AB0359 CRANIAL INVOLVEMENT IN BEHÇET’S DISEASE
Authors: Ö. Özdemir Işik, S. Şan, A. Yazici, A. Cefle
Journal: Annals of the Rheumatic Diseases (2021)

Background: Behçet’s disease (BD) is a vasculitic multisystem inflammatory disorder. It may also involve the skin, mucosa, eyes, blood vessels, joints, gastrointestinal system, and central nervous system (CNS). Objectives: In this study, we aimed to present CNS involvement data in patients followed up with a diagnosis of BD. Methods: The clinical, demographic, laboratory and medication data of 394 patients who were followed up with a diagnosis of BD in our rheumatology outpatient clinic between 2000 and 2020 were retrospectively evaluated. Results: CNS involvement was detected in %5.6(24) patients who were followed up with a diagnosis of BD. 66.7% of the patients were male, and their mean diagnosis age was 25.1±8.2 years, mean CNS involvement age was 28.39±9.6 years. Neurological symptoms occurred in an average of 3.3 years after the diagnosis of BD. Parenchymal involvement was present in 54.2% of the patients with CNS involvement, 45.8 % had cerebral venous thrombosis. Oral apthous ulcer was found in 91.7 % of the patients, genital ulcer in 70.8%, pathergy positivity in %45.8 papulopustular lesion in %37.5, erythema nodosum in %45.8. Patients with CNS involvement had headache, vision loss, diplopia, hemiparesis, epilepsy, proptosis, and ataxic gait and walking difficulty in 66.6%, 12.5%, 25%, 12.5%, 8.3%, 8.3% and 12.5%, respectively. Patients with and without CNS involvement were compared in terms of clinical findings and medications. A significant difference was observed in terms of cardiac involvement (Table 1). While one of the 2 patients with cardiac involvement had a thrombus in the right ventricle, the other had pericardial effusion. The patient with cardiac thrombus also had cerebral venous thrombus. Steroid, azathioprine, cyclophosphamide treatments have been used at a higher in patients with CNS involvement. In addition, the patients with parenchymal and cerebral venous involvement were compared in terms of clinical, demographics findings and medications. The presence of papulopustular lesions (9.1% vs 57.1%) and pathergy positivity (18.2% vs 71.4%) were higher in patients with parenchymal involvement (p:0.033, p: 0.020, respectively). Table 1. Comparison of data of patients with and without CNS involvement n (%) CNS Involvement (+) n:24 CNS Involvement (-) n:370 P Gender 0.396 Female 8(33.3) 164(44.3) Male 16(66.7) 206(55.7) Oral Aphthous Ulcer 22(91.7) 357(96.5) 0.230 Genital Ulcer 17(70.8) 239(64.6) 0.661 Papulopustular lesion 9(37.5) 120(32.4) 0.655 Erythema Nodosum 11(45.8) 139(37.6) 0.516 Pathergy 11(45.8) 174(47) 0.988 Uveitis 9(37.5) 129(34.9) 0.827 Retinal Vasculitis 1(4.2) 10(2.7) 0.504 Arthritis 3(12.5) 95(25.7) 0.221 Deep Venous Involvement 6(25) 59(15.9) 0.256 Artery Occlusion 1(4.2) 8(2.2) 0.436 Arterial Aneurysm 1(4.2) 14(3.8) 1 GIS involvement 2(8.3) 15(4.1) 0.277 Cardiac involvement 2(8.3) 4(1.1) 0.046 Conclusion: It has been reported that neurological symptoms occur in an average of 2.5 to 6.5 years after the diagnosis of BD. On the other hand, it has been stated that neurological complications may be the first involvement in 3-33% of the cases and this situation will cause diagnostic difficulties 1 . These data are consistent with our study. References: [1]Noel N, Drier A, Wechsler B, ve ark. Neurological manifestations of Behcet’s disease. Rev Med Interne 2014;35(2):112–120. Disclosure of Interests: None declared