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Neuro-ophthalmological manifestations of Behçet’s disease
  1. Ammr Alghamdi1,2,
  2. Bahram Bodaghi1,
  3. Chloé Comarmond2,
  4. Anne Claire Desbois2,
  5. Fanny Domont2,
  6. Bertrand Wechsler2,
  7. Raphael Depaz3,
  8. Phuc Le Hoang1,
  9. Patrice Cacoub2,
  10. Valérie Touitou1,
  11. David Saadoun2
  1. 1 Ophthalmology Department, Assistance Publique – Hôpitaux de Paris, Pitié Salpêtrière University Hospital, Pierre et Marie Curie University Paris VI, Paris, France
  2. 2 Internal Medicine and Clinical Immunology Department, Assistance Publique – Hôpitaux de Paris, Pitié Salpêtrière University Hospital, Pierre et Marie Curie University Paris VI, Paris, France
  3. 3 Neurology Department, Assistance Publique – Hôpitaux de Paris, Pitié Salpêtrière University Hospital, Pierre et Marie Curie University Paris VI, Paris, France
  1. Correspondence to Dr Valérie Touitou, Service d’Ophtalmologie Groupe Hospitalier Pitié-Salpêtrière, Paris 75013, France; vtouitou{at}gmail.com

Abstract

Background The neuro-ophthalmological manifestations of Behcet’s disease (BD) are rare, and data regarding their characteristics and outcome are lacking.

Objective To report prevalence, main characteristics and outcome of neuro-ophthalmological manifestations in BD.

Patients and methods This is a retrospective monocentric study of 217 patients diagnosed with neuro-Behçet’s disease (NBD), of whom 29 (13.3%) patients presented with neuro-ophthalmological manifestations (55% of men and mean±SD age of 26±8 years). All patients underwent a detailed ophthalmological examination and were followed up in the internal medicine and the ophthalmology departments.

Results Neuro-ophthalmological manifestations were the first presentation of BD in 45% of patients and developed later in the course of the disease in 55% of patients. They are divided into parenchymal (PM) and non-parenchymal (NPM)-related manifestations in, respectively, 13 patients (45%) and 16 patients (55%). PM included papillitis in seven patients (53.8%), retrobulbar optic neuritis in four patients (30.8%) and third cranial nerve palsy in two patients (15.4%). NPM included papilloedema related to cerebral venous thrombosis in all 16 patients, of whom 6 patients (37.5%) had sixth cranial nerve palsy. At initial examination, 93.1% of patients had visual alterations, including mainly decreased visual acuity visual field defects and/or diplopia. All patients were treated with corticosteroids and 79% of patients received immunosuppressive agents. After treatment, the visual outcome improved or stabilised in 66.7% of patients while it worsened in 33.3. The mean±SD logarithm of the minimum angle of resolution visual acuity improved from 0.4±0.3 at diagnosis to 0.2±0.3 after therapy. 10.3% and 3.4% of patients were legally blind at diagnosis and after therapy, respectively.

Conclusion Neuro-ophthalmological manifestations of BD represented 13% of NBD. They could be potentially severe and disabling. Prompt treatment is the key factor in improving visual outcome.

  • optic nerve
  • inflammation
  • imaging
  • immunology

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Footnotes

  • Contributors AA, BB, VT and DS: contributed to the conception of this study and designing it, as well as acquiring and analysing the data, writing the paper, revising it critically and approving its final version. CC, ACD, FD, BW, RD, PLH and PC: contributed to the conception and the design of this study, acquiring and analysing the data, as well as revising it critically and approving its final version. AA, BB, CC, ACD, FD, BW, RD, PLH, PC, VT and DS agree to be accountable for all aspects of the work.

  • 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 Not required.

  • Ethics approval The study was approved by the institutional review board of Pitié Salpêtrière University Hospital.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Data sharing statement There are no additional unpublished data from this study.

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