title: IgG4-related inflammatory pseudotumor of the central nervous system responsive to mycophenolate mofetil.
abstract: Orbital apex and skull base masses often present with neuro-ophthalmic signs and symptoms. Though the localization of these syndromes and visualization of the responsible lesion on imaging is typically straightforward, definitive diagnosis usually relies on biopsy. Immunohistochemistry is important for categorization and treatment planning. IgG4-related disease is emerging as a pathologically defined inflammatory process that can occur in multiple organ systems. We present two patients with extensive inflammatory mass lesions of the central nervous system with immunohistochemistry positive for IgG4 and negative for ALK-1 as examples of meningeal based IgG4-related inflammatory pseudotumors. In both patients, there was treatment response to mycophenolate mofetil.
authors.affiliation: Department of Neurology, University of Pennsylvania, Philadelphia, PA, USA. hemoss@uic.edu
authors.forename: Heather E
authors.initials: HE
authors.lastname: Moss
authors.forename: Luis J
authors.initials: LJ
authors.lastname: Mejico
authors.forename: Gustavo
authors.initials: G
authors.lastname: de la Roza
authors.forename: Thomas M
authors.initials: TM
authors.lastname: Coyne
authors.forename: Steven L
authors.initials: SL
authors.lastname: Galetta
authors.forename: Grant T
authors.initials: GT
authors.lastname: Liu
mesh_terms: D000328:Adult; D002490:Central Nervous System / Q000276:immunology / Q000473:pathology*; D005260:Female; D006104:Granuloma, Plasma Cell / Q000188:drug therapy* / Q000473:pathology*; D006801:Humans; D007074:Immunoglobulin G / Q000096:biosynthesis*; D007166:Immunosuppressive Agents / Q000008:administration & dosage; D008581:Meningitis / Q000188:drug therapy* / Q000473:pathology; D008875:Middle Aged; D009173:Mycophenolic Acid / Q000008:administration & dosage / Q000031:analogs & derivatives*
chemical_list: D007074:Immunoglobulin G; D007166:Immunosuppressive Agents; D009173:Mycophenolic Acid