{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,11,1]],"date-time":"2025-11-01T17:15:39Z","timestamp":1762017339009},"reference-count":35,"publisher":"Journal of Neurosurgery Publishing Group (JNSPG)","issue":"5","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["PED"],"published-print":{"date-parts":[[2008,11]]},"abstract":"<jats:p>The second most frequent central nervous system involvement pattern in Langerhans cell histiocytosis (LCH) is a rare condition documented in a number of reports called \u201cneurodegenerative LCH\u201d (ND-LCH). Magnetic resonance images confirming the presence of the disease usually demonstrate striking symmetric bilateral hyperintensities predominantly in the cerebellum, basal ganglia, pons, and\/or cerebral white matter. The authors here describe for the first time in the literature a patient with ND-LCH and concomitant hydrocephalus initially treated using endoscopic third ventriculostomy (ETV). This 9-year-old boy, who had undergone chemotherapy for skin and lung LCH without central nervous system involvement at the age of 10 months, presented with acute ataxia, headaches, and paraparesis and a 1-year history of gradually increasing clumsiness. Magnetic resonance images showed obstructive hydrocephalus at the level of the aqueduct of Sylvius and signs of ND-LCH. After registering high intracranial pressure (ICP) spikes with an intraparenchymal pressure monitor, an ETV was performed. A second ETV was required months later because of ostomy occlusion, and finally a ventriculoperitoneal shunt was placed because of ostomy reocclusion. Endoscopic third ventriculostomy was initially considered the treatment of choice to divert cerebrospinal fluid without leaving a ventriculoperitoneal shunt and to obtain biopsy specimens from the periinfundibular recess area. The third ventriculostomy occluded twice, and an endoscopic aqueduct fenestration was unsuccessful. The authors hypothesized that an inflammatory process related to late ND disease was responsible for the occlusions. Biopsy specimens from the infundibular recess and fornix column did not show histopathogical abnormalities. Increased ICP symptoms resolved with cerebrospinal fluid diversion. This case is the first instance of ND-LCH with hydrocephalus reported in the literature to date. Shunt placement rather than ETV seems to be the favorable choice in relieving elevated ICP.<\/jats:p>","DOI":"10.3171\/ped.2008.2.11.304","type":"journal-article","created":{"date-parts":[[2008,10,31]],"date-time":"2008-10-31T18:59:56Z","timestamp":1225479596000},"page":"304-309","source":"Crossref","is-referenced-by-count":5,"title":["Failure to treat obstructive hydrocephalus with endoscopic third ventriculostomy in a patient with neurodegenerative Langerhans cell histiocytosis"],"prefix":"10.3171","volume":"2","author":[{"given":"Amir","family":"Kershenovich","sequence":"first","affiliation":[{"name":"Departments of Pediatric Neurosurgery and"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Angela V.","family":"Price","sequence":"additional","affiliation":[{"name":"Departments of Pediatric Neurosurgery and"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Korgun","family":"Koral","sequence":"additional","affiliation":[{"name":"Radiology, Children's Medical Center Dallas, University of Texas Southwestern; and"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Stan","family":"Goldman","sequence":"additional","affiliation":[{"name":"Department of Pediatric Hemato-Oncology, Medical City Dallas Hospital, Dallas, Texas"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Dale M.","family":"Swift","sequence":"additional","affiliation":[{"name":"Departments of Pediatric Neurosurgery and"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1754","reference":[{"key":"b1-0020304","series-title":"Eur J Cancer","doi-asserted-by":"publisher","first-page":"747","DOI":"10.1016\/S0959-8049(99)00039-8","article-title":"The histiocytoses: the fall of the tower of Babel","volume":"35","author":"Arceci","year":"1999"},{"key":"b2-0020304","series-title":"J Child Neurol","doi-asserted-by":"publisher","first-page":"150","DOI":"10.1177\/088307380001500302","article-title":"Langerhans cell histiocytosis and the central nervous system in childhood: evolution and prognostic factors. Results of a collaborative study","volume":"15","author":"Barthez","year":"2000"},{"key":"b3-0020304","series-title":"Nervenarzt","first-page":"437","article-title":"[Cerebellar syndromes in Langerhans' cell histiocytosis.]","volume":"78","author":"B\u00f6s","year":"2007"},{"key":"b4-0020304","series-title":"Pediatr Blood Cancer","doi-asserted-by":"publisher","first-page":"286","DOI":"10.1002\/pbc.20175","article-title":"LCH with CNS involvement: Follow up by FDG PET during treatment with cladribine","volume":"44","author":"B\u00fcchler","year":"2005"},{"key":"b5-0020304","series-title":"Neuroradiology","doi-asserted-by":"publisher","first-page":"548","DOI":"10.1007\/s002340050802","article-title":"Cerebral localization of LCH: MRI neuropathologic correlations","volume":"41","author":"Colombo","year":"1999"},{"key":"b6-0020304","series-title":"Pediatr Blood Cancer","doi-asserted-by":"publisher","first-page":"72","DOI":"10.1002\/pbc.21225","article-title":"Analysis of outcome for patients with mass lesions of the central nervous system due to Langerhans cell histiocytosis treated with 2-chlorodeoxyadenosine","volume":"50","author":"Dhall","year":"2008"},{"key":"b7-0020304","series-title":"J Pediatr","doi-asserted-by":"publisher","first-page":"344","DOI":"10.1016\/j.jpeds.2003.12.030","article-title":"Endocrine involvement in pediatric onset LCH: a population based study","volume":"144","author":"Donadieu","year":"2004"},{"key":"b8-0020304","series-title":"J Pediatr","doi-asserted-by":"publisher","first-page":"1","DOI":"10.1016\/S0022-3476(95)70248-2","article-title":"Langerhans Cell Histiocytosis","volume":"127","author":"Egeler","year":"1995"},{"key":"b9-0020304","series-title":"Sem Hop","first-page":"2229","article-title":"[Posterior fossa syndrome terminating the course of histiocytosis X. Study of lesions of the central nervous system. Association with polyvinylpyrrolidone thesaurismosis.]","volume":"51","author":"Faivre","year":"1975"},{"key":"b10-0020304","series-title":"J Pediatr","doi-asserted-by":"publisher","first-page":"728","DOI":"10.1067\/mpd.2001.111331","article-title":"A randomized trial of treatment for multisystem LCH","volume":"138","author":"Gadner","year":"2001"},{"key":"b11-0020304","series-title":"Med Pediatr Oncol","first-page":"72","article-title":"Treatment strategy for disseminated LCH, DAL HX-83 study Group","volume":"23","author":"Gadner","year":"1994"},{"key":"b12-0020304","series-title":"J Neurol Neurosurg Psychiatry","doi-asserted-by":"publisher","first-page":"180","DOI":"10.1136\/jnnp.58.2.180","article-title":"Progressive spinocerebellar degeneration plus associated with LCH: a new paraneoplastic syndrome?","volume":"58","author":"Goldberg-Stern","year":"1995"},{"key":"b13-0020304","series-title":"Hematol Oncol Clin North Am","doi-asserted-by":"publisher","first-page":"287","DOI":"10.1016\/S0889-8588(05)70511-6","article-title":"Central nervous system disease in LCH","volume":"12","author":"Grois","year":"1998"},{"key":"b14-0020304","series-title":"Brain","doi-asserted-by":"publisher","first-page":"829","DOI":"10.1093\/brain\/awh403","article-title":"Neuropathology of CNS disease in LCH","volume":"128","author":"Grois","year":"2005"},{"key":"b15-0020304","series-title":"Pediatr Blood Cancer","doi-asserted-by":"publisher","first-page":"438","DOI":"10.1002\/pbc.20021","article-title":"Permanent consequences in LCH patients: a pilot study from the Histiocyte Society-Late Effects Study Group","volume":"42","author":"Haupt","year":"2004"},{"key":"b16-0020304","series-title":"Cancer","doi-asserted-by":"publisher","first-page":"2278","DOI":"10.1002\/(SICI)1097-0142(19990515)85:10<2278::AID-CNCR25>3.0.CO;2-U","article-title":"Langerhans Cell Histiocytosis: diagnosis, natural history, management and outcome","volume":"85","author":"Howarth","year":"1999"},{"key":"b17-0020304","series-title":"Pediatr Blood Cancer","doi-asserted-by":"publisher","first-page":"55","DOI":"10.1002\/pbc.20040","article-title":"Retinoic acid therapy in \u201cdegenerativelike\u201d neuro-Langerhans Cell Histiocytosis: a prospective pilot study","volume":"43","author":"Idbaih","year":"2004"},{"key":"b18-0020304","series-title":"J Pediatr Hematol Oncol","doi-asserted-by":"publisher","first-page":"735","DOI":"10.1097\/00043426-200411000-00009","article-title":"Cerebellar ataxia in pediatric patients with LCH","volume":"26","author":"Imashuku","year":"2004"},{"key":"b19-0020304","series-title":"Pediatr Blood Cancer","doi-asserted-by":"publisher","first-page":"308","DOI":"10.1002\/pbc.21259","article-title":"Treatment of neurodegenerative CNS disease in Langerhans cell histiocytosis with a combination of intravenous immunoglobulin and chemotherapy","volume":"50","author":"Imashuku","year":"2008"},{"key":"b20-0020304","series-title":"Haematologica","doi-asserted-by":"publisher","first-page":"615","DOI":"10.3324\/haematol.11827","article-title":"Neurodegenerative central nervous system disease as late sequelae of Langerhans cell histiocytosis. Report from the Japan LCH Study Group","volume":"93","author":"Imashuku","year":"2008"},{"key":"b21-0020304","series-title":"Brain","first-page":"116","article-title":"Histiocytosis \u2018X\u2019 of the central nervous system. Clinical and pathological report of a case with predominant cerebellar involvement Childs","volume":"5","author":"Iraci","year":"1979"},{"key":"b22-0020304","series-title":"Rinsho Shinkeigaku","first-page":"725","article-title":"[Adult onset Langerhans cell histiocytosis with progressive cerebellar ataxia and spastic paraparesis.]","volume":"46","author":"Kobessho","year":"2006"},{"key":"b23-0020304","series-title":"Eur Radiol","doi-asserted-by":"publisher","first-page":"2074","DOI":"10.1007\/s00330-006-0200-y","article-title":"MRI features of neurodegenerative LCH","volume":"16","author":"Martin-Duverneuil","year":"2006"},{"key":"b24-0020304","series-title":"AJNR Am J Neuroradiol","first-page":"1193","article-title":"Hyperintense basal ganglia on T1-weighted MR in a patient with Langerhans Cell Histiocytosis","volume":"17","author":"Miaux","year":"1996"},{"key":"b25-0020304","series-title":"Pediatr Blood Cancer","doi-asserted-by":"publisher","first-page":"50","DOI":"10.1002\/pbc.20760","article-title":"CNS related permanent consequences in patients with Langerhans cell histiocytosis","volume":"48","author":"Mittheisz","year":"2007"},{"key":"b26-0020304","series-title":"Clin Endocrinol (Oxf)","doi-asserted-by":"publisher","first-page":"509","DOI":"10.1046\/j.1365-2265.2000.01125.x","article-title":"Growth and endocrine disorder in multisystem Langerhans' cell histiocytosis","volume":"53","author":"Nanduri","year":"2000"},{"key":"b27-0020304","series-title":"AJNR Am J Neuroradiol","first-page":"1921","article-title":"Demyelinating and gliotic cerebellar lesions in Langerhans cell histiocytosis","volume":"15","author":"Poe","year":"1994"},{"key":"b28-0020304","series-title":"Arch Dis Child","doi-asserted-by":"publisher","first-page":"130","DOI":"10.1136\/adc.86.2.130","article-title":"Acute ataxia complicating Langerhans cell histiocytosis","volume":"86","author":"Polizzi","year":"2002"},{"key":"b29-0020304","series-title":"AJNR Am J Neuroradiol","first-page":"880","article-title":"MR imaging presentation of intracranial disease associated with Langerhans cell histiocytosis","volume":"25","author":"Prayer","year":"2004"},{"key":"b30-0020304","series-title":"AJNR Am Neuroradiol","doi-asserted-by":"publisher","first-page":"1022","DOI":"10.3174\/ajnr.A0509","article-title":"Long term MR imaging course of neurodegenerative Langerhans cell histiocytosis","volume":"28","author":"Prosch","year":"2007"},{"key":"b31-0020304","series-title":"Pediatr Radiol","doi-asserted-by":"publisher","first-page":"433","DOI":"10.1007\/BF02075201","article-title":"Brain MR in patients with Langerhans cell histiocytosis: findings and enhancement with Gd-DTPA","volume":"20","author":"Rosenfield","year":"1990"},{"key":"b32-0020304","series-title":"J Child Neurol","doi-asserted-by":"publisher","first-page":"824","DOI":"10.1177\/088307380001501214","article-title":"Cerebellar involvement in Langerhans' cell histiocytosis: a progressive neuropsychiatric disease","volume":"15","author":"Shuper","year":"2000"},{"key":"b33-0020304","series-title":"J Child Neurol","doi-asserted-by":"publisher","first-page":"253","DOI":"10.1177\/08830738050200031501","article-title":"Modern Imaging Methods for the assessment of Langerhans' cell histiocytosis associated ND syndrome: case report","volume":"20","author":"Steiner","year":"2005"},{"key":"b34-0020304","series-title":"Arch Dis Child","doi-asserted-by":"publisher","first-page":"17","DOI":"10.1136\/adc.75.1.17","article-title":"A multicenter retrospective survey of Langerhan's cell hisitiocytosis: 348 cases observed between 1983 and 1993","volume":"75","author":"The French Langerhans Cell Histiocytosis Study Group","year":"1996"},{"key":"b35-0020304","series-title":"Med Pediatr Oncol","doi-asserted-by":"publisher","first-page":"108","DOI":"10.1002\/mpo.1178","article-title":"Pattern and course of single system disease in Langerhans cell histiocytosis data from the DAL HX 83 and 90 Study","volume":"37","author":"Titgemeyer","year":"2001"}],"container-title":["Journal of Neurosurgery: Pediatrics"],"original-title":[],"link":[{"URL":"https:\/\/thejns.org\/view\/journals\/j-neurosurg-pediatr\/2\/5\/article-p304.xml","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/thejns.org\/downloadpdf\/journals\/j-neurosurg-pediatr\/2\/5\/article-p304.xml","content-type":"text\/html","content-version":"vor","intended-application":"syndication"},{"URL":"https:\/\/thejns.org\/downloadpdf\/journals\/j-neurosurg-pediatr\/2\/5\/article-p304.xml","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,4,15]],"date-time":"2023-04-15T14:00:32Z","timestamp":1681567232000},"score":1,"resource":{"primary":{"URL":"https:\/\/thejns.org\/view\/journals\/j-neurosurg-pediatr\/2\/5\/article-p304.xml"}},"subtitle":["Case report"],"short-title":[],"issued":{"date-parts":[[2008,11]]},"references-count":35,"journal-issue":{"issue":"5"},"URL":"https:\/\/doi.org\/10.3171\/ped.2008.2.11.304","relation":{},"ISSN":["1933-0707","1933-0715"],"issn-type":[{"value":"1933-0707","type":"print"},{"value":"1933-0715","type":"electronic"}],"subject":[],"published":{"date-parts":[[2008,11]]}}}