Characteristics of optic neuropathy in Behçet disease

Abstract

ObjectiveWe present the clinical profile, features, and neuroimaging findings of 25 patients with Behçet disease (BD), and optic neuropathy (ON), which has been rarely reported in BD.MethodsData from 5 university hospitals were retrospectively reviewed, and patients with BD and ON were evaluated. There were 2 groups: (1) those already diagnosed with BD when ON developed (BD→ON group) and (2) those diagnosed with BD during the evaluation of ON (ON→BD group).ResultsThere were 25 BD patients with ON (13 males). Among these, 13 had ON→BD, and 12 had BD→ON. Seventeen patients had unilateral ON, and 7 patients had recurrent ON. BD→ON patients were older. Disc edema was seen more in ON→BD than in BD→ON patients (10 vs 3). Fourteen patients also had uveitis, 7 with BD→ON and 7 with ON→BD. There was other neurologic involvement in 8 patients; in the BD→ON group, 4/4 had MS-like disease, in the ON→BD group, 3 had typical parenchymal BD, and 1 had MS-like disease. Twenty of 21 patients received immunosuppressive medications, corticosteroids, or both. Prognosis was favorable in most: vision improved in 20 patients, more often in those receiving combined therapies.ConclusionBD may be diagnosed earlier if it is considered and investigated during the assessment of ON, particularly in high-risk regions. Prognosis of ON related to BD seems to be favorable. Immunosuppressants should be given along with corticosteroids. MS-like presentations should also be kept in mind in patients with BD and ON.

Removed: Atypical Optic Neuritis

“…Therefore, physician should look for characteristic burdens and changes in the disease (mucus membranes and skin efflorescence, joint pain, and other changes) and immunological analyzes that can lead to a diagnosis. Immunosuppressants should be given along with corticosteroids [63].…”

Neurological involvement by Behçet’s syndrome: clinical features, diagnosis, treatment and outcome

“…About 45% develop a subacute optic neuritis in which pain is uncommon (20% of cases) with disc swelling. [76][77][78] In one series, relapse occurred in 20%, and two-thirds returned to normal acuity following treatment; the nadir acuity was the determinant of visual outcome following treatment. 77 Another one-third had other neurological features of the condition.…”

“…77 Another one-third had other neurological features of the condition. 78 Two papers using 3T MRI have shown that there is enhancement of the optic nerve sheath rather than the nerve itself, implying that the disorder is a perineuritis. 79 80…”

Behcet’s Disease

“…Optic neuropathy (ON), although considered a rare manifestation of Behcet´s disease, might actually be overshadowed by uveitis' complications. It can appear during the course of already known BD (and should be considered as part of the neuro-BD disease spectrum), or it can even be the first manifestation of the disease (BD should then be kept in mind as a differential diagnosis of optic neuropathy Behcet's Disease DOI: http://dx.doi.org /10.5772/intechopen.85265 in regions where its prevalence is high) [17]. The prognosis of BD-associated ON seems not to be as poor as the one of BD uveitis, with excellent response to the combination of corticosteroids and immunosuppressants and recovery as the rule [18,19].…”