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Neuro-inflammation
Review
Immune-mediated conditions affecting the brain, eye and ear (BEE syndromes)
  1. James D Triplett1,
  2. Katherine A Buzzard2,
  3. Michal Lubomski1,
  4. D Sean Riminton1,
  5. Michael H Barnett3,
  6. Miriam S Welgampola3,
  7. G Michael Halmagyi3,
  8. MaiAnh Nguyen1,
  9. Klara Landau4,
  10. Andrew G Lee5,6,7,
  11. Gordon T Plant8,
  12. Clare L Fraser9,
  13. Stephen W Reddel1,
  14. Todd A Hardy1
  1. 1 Neuroimmunology Clinic, Concord Hospital, University of Sydney, Sydney, New South Wales, Australia
  2. 2 Department of Neurosciences, Eastern Health, Monash University, Clayton, Victoria, Australia
  3. 3 Department of Neurology, Royal Prince Alfred Hospital, University of Sydney, Sydney, New South Wales, Australia
  4. 4 Department of Ophthalmology, University Hospital Zurich and University of Zurich, Zurich, Switzerland
  5. 5 Department of Ophthalmology, Blanton Eye Institute, Houston Methodist Hospital in Houston, Houston, Texas, USA
  6. 6 Weill Cornell Medical College, Cornell University, New York City, New York, USA
  7. 7 University of Texas Medical Branch (UTMB), Baylor College of Medicine, The UT MD Anderson Cancer Center, Texas A and M College of Medicine (AGL), Houston, Texas, USA
  8. 8 National Hospital for Neurology and Neurosurgery and Moorfield’s Eye Hospital, University College London, London, UK
  9. 9 Brain and Mind Centre, University of Sydney, Syndey, New South Wales, Australia
  1. Correspondence to Dr Todd A Hardy, Neuroimmunology Clinic, Concord Hospital, University of Sydney, Sydney NSW 2006, Australia; thardy{at}med.usyd.edu.au

Abstract

The triad of central nervous system symptoms, visual disturbance and hearing impairment is an oft-encountered clinical scenario. A number of immune-mediated diseases should be considered among the differential diagnoses including: Susac syndrome, Cogan syndrome or Vogt-Koyanagi-Harada disease; demyelinating conditions such as multiple sclerosis or neuromyelitis optica spectrum disorder; systemic diseases such as systemic lupus erythematosus, Sjögren syndrome or Behcet disease and granulomatous diseases such as sarcoidosis. In this article, we coin the term ‘BEE syndromes’ to draw attention to the various immune-mediated diseases that affect the brain, eye and ear. We present common disease manifestations and identify key clinical and investigation features.

  • Susac
  • Cogan
  • Vogt koyanagi harada
  • Behcet
  • Sjögren

https://doi.org/10.1136/jnnp-2018-319002

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    Footnotes

    • Contributors JT: undertook research and drafting of manuscript, identification of images and conceptualisation of the article. KB, ML: undertook research and drafting of manuscript. DR, MH, MN, KL, CF, SW: undertook redrafting of the article and identification of images. MW, AL, GP: undertook redrafting of the article. TH: undertook the conceptualisation of the article, reviewed article and supervised research.

    • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

    • Competing interests None declared.

    • Patient consent for publication Obtained.

    • Provenance and peer review Commissioned; externally peer reviewed.