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Intracranial spread of IgG4-related disease via skull base foramina
  1. C M Rice1,2,
  2. T Spencer1,
  3. G Bunea3,
  4. N J Scolding1,2,
  5. P Sloan4,
  6. U Nath5
  1. 1Department of Neurology, North Bristol NHS Trust, Bristol, UK
  2. 2Clinical Neuroscience, School of Clinical Sciences, University of Bristol, Bristol, UK
  3. 3Department of Radiology, Sunderland Royal Hospital, Sunderland, UK
  4. 4Department of Pathology, Royal Victoria Infirmary, Newcastle upon Tyne, UK
  5. 5Department of Neurology, Sunderland Royal Hospital, Sunderland, UK
  1. Correspondence to Dr Claire Rice, Department of Neurology, Southmead Hospital, Bristol, BS10 5NB, UK; c.m.rice{at}bristol.ac.uk

Abstract

IgG4-related disease (IgG4-RD) is a newly recognised, multiorgan, inflammatory disease, and its full clinical spectrum remains undefined. We present a biopsy-proven case of IgG4-RD presenting with a parapharyngeal mass with intracranial extension and possible involvement of the brain parenchyma. We highlight the importance of considering the diagnosis in those presenting with tumefactive lesions, leptomeningitis or pachymeningitis and emphasise the value of securing a tissue diagnosis so that appropriate long-term treatment can be instigated and complications avoided.

  • IgG4-related disease
  • NEUROIMMUNOLOGY
  • dacryosialoadenopathy
  • pachymeningitis
  • leptomeningitis

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