{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,1,9]],"date-time":"2026-01-09T21:20:55Z","timestamp":1767993655248,"version":"3.49.0"},"reference-count":15,"publisher":"Journal of Neurosurgery Publishing Group (JNSPG)","issue":"3","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JNS"],"published-print":{"date-parts":[[2012,9]]},"abstract":"<jats:p>Rosai-Dorfman disease (RDD) is a rare non-Langerhans histiocytosis that usually presents with lymphadenopathy. Although isolated involvement of the CNS was considered to be uncommon, numerous cases have been reported in recent years. For RDD of the CNS, the treatment consists, in general, of surgery. In cases of partial resection or relapse, chemotherapy regimens, corticosteroids, and\/or radiotherapy have yielded negative results. The authors describe the case of a 57-year-old man with a history of chronic Q fever who presented with aphasia and partial seizure. Computed tomography of the brain revealed a left frontotemporal lesion that was suggestive of a meningioma. The lesion was partially resected and histopathological evaluation revealed the presence of RDD. Nineteen months later, a Jacksonian seizure prompted MRI evaluation, which disclosed a local recurrence of the tumor. Computed tomography and FDG-PET demonstrated that the RDD involved no other site, but the presence of ileitis, noted on ileoscopy, led to the diagnosis of Crohn disease. Treatment with the purine analog azathioprine was initiated, leading to an objective and sustained response in both the RDD tumor and ileitis over 35 months of follow-up. This case report highlights the potential use of a purine analog in cases of relapsing RDD of the CNS and a possible common defect of macrophage regulation in RDD, Crohn disease, and Q fever.<\/jats:p>","DOI":"10.3171\/2012.5.jns12148","type":"journal-article","created":{"date-parts":[[2012,6,22]],"date-time":"2012-06-22T20:32:50Z","timestamp":1340397170000},"page":"486-489","source":"Crossref","is-referenced-by-count":22,"title":["Successful treatment with azathioprine of relapsing Rosai-Dorfman disease of the central nervous system"],"prefix":"10.3171","volume":"117","author":[{"given":"Guillaume","family":"Le Guenno","sequence":"first","affiliation":[{"name":"Departments of Internal Medicine and"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Lionel","family":"Galicier","sequence":"additional","affiliation":[{"name":"Department of Clinical Immunology, H\u00f4pital Saint Louis, Assistance Publique\u2013H\u00f4pitaux de Paris; and"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Emmanuelle","family":"Uro-Coste","sequence":"additional","affiliation":[{"name":"Department of Pathology, H\u00f4pital du Rangueil, Toulouse, France"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Virginie","family":"Petitcolin","sequence":"additional","affiliation":[{"name":"Radiology, H\u00f4pital Estaing, Clermont-Ferrand;"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Virginie","family":"Rieu","sequence":"additional","affiliation":[{"name":"Departments of Internal Medicine and"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Marc","family":"Ruivard","sequence":"additional","affiliation":[{"name":"Departments of Internal Medicine and"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1754","reference":[{"key":"b1-jns12148","series-title":"Neurol Res","first-page":"572","article-title":"Diagnosis and management of Rosai-Dorfman disease involving the 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macrophages","volume":"206","author":"Casanova","year":"2009"},{"key":"b6-jns12148","series-title":"Semin Diagn Pathol","first-page":"19","article-title":"Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): review of the entity","volume":"7","author":"Foucar","year":"1990"},{"key":"b7-jns12148","series-title":"Med Pediatr Oncol","first-page":"187","article-title":"Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): response to methotrexate and mercaptopurine","volume":"27","author":"Horneff","year":"1996"},{"key":"b8-jns12148","series-title":"Pathol Int","first-page":"396","article-title":"Isolated intracranial Rosai-Dorfman disease: case report and literature review","volume":"48","author":"Huang","year":"1998"},{"key":"b9-jns12148","series-title":"Int J Surg Pathol","first-page":"285","article-title":"Rosai-Dorfman disease: successful long-term results by combination chemotherapy with prednisone, 6-mercaptopurine, methotrexate, and vinblastine: a case report","volume":"13","author":"Jabali","year":"2005"},{"key":"b10-jns12148","series-title":"J Leukoc Biol","first-page":"936","article-title":"Macrophage colony stimulatory factor-activated bone marrow macrophages suppress lymphocytic responses through phagocytosis: a tentative in vitro model of Rosai-Dorfman disease","volume":"57","author":"Jadus","year":"1995"},{"key":"b11-jns12148","series-title":"Br J Dermatol","first-page":"213","article-title":"Dramatic efficiency of pegylated interferon in sinus histiocytosis with massive lymphadenopathy","volume":"164","author":"Le Guenno","year":"2011"},{"key":"b12-jns12148","series-title":"J Hand Surg Am","first-page":"1409","article-title":"Cutaneous Rosai-Dorfman disease of the forearm: case report","volume":"33","author":"Potts","year":"2008"},{"key":"b13-jns12148","series-title":"Am J Hematol","first-page":"67","article-title":"Treatment of sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): 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