INTRODUCTION Behçet's disease (BD) is an inflammatory disease of unknown etiology with periods of relapses and remissions. Neuro-Behçet syndrome (NBS) is one of the main causes of long-term morbidity and mortality, making its prompt recognition and early treatment fundamental to achieving a better outcome. Currently there are no treatment guidelines either for BS or NB, making the management of these patients particularly difficult. CASE PRESENTATION We present the case report of a patient with pseudo-tumoral lesion and myelitis refractory to steroids and cyclophosphamide who successfully showed remission after treatment with an anti-CD20 therapy. CONCLUSION This is the first report of concomitant pseudo-tumoral lesion and myelitis secondary to BS. We found rituximab treatment to be a safe and effective therapeutic option for NB supported by the radiological and clinical improvement achieved in our patient.

中文翻译：

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利妥昔单抗是难治性神经白塞综合征的有效治疗选择

引言 白塞氏病 (BD) 是一种病因不明的炎症性疾病，具有复发和缓解期。Neuro-Behçet 综合征 (NBS) 是导致长期发病率和死亡率的主要原因之一，因此迅速识别和早期治疗是获得更好结果的基础。目前没有针对 BS 或 NB 的治疗指南，这使得这些患者的管理特别困难。病例报告 我们介绍了一名对类固醇和环磷酰胺无效的假性肿瘤病变和脊髓炎患者的病例报告，该患者在接受抗 CD20 治疗后成功缓解。结论 这是继发于 BS 的伴随假性肿瘤病变和脊髓炎的第一份报告。