title: IgG4-related disease: a rare but treatable cause of refractory intracranial hypertension.
abstract: Hypertrophic pachymeningitis secondary to IgG4-related disease is a rare but sometimes devastating cause of intracranial hypertension. It has the potential for an excellent response to corticosteroids or rituximab. We discuss the clinical presentation, imaging, histology (with its difficult distinction from lymphoma), management and follow-up of a case, including relapse and re-treatment following an initial response to rituximab.
authors.affiliation: Queen Mary University of London, Blizard Institute, Barts and The London School of Medicine and Dentistry, London, UK.
authors.forename: Thomas
authors.initials: T
authors.lastname: Williams
authors.affiliation: Queen Mary University of London, Blizard Institute, Barts and The London School of Medicine and Dentistry, London, UK.
authors.forename: Monica
authors.initials: M
authors.lastname: Marta
authors.affiliation: Queen Mary University of London, Blizard Institute, Barts and The London School of Medicine and Dentistry, London, UK.
authors.forename: Gavin
authors.initials: G
authors.lastname: Giovannoni
mesh_terms: D000328:Adult; D005260:Female; D006801:Humans; D006984:Hypertrophy / Q000175:diagnosis* / Q000276:immunology; D007074:Immunoglobulin G; D019586:Intracranial Hypertension / Q000175:diagnosis* / Q000276:immunology; D008581:Meningitis
chemical_list: D007074:Immunoglobulin G
keywords: Hypertrophic Pachymeningitis; IgG4-Related Disease; Intracranial Hypertension; Rituximab