IgG4-related disease (IgG4RD) with intracranial involvement is rare. We report a 56-year-old male who had an excellent response to rituximab and dexamethasone after going undiagnosed for 5 years. After 3 years of rituximab maintenance, he has no evidence of disease on brain MRI.
Keywords: IgG4 disease; headache; lymphoproliferative disorder; maintenance rituximab; pachymeningitis.
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