J Korean Neurol Assoc.
2018 Aug;36(3):207-209. 10.17340/jkna.2018.3.14.
IgG4-Related Hypertrophic Pachymeningitis Mimicking Cerebral Venous Thrombosis
- Affiliations
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- 1Department of Neurology, Hanyang University College of Medicine, Seoul, Korea. hyoungkim1@hanyang.ac.kr
- 2Department of Pathology, Hanyang University College of Medicine, Seoul, Korea.
- KMID: 2436080
- DOI: http://doi.org/10.17340/jkna.2018.3.14
Abstract
- Immunoglobulin G4-related hypertrophic pachymeningitis (IgG4-RHP) is an increasingly recognized manifestation of IgG4-related disease (IgG4-RD), which is a fibroinflammatory condition that can affect virtually any organ. The three hallmark histopathological features of IgG4-RD are lymphoplasmacytic infiltration of IgG4-positive plasma cells, storiform fibrosis, and obliterative phlebitis. We report a case of biopsy-confirmed IgG4-RHP that was initially misdiagnosed as cerebral venous thrombosis.
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