A 58-year-old female patient presented with a 4-year history of right-sided headache with tinnitus. She had been hospitalized previously due to dysfunction of multiple cranial nerves, such as diplopia, right hemiface hypoesthesia, right peripheral facial palsy, right hearing loss, dysphonia, and dysphagia, followed by venous thrombosis of the right sigmoid sinus (Figure 1), which was treated with warfarin. The cerebrospinal fluid yielded 90 leukocytes/mm3 (92% of lymphocytes) and 158 mg/dL of proteins. A magnetic resonance imaging (MRI) scan revealed tentorium-temporo-parietal hypertrophic pachymeningitis (Figure 2). A meningeal biopsy demonstrated dense lymphoplasmacytic infiltrate and storiform fibrosis (Figure 3), two of the three histopathological criteria.11 De Virgilio A, de Vincentiis M, Inghilleri M, et al. Idiopathic hypertrophic pachymeningitis: an autoimmune IgG4-related disease. Immunol Res 2017;65(01):386–394. Doi: 10.1007/s12026-016-8863-1
https://doi.org/10.1007/s12026-016-8863-...
,22 Deshpande V, Zen Y, Chan JK, et al. Consensus statement on the pathology of IgG4-related disease. Mod Pathol 2012;25(09): 1181–1192. Doi: 10.1038/modpathol.2012.72
https://doi.org/10.1038/modpathol.2012.7...
Therefore, the diagnosis was made, and rituximab33 Lu LX, Della-Torre E, Stone JH, Clark SW. IgG4-related hypertrophic pachymeningitis: clinical features, diagnostic criteria, and treatment. JAMA Neurol 2014;71(06):785–793. Doi: 10.1001/jamaneurol.2014.243
https://doi.org/10.1001/jamaneurol.2014....
,44 Perugino CA, Stone JH. IgG4-related disease: an update on pathophysiology and implications for clinical care. Nat Rev Rheumatol 2020;16(12):702–714. Doi: 10.1038/s41584-020-0500-7
https://doi.org/10.1038/s41584-020-0500-...
was initiated, with a very important response in terms of symptoms and imaging exams (Figure 4).
T2 (upper) and postcontrast T1 (bottom) weighted magnetic resonance imaging (MRI) scans, showing dural thickening on coronal view before treatment.
References
-
1De Virgilio A, de Vincentiis M, Inghilleri M, et al. Idiopathic hypertrophic pachymeningitis: an autoimmune IgG4-related disease. Immunol Res 2017;65(01):386–394. Doi: 10.1007/s12026-016-8863-1
» https://doi.org/10.1007/s12026-016-8863-1 -
2Deshpande V, Zen Y, Chan JK, et al. Consensus statement on the pathology of IgG4-related disease. Mod Pathol 2012;25(09): 1181–1192. Doi: 10.1038/modpathol.2012.72
» https://doi.org/10.1038/modpathol.2012.72 -
3Lu LX, Della-Torre E, Stone JH, Clark SW. IgG4-related hypertrophic pachymeningitis: clinical features, diagnostic criteria, and treatment. JAMA Neurol 2014;71(06):785–793. Doi: 10.1001/jamaneurol.2014.243
» https://doi.org/10.1001/jamaneurol.2014.243 -
4Perugino CA, Stone JH. IgG4-related disease: an update on pathophysiology and implications for clinical care. Nat Rev Rheumatol 2020;16(12):702–714. Doi: 10.1038/s41584-020-0500-7
» https://doi.org/10.1038/s41584-020-0500-7
Publication Dates
-
Publication in this collection
19 Feb 2024 -
Date of issue
2024
History
-
Received
23 Feb 2023 -
Reviewed
26 Apr 2023 -
Accepted
19 Apr 2023