Neuro-ophthalmological manifestations of Behçet’s disease

2018 ◽  
Vol 103 (1) ◽  
pp. 83-87 ◽  
Author(s):  
Ammr Alghamdi ◽  
Bahram Bodaghi ◽  
Chloé Comarmond ◽  
Anne Claire Desbois ◽  
Fanny Domont ◽  
...  

BackgroundThe neuro-ophthalmological manifestations of Behcet’s disease (BD) are rare, and data regarding their characteristics and outcome are lacking.ObjectiveTo report prevalence, main characteristics and outcome of neuro-ophthalmological manifestations in BD.Patients and methodsThis 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.ResultsNeuro-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.ConclusionNeuro-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.

Author(s):  
Narumon Keorochana ◽  
Nathamon Homchampa ◽  
Sritatath Vongkulsiri ◽  
Raveewan Choontanom

Abstract Purpose To determine the application of fluorescein angiographic (FA) findings and Behcet’s disease ocular attack score 24 (BOS24) scoring system in predicting poor visual outcome in patients with ocular Behcet’s disease. Study design Retrospective cohort study. Methods We included 73 eyes of 38 patients with ocular Behcet’s disease who underwent FA and reviewed FA images, anterior chamber cells, vitreous opacity, retinal and optic disc lesions, which are parameters in BOS24. The correlation between FA findings, BOS24, and visual acuity was assessed. Results Optic disc hyperfluoresence (74%), diffuse posterior pole leakage (52%) and diffuse peripheral leakage (52%) were the three most common findings. Common complications were peripheral capillary nonperfusion (29%), arterial narrowing (22%), and macular ischemia (19%). BOS24 scores of ≥ 6 (p < 0.0001), arterial narrowing (p < 0.0001), and severe posterior pole leakage (p = 0.004) were significantly associated with poor visual outcome. Combining significant FA findings: arterial narrowing and severe posterior pole leakage, to BOS24 ≥ 6 results in an increased relative risk of developing poor visual acuity from 7.30 to 10.43 and 1.89 to 2.02 respectively. Conclusion Fluorescein angiography is an important investigation for predicting poor visual outcome. BOS24 may be a useful alternative when fluorescein angiographic is unavailable.


2012 ◽  
Vol 69 (2) ◽  
pp. 168-174 ◽  
Author(s):  
Gordana Zlatanovic ◽  
Svetlana Jovanovic ◽  
Dragan Veselinovic ◽  
Maja Zivkovic

Bacground/Aim. Behcet's disease is genetically conditioned, immune-mediated multisystem occlusive vasculitis of small blood vessels, espesially venules, of unknown etiology. The aim of this study was to analyze the clinical features, disease activity and therapy of the patients with ophthalmologic manifestation of Behcet's disease. Methods. In this study symptoms and signs of the disease were analyzed both prospectively and retrospectively during the active manifestation of the disease. The diagnosis was reached according to the International Criteria for Behcet's Disease (2006). The treatment effects were evaluated based on the presence of the best corrected visual acuity and the inflammation of the vitreous humour before and after the application of our therapeutic method. The applied therapeutic modality consisted of the primary application of corticosteroid therapy in the active stage of the disease complemented with the choice of drugs from the immunosuppressive group. In this study there drugs were cyclosporine or methotrexate. A treatment refractory patients with poor vision prognosis were treated with a third drug, the biological preparation infliximab, a tumor necrosis factor-alpha (TNF-?) antagonist. Results. The mean age of 11 patients with ophthalmologic manifestation of Behcet's disease was 50.6 years. HLA B-5(51) was positive in 81% of the patients while 36% of the patients had positive pathergy test. Changes in affected eyes included vitritis (100%), posterior uveitis (45%), panuveitis (54%), retinal vasculitis (54%), cystoid macular edema (54%), and cystoid degeneration (18%). Increased intraocular pressure was observed in 27% of the patients. There was no statistically significant variation in disease activity parameters in any of the patients (p > 0.05). A statistically significant improvement in visual acuity (p < 0.05) and a high statistically significant decrease of inflammation of the worst affected eyes (p = 0.001) were detected. Conclusion. Our therapeutic method is useful for producing the optimal therapeutic plan for the acute - chronic stage of the difficult ophtamological manifestation of Behcet's desease as well as the prevention of relapse. However the high cost of the therapy and the potential complications should be taken into consideration when prescribing this therapy, especially a TNF-? antagonist.


1993 ◽  
Vol 14 ◽  
pp. 47s
Author(s):  
KF. Tabbara ◽  
PS. Chavis ◽  
S. Al Balla ◽  
AK. Al-Momen

1995 ◽  
Vol 3 (2) ◽  
pp. 99-106 ◽  
Author(s):  
J. Palmares ◽  
J. Castro-correia ◽  
M. F. Coutinho ◽  
D. Araújo ◽  
L. Delgado

2020 ◽  
Vol 11 (3) ◽  
pp. 516-522
Author(s):  
Gilad Rabina ◽  
Gil Amarilyo ◽  
Dinah Zur ◽  
Liora Harel ◽  
Zohar Habot-Wilner

We describe a case of Behçet’s disease (BD) in a young child that presented with recurrent neuroretinitis and developed retinal lesions during follow-up. A 4.5-year-old girl presented with fever of 39.5°C, erythema nodosum in her legs, bilateral knee arthritis, and perineum aphthae. On ocular examination, visual acuity was 20/25 in both eyes. Right eye examination was normal and the left eye (LE) showed mild anterior and intermediate uveitis, normal optic disc, and a macular star appearance. Laboratory workup demonstrated elevated C-reactive protein levels, a normal abdominal ultrasound, and a normal colonoscopy. The patient was diagnosed with BD. One month post initial presentation, the patient presented with visual acuity of finger counting in the LE with significant anterior uveitis, mild intermediate uveitis, and recurrent neuroretinitis. Under treatment of IV methylprednisolone, oral betamethasone, infliximab, and colchicine, a complete systemic remission was noticed, and uveitis became quiescent. On last examination, 4.5 years post first presentation, visual acuity was 20/25 in both eyes and the LE demonstrated a remnant of a juxtafoveal retinal scar. To the best of our knowledge, this is the first case of neuroretinitis presenting as a manifestation of pediatric BD. Ophthalmologists should be aware of these unique manifestations of ocular BD.


Eye ◽  
2009 ◽  
Vol 23 (11) ◽  
pp. 2034-2041 ◽  
Author(s):  
M Kramer ◽  
R Amer ◽  
M Mukamel ◽  
M Snir ◽  
T Jaouni ◽  
...  

2014 ◽  
Vol 2014 ◽  
pp. 1-8 ◽  
Author(s):  
Marina Mesquida ◽  
Blanca Molins ◽  
Victor Llorenç ◽  
Maite Sainz de la Maza ◽  
María Victoria Hernandez ◽  
...  

The aim of the present study was to determine the serum cytokine profile and levels of high sensitivity C-reactive protein (hsCRP) in patients with uveitis associated with Behçet’s disease (BD) and to compare them with those obtained from healthy control subjects. We determined the serum concentration of interferon-γ(IFN-γ), interleukin-1β(IL-1β), IL-12p70, IL-17A, tumor necrosis factor-α(TNF-α), and hsCRP in 13 patients with active uveitis associated to BD, 24 inactive BD patients, and 20 controls. In a subgroup of 10 active patients, a second serum sample was obtained when the disease was inactive. Cytokine profiles and hsCRP levels were correlated with disease activity, severity, complications, and visual outcome. Levels of IFN-γand TNF-αwere significantly increased in patients with active uveitis associated to BD compared to controls (P<0.05). IFN-γ, TNF-α, and hsCRP were significantly higher during active uveitis associated to BD compared to inactive disease (P<0.05). Furthermore, IL-17A was significantly increased in patients with active BD without pharmacological treatment compared to controls (P<0.05). No significant correlations were found with specific cytokine profiles and disease severity, visual outcome, or complications. In summary, increased serum levels of IFN-γ, TNF-α, IL-17A, and hsCRP were associated with active uveitis associated with BD and might serve as markers of disease activity.


2013 ◽  
Vol 74 (10) ◽  
pp. 1363-1367 ◽  
Author(s):  
Eun Ha Kang ◽  
Jun Won Park ◽  
Chaerin Park ◽  
Hyeong Gon Yu ◽  
Eun Bong Lee ◽  
...  

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