Bilateral Retrobulbar Optic Neuritis as the First Manifestation of Neuro-Behçet Disease

Abstract

Background. Behçet disease (BD) is a polygenic and chronic autoinflammatory multisystem vasculitis. Acute optic neuritis has been rarely reported in patients with BD, especially in children. Case Presentation. We reported an 8-year-old girl with a sudden visual loss and color vision impairment. The patient had a history of recurrent oral aphthous ulcers, genital ulcers, and chronic abdominal pain. On ophthalmic examination, anterior and posterior chambers and funduscopy of both eyes were normal. The results of laboratory tests for infectious and rheumatic diseases were normal. Brain magnetic resonance imaging and the result of cerebrospinal fluid analysis for oligoclonal bands and auto-antibodies were also normal. Pathergy skin test and human leukocyte antigens (HLA) B5 and HLA-B51 were positive. The patient was recognized as a case of BD-related bilateral retrobulbar optic neuritis and was treated by corticosteroids, azathioprine, colchicine, and infliximab. Conclusion. Retrobulbar optic neuritis may be the first manifestation of neuro-BD.

Diagnosis and classification of optic neuritis

“…UNKNOWN AETIOLOGY 1 0.39 Subgroups of optic neuritis from Delphi round #1-#12 not yet included at level 2 Amiodarone 138 ANCA+ 139,140 Anti-TNF-α 141,142 Associated with malformations Syndromic AZOOR [143][144][145] Behçet's 146 Cell mediated 147,148 GFAP autoantibody [149][150][151] GQ1b autoantibody [152][153][154] Giant cell arteritis 155 IgG4 156 MDEM 157 NMDA autoantibody [158][159][160] Optico-spinal optic neuritis [161][162][163] Paranasal sinusitis [164][165][166][167][168] Appendix Figure 17: Interim Classification for Delphi Rounds 12-22. This figure was then further developed incorporating the feed-back from the experts.…”

Episcleritis; and posterior uveitis misdiagnosed as orbital cellulitis in a child patient with Behçet’s disease

“…Anticardiolipin antibodies have been suggested to be associated with retinal vascular disease. 2,3 Genetics bases of BD demonstrate in different geographic areas; there are some HLA typing that are strongly related to BD. For example, HLA-A28 and HLA-B12 are elevated in American people.…”

“…Its manifestations can show themselves as anterior uveitis, posterior uveitis, retinal vasculitis, choroidal vasculitis, optic neuritis, and iridocyclitis. 2 Episcleritis is a rare ocular manifestation of this disease. In this report, we introduce an 11-year-old patient with episcleritis and posterior uveitis as the first manifestation of BD.…”

Optic neuritis classification in 2021

“…Few cases of ON associated with SLE, 51,52 sarcoïdosis, 95 GPA, 56 or Neuro-Behçet's disease 96 have been described in adolescents so clinician must keep in mind these differential diagnosis and apply the same diagnostic criteria as in adults described in the paragraph "secondary optic neuritis. "…”