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A difficult case
Rituximab for corticosteroid-resistant relapsing IgG4-related intracranial pachymeningitis: report of two cases
  1. Arthur Mageau1,
  2. Natalia Shor2,
  3. Matthieu Fisselier1,
  4. Jennifer Aboab1,
  5. Augustin Lecler2,
  6. Celeste Rebours3,
  7. Homa Adle-Biassette4,
  8. Olivier Gout1,
  9. Romain Deschamps1
  1. 1 Department of Neurology, Fondation Ophtalmologique Adolphe de Rothschild, Paris, France
  2. 2 Department of Radiology, Fondation Ophtalmologique Adolphe de Rothschild, Paris, Île-de-France, France
  3. 3 Department of Otorhinolaryngology-Head and Neck Surgery, Fondation Ophtalmologique Adolphe de Rothschild, Paris, France
  4. 4 Service d’Anatomie et Cytologie Pathologiques Hôpital Lariboisière, Université Paris Diderot, Sorbonne Paris Cité, Paris, France
  1. Correspondence to Dr Arthur Mageau, Department of Neurology, Fondation Ophtalmologique Adolphe de Rothschild, 75019 Paris, France; arthur_mageau{at}hotmail.fr

Abstract

IgG4-related disease is now recognised as an important cause of intracranial and spinal hypertrophic pachymeningitis. Treatment with corticosteroids generally leads to significant clinical improvement. We present two cases of IgG4 pachymeningitis unresponsive to corticosteroids who improved with rituximab.

  • neuroimmunology
  • neuroophthalmology

http://dx.doi.org/10.1136/practneurol-2017-001826

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    Footnotes

    • Contributors AM and RD were responsible for the planning, conducting and reporting of the study. NS, MF and AL were responsible for the reporting. JA, HA-B and CR were involved in the conducting. OG did the planning.

    • Competing interests None declared.

    • Patient consent Obtained.

    • Provenance and peer review Not commissioned; externally peer reviewed. This paper was reviewed by Neil Scolding, Bristol, UK.

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