DOI 10.5414/NP300575

Clinical Neuropathology, Volume 32 - July/August (291 - 297)

Probable IgG4-related pachymeningitis: a case with transverse sinus obliteration

Jantima Tanboon1, Michelle Madden Felicella2, Juan Bilbao3, Todd Mainprize4, Arie Perry2
1 Department of Pathology, Faculty of Medicine, Siriraj Hospital, Mahidol University, Bangkok, 2 Department of Pathology, Division of Neuropathology, University of California, San Francisco (UCSF), San Francisco, CA, USA, 3 Department of Pathology, 4 Department of Neurosurgery, Sunnybrook Hospital, University of Toronto, Sunnybrook Health Sciences Centre, Toronto, ON, Canada

Abstract

IgG4-related disease (IgG4-RD) is a recently recognized fibro-inflammatory condition which often shows a dramatic response to steroid therapy. IgG4-RD can present either as a single lesion or as a systemic multi-organ disorder. Common histological findings include a dense lymphoplasmacytic infiltrate rich in IgG4-positive plasma cells, storiform fibrosis, and phlebitis. Although diagnostic criteria for IgG4-RD have been proposed in many organs/sites, they are not well established in the central nervous system. Published data on IgG4-RD in meninges is also limited. To our knowledge, only 15 potential cases of meningeal IgG4-RD have been reported. We add a case of probable IgG4-related pachymeningitis in a 42-year-old woman who presented with headache and left transverse sinus obstruction. Follow-up after 2-months of high dose steroids shows dramatic clinical and imaging improvement. The differential diagnosis for IgG4-related pachymeningitis, including lymphoplasmacyte-rich meningioma, idiopathic


hypertrophic pachymeningitis, and lymphoproliferative disease is discussed.

Author Details

Authors

  • Jantima Tanboon1
  • Michelle Madden Felicella2
  • Juan Bilbao3
  • Todd Mainprize4
  • Arie Perry2

Departments

  • 1 Department of Pathology, Faculty of Medicine, Siriraj Hospital, Mahidol University, Bangkok,
  • 2 Department of Pathology, Division of Neuropathology, University of California, San Francisco (UCSF), San Francisco, CA, USA,
  • 3 Department of Pathology,
  • 4 Department of Neurosurgery, Sunnybrook Hospital, University of Toronto, Sunnybrook Health Sciences Centre, Toronto, ON, Canada

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