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Pathological analysis reveals lymphoplasmacytic infiltrate rich in IgG4-positive cells and characteristic storiform fibrosis. Early treatment with glucocorticoids may prevent progression to poorly responsive fibrotic disease.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>OBSERVATIONS<\/jats:title>\n                    <jats:p>A 63-year-old female patient presented with reports of left-sided headaches, nausea, and photophobia in addition to recently diagnosed chronic rhinosinusitis (CRS). Neurological examination revealed dysarthria secondary to left hypoglossal nerve palsy. Computed tomography (CT) revealed a contrast-enhancing extraaxial mass at the left craniocervical junction, CRS with secondary hyperostotic reaction, and multiple hypodense lesions involving the occipital bone. Magnetic resonance imaging revealed a dural-based lesion involving the foramen magnum and invading the left hypoglossal canal. The patient underwent a far-lateral craniotomy. Histopathological analysis revealed severe lymphoplasmacytic inflammation, storiform fibrosis and rich plasma-cell population positive for IgG4. Serum IgG4 was markedly elevated. Total-body CT showed no systemic involvement. The patient was diagnosed with IgG4-RD and was prescribed prednisone, with normalization of her IgG4 levels after 1 month.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>LESSONS<\/jats:title>\n                    <jats:p>IgG4-RD may mimic a variety of diseases, including skull-base meningiomas and CRS. Accurate diagnosis and expedited administration of steroids may prevent unnecessary interventions and progression to treatment-resistant fibrosis.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.3171\/case21398","type":"journal-article","created":{"date-parts":[[2021,12,6]],"date-time":"2021-12-06T02:05:37Z","timestamp":1638756337000},"source":"Crossref","is-referenced-by-count":4,"title":["IgG4-related pachymeningitis masquerading as foramen magnum meningioma: illustrative case"],"prefix":"10.3171","volume":"2","author":[{"given":"Haggai","family":"Suisa","sequence":"first","affiliation":[{"name":"Department of Neurosurgery, Galilee Medical Center, Nahariya, Israel; and"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Jean Francois","family":"Soustiel","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Galilee Medical Center, Nahariya, Israel; and"},{"name":"The Azrieli Faculty of Medicine, Bar-Ilan University, Safed, Israel"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yuval","family":"Grober","sequence":"additional","affiliation":[{"name":"Department of Neurosurgery, Galilee Medical Center, Nahariya, Israel; and"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"1754","reference":[{"issue":"6","key":"b1","series-title":"N Engl J Med","doi-asserted-by":"publisher","first-page":"539","DOI":"10.1056\/NEJMra1104650","article-title":"IgG4-related disease","volume":"366","author":"Stone JH","year":"2012","unstructured":"Stone JH, Zen Y, Deshpande V. 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