A Case of Definite IgG4-Related Pachymeningitis

Immunoglobulin G (IgG)4-related disease is a recently defined disease entity that is characterized by elevated serum IgG4 levels and abundant infiltration of IgG4-positive plasma cells and high-grade sclerosis of the affected region.1 This disease includes autoimmune pancreatitis, Mikulicz disease, pseudotumor of the lung, tubulointerstitial nephritis, and Riedel thyroiditis,2 but the only CNS involvement that has been reported so far is...