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2 results

  • IgG4-Related Disease of the Central Nervous System: A Case Series

  • Ghada Abbas, Jason Karamchandani, Anthony Ciarallo, Liam Durcan
  • Journal:
    Canadian Journal of Neurological Sciences / Volume 50 / Issue 6 / November 2023
    Published online by Cambridge University Press:
    11 November 2022, pp. 907-913
    • Article
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  • IgG4-related disease (IgG4-RD) is a rare and often misdiagnosed disorder with limited literature that highlights the different neurological presentations of this treatable disease. The diagnosis of IgG4-RD could be challenging, while imaging is fundamental for the diagnosis, biopsy is considered the gold standard. Most cases respond well to steroids and immunosuppressive therapy. This is a case series study that illustrates the varied neurological presentations of IgG4-RD through three different patients that were followed at the Montreal Neurological Institute. This paper takes you through the diagnostic strategy that we followed to accurately diagnose and treat those patients.

  • Cochleovestibular nerve involvement in multifocal fibrosclerosis

  • R K Tsuji, A G Bittencourt, M H Arai, E M Santiago Gebrim
  • Journal:
    The Journal of Laryngology & Otology / Volume 126 / Issue 9 / September 2012
    Published online by Cambridge University Press:
    05 July 2012, pp. 935-938
    Print publication:
    September 2012
  • Objectives:

    To report a case of multifocal fibrosclerosis with a nine-year follow up, and to discuss this disease's radiological appearance and management. The disease is a rare systemic disorder of unknown cause characterised by fibrous proliferation involving multiple anatomical sites.

    Case report:

    A 50-year-old woman presented with histological findings characterised by similar inflammatory processes involving the meninges, pituitary gland, peritoneum, retroperitoneum and orbits, prompting a search for a common pathophysiology. A diagnosis of multifocal fibrosclerosis was postulated. Symptom improvement was noted after treatment with prednisone and azathioprine.

    Conclusion:

    This is the first documented case of involvement of the cochleovestibular nerve in a patient with multifocal fibrosclerosis. The rare association between fibrotic diseases and masses showing various clinical patterns should be kept in mind by otolaryngologists, and imaging performed to investigate for multifocal fibrosclerosis. However, diagnosis can only be confirmed with tissue biopsy and histopathological examination.