\/jats:p><\/jats:sec>","DOI":"10.1002\/pbc.22326","type":"journal-article","created":{"date-parts":[[2009,11,11]],"date-time":"2009-11-11T19:07:31Z","timestamp":1257966451000},"page":"416-423","source":"Crossref","is-referenced-by-count":84,"title":["Neurodegenerative central nervous system Langerhans cell histiocytosis and coincident hydrocephalus treated with vincristine\/cytosine arabinoside"],"prefix":"10.1002","volume":"54","author":[{"given":"Carl E.","family":"Allen","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ricardo","family":"Flores","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ronald","family":"Rauch","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Robert","family":"Dauser","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jeffrey C.","family":"Murray","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Diane","family":"Puccetti","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David A.","family":"Hsu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paul","family":"Sondel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maxine","family":"Hetherington","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Stan","family":"Goldman","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kenneth L.","family":"McClain","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2009,11,11]]},"reference":[{"key":"e_1_2_5_2_2","first-page":"287","volume-title":"Hematology\/Oncology Clinics of North America: Langerhans Cell Histiocytosis, Vol. 12","author":"Grois N","year":"1998"},{"key":"e_1_2_5_3_2","first-page":"880","article-title":"MR imaging presentation of intracranial disease associated with Langerhans cell histiocytosis","volume":"25","author":"Prayer D","year":"2004","journal-title":"Am J Neuroradiol"},{"key":"e_1_2_5_4_2","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.20425"},{"key":"e_1_2_5_5_2","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.20760"},{"key":"e_1_2_5_6_2","doi-asserted-by":"publisher","DOI":"10.1046\/j.1440-1681.2000.03259.x"},{"key":"e_1_2_5_7_2","doi-asserted-by":"publisher","DOI":"10.3174\/ajnr.A0509"},{"key":"e_1_2_5_8_2","doi-asserted-by":"publisher","DOI":"10.1007\/s00330-006-0200-y"},{"key":"e_1_2_5_9_2","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpeds.2007.12.042"},{"key":"e_1_2_5_10_2","doi-asserted-by":"publisher","DOI":"10.1093\/brain\/awh403"},{"key":"e_1_2_5_11_2","doi-asserted-by":"publisher","DOI":"10.3171\/PED.2008.2.11.304"},{"key":"e_1_2_5_12_2","first-page":"249","article-title":"Unusual complications in the course of histiocytosis X (author's transl)","volume":"189","author":"Schwenk HU","year":"1977","journal-title":"Klin Pediatr"},{"key":"e_1_2_5_13_2","first-page":"855","article-title":"Hand\u2010Sch\u00fcller\u2010Christian disease with tumor localization in the posterior fossa","volume":"7","author":"Jacquet G","year":"1988","journal-title":"Presse Med"},{"key":"e_1_2_5_14_2","doi-asserted-by":"publisher","DOI":"10.3171\/jns.1993.78.2.0297"},{"key":"e_1_2_5_15_2","doi-asserted-by":"publisher","DOI":"10.1016\/0197-0186(95)80002-3"},{"key":"e_1_2_5_16_2","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.20040"},{"key":"e_1_2_5_17_2","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.21259"},{"key":"e_1_2_5_18_2","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.21225"},{"key":"e_1_2_5_19_2","first-page":"199","article-title":"Langerhans cell histiocytosis: Central nervous system involvement treated successfully with 2\u2010chlorodeoxyadenosine","volume":"18","author":"Watts J","year":"2001","journal-title":"PediatrHematolOncol"},{"key":"e_1_2_5_20_2","doi-asserted-by":"publisher","DOI":"10.1002\/pbc.20175"},{"key":"e_1_2_5_21_2","first-page":"4141","article-title":"Plasma and cerebrospinal fluid pharmacokinetics of 1\u2010\u03b2\u2010D\u2010arabinofuranosylcytosine and 1\u2010\u03b2\u2010D\u2010arabinofuranosyluracil following the repeated intravenous administration of high\u2010 and intermediate\u2010dose 1\u2010\u03b2\u2010D\u2010arabinofuranosylcytosine","volume":"51","author":"Damon LE","year":"1991","journal-title":"Cancer Res"},{"key":"e_1_2_5_22_2","doi-asserted-by":"publisher","DOI":"10.1007\/BF00686248"},{"key":"e_1_2_5_23_2","doi-asserted-by":"publisher","DOI":"10.1002\/mpo.2950210406"},{"key":"e_1_2_5_24_2","doi-asserted-by":"publisher","DOI":"10.1212\/WNL.33.11.1444"},{"key":"e_1_2_5_25_2","doi-asserted-by":"publisher","DOI":"10.1111\/j.1471-4159.2004.02774.x"}],"container-title":["Pediatric Blood &amp; Cancer"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fpbc.22326","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fpbc.22326","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1002\/pbc.22326","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,10,19]],"date-time":"2023-10-19T00:17:20Z","timestamp":1697674640000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1002\/pbc.22326"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2009,11,11]]},"references-count":24,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2010,3]]}},"alternative-id":["10.1002\/pbc.22326"],"URL":"https:\/\/doi.org\/10.1002\/pbc.22326","archive":["Portico"],"relation":{},"ISSN":["1545-5009","1545-5017"],"issn-type":[{"value":"1545-5009","type":"print"},{"value":"1545-5017","type":"electronic"}],"subject":[],"published":{"date-parts":[[2009,11,11]]}}}