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IgG4-related disease: a rare but treatable cause of refractory intracranial hypertension
  1. Thomas Williams,
  2. Monica Marta,
  3. Gavin Giovannoni
  1. Queen Mary University of London, Blizard Institute, Barts and The London School of Medicine and Dentistry, London, UK
  1. Correspondence to Dr Monica Marta, Neuroimmunology Unit, Neuroscience and Trauma Centre, Blizard Institute, Queen Mary University London, 4 Newark Street, Whitechapel, London E1 2AT, UK; m.calado-marta{at}


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.

  • IgG4-Related Disease
  • Hypertrophic Pachymeningitis
  • Intracranial Hypertension
  • Rituximab

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