{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,10,11]],"date-time":"2025-10-11T13:28:45Z","timestamp":1760189325074,"version":"3.44.0"},"reference-count":11,"publisher":"BMJ","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["BMJ Case Reports"],"abstract":"<jats:p>Erdheim-Chester disease (ECD) is a rare non-Langerhans form of histiocytosis with distinctive radiographic and pathological features. Intracranial involvement is further a rarity, usually producing diabetes insipidus or cerebellar-brainstem symptoms. We report a 40-year-old man presenting with recurrent secondarily generalised seizures. An MRI scan of the brain revealed multiple enhancing intracranial masses in frontal, temporal and parietal regions. Biopsy from the left frontotemporal lesion confirmed it to be a rare case of ECD. The patient received a short course of corticosteroids initially and subsequently remained well-controlled on antiepileptic therapy alone. A repeat MRI of his brain showed significant resolution of lesions. Osteolytic lesions in the skull vault were detected during follow-up which also disappeared. Interestingly, there was no involvement of long bones or any other system even after 12\u2005years of follow-up.<\/jats:p>","DOI":"10.1136\/bcr-2012-006823","type":"journal-article","created":{"date-parts":[[2013,4,16]],"date-time":"2013-04-16T01:34:27Z","timestamp":1366076067000},"page":"bcr2012006823","source":"Crossref","is-referenced-by-count":7,"title":["Erdheim-Chester disease with isolated craniocerebral involvement"],"prefix":"10.1136","volume":"2013","author":[{"given":"Rajendra Singh","family":"Jain","sequence":"first","affiliation":[{"name":"Department of Neurology, SMS Medical College Hospital, Jaipur, Rajasthan, India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Raghavendra Bakki","family":"Sannegowda","sequence":"additional","affiliation":[{"name":"Department of Neurology, SMS Medical College Hospital, Jaipur, Rajasthan, India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Rahul","family":"Jain","sequence":"additional","affiliation":[{"name":"Department of Neurology, SMS Medical College Hospital, Jaipur, Rajasthan, India"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tarun","family":"Mathur","sequence":"additional","affiliation":[{"name":"Department of Neurology, SMS Medical College Hospital, Jaipur, Rajasthan, India"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"239","published-online":{"date-parts":[[2013,4,15]]},"reference":[{"key":"2025082801102017000_2013.apr15_1.bcr2012006823.1","doi-asserted-by":"crossref","first-page":"586","DOI":"10.1148\/radiol.10090320","article-title":"Cerebral, facial, and orbital involvement in Erdheim-Chester disease: CT and MR findings","volume":"2013","author":"Drier","year":"2010","journal-title":"Radiology"},{"key":"2025082801102017000_2013.apr15_1.bcr2012006823.2","doi-asserted-by":"crossref","first-page":"2120","DOI":"10.1212\/WNL.0b013e3181c67bbc","article-title":"Characteristic brain MRI appearance of Erdheim-Chester disease","volume":"2013","author":"Bianco","year":"2009","journal-title":"Neurology"},{"key":"2025082801102017000_2013.apr15_1.bcr2012006823.3","doi-asserted-by":"crossref","first-page":"111","DOI":"10.1148\/radiol.2341021806","article-title":"Case 78: Erdheim-Chester disease with central nervous system involvement","volume":"2013","author":"Adem","year":"2005","journal-title":"Radiology"},{"key":"2025082801102017000_2013.apr15_1.bcr2012006823.4","doi-asserted-by":"crossref","first-page":"1207","DOI":"10.1097\/00005072-199711000-00005","article-title":"Pathology of the central nervous system in Chester-Erdheim disease: report of three cases","volume":"2013","author":"Adle-Biassette","year":"1997","journal-title":"J Neuropathol Exp Neurol"},{"key":"2025082801102017000_2013.apr15_1.bcr2012006823.5","doi-asserted-by":"crossref","first-page":"157","DOI":"10.1097\/00005792-199605000-00005","article-title":"Erdheim-Chester disease: clinical and radiologic characteristics of 59 cases","volume":"2013","author":"Veyssier-Belot","year":"1996","journal-title":"Medicine"},{"key":"2025082801102017000_2013.apr15_1.bcr2012006823.6","doi-asserted-by":"crossref","first-page":"3504","DOI":"10.1002\/art.27672","article-title":"Pulmonary involvement in Erdheim-Chester disease: a single-center study of thirty-four patients and a review of the literature","volume":"2013","author":"Arnaud","year":"2010","journal-title":"Arthritis Rheum"},{"key":"2025082801102017000_2013.apr15_1.bcr2012006823.7","doi-asserted-by":"crossref","first-page":"72","DOI":"10.1136\/jnnp.66.1.72","article-title":"Neurological manifestations of Erdheim-Chester disease","volume":"2013","author":"Wright","year":"1999","journal-title":"J Neurol Neurosurg Psychiatry"},{"key":"2025082801102017000_2013.apr15_1.bcr2012006823.8","first-page":"134","article-title":"Erdheim-Chester disease mimicking multiple meningioma syndrome","volume":"2013","author":"Johnsona","year":"2004","journal-title":"Am J Neuroradiol"},{"key":"2025082801102017000_2013.apr15_1.bcr2012006823.9","doi-asserted-by":"crossref","first-page":"1267","DOI":"10.1007\/s00415-006-0160-9","article-title":"Neurological manifestations and neuroradiological presentation of Erdheim-Chester disease: report of 6 cases and systematic review of the literature","volume":"2013","author":"Lachenal","year":"2006","journal-title":"J Neurol"},{"key":"2025082801102017000_2013.apr15_1.bcr2012006823.10","doi-asserted-by":"crossref","first-page":"1115","DOI":"10.3171\/jns.2004.100.6.1115","article-title":"Erdheim-Chester disease mimicking a primary brain tumor. 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