Tang Qi, Tian Jing
Department of Rheumatology, Second Xiangya Hospital of Central South University, Changsha, Hunan, China.
Arch Rheumatol. 2015 Nov 3;31(1):91-93. doi: 10.5606/ArchRheumatol.2016.5441. eCollection 2016 Mar.
Behçet's disease is a chronic, relapsing inflammatory disorder of unknown etiology. Neuro-Behçet's disease (NBD) occurs in approximately 5 to 49% of patients with Behçet's disease. Spinal cord involvement is very rare in NBD. In this article, we report a 22-year-old male patient of NBD with longitudinal myelitis involving the entire spinal cord. Patient was admitted with one-week of headache, vomiting, and urinary incontinence. Before admission, he felt motor weakness for two years and had noticed recurrent genital and oral ulcers with a frequency of more than three episodes per year for five years. T2-weighted spinal magnetic resonance images showed hyperintensities within the entire spinal cord. He was diagnosed as NBD with longitudinal myelitis. Intravenous methylprednisolone (120 mg/day) was administered for three days, followed by an oral administration of prednisolone (45 mg/day). In conjunction with the steroid therapy, intravenous cyclophosphamide (0.4 g/week) was administered twice. Rapid improvements were detected after receiving treatments for half a month. NBD associated longitudinal myelitis is really rare. This case provided important implications for the diagnosis and treatment of longitudinal myelitis in NBD patients.
白塞病是一种病因不明的慢性复发性炎症性疾病。神经白塞病(NBD)发生于约5%至49%的白塞病患者中。脊髓受累在NBD中非常罕见。在本文中,我们报告了一名22岁的NBD男性患者,患有累及整个脊髓的纵向脊髓炎。患者因头痛、呕吐和尿失禁一周入院。入院前,他感到运动无力两年,并且注意到复发性生殖器和口腔溃疡,每年发作频率超过三次,持续了五年。T2加权脊髓磁共振成像显示整个脊髓内有高信号。他被诊断为患有纵向脊髓炎的NBD。静脉注射甲泼尼龙(120毫克/天)三天,随后口服泼尼松龙(45毫克/天)。结合类固醇治疗,静脉注射环磷酰胺(0.4克/周)两次。接受治疗半个月后发现有快速改善。NBD相关的纵向脊髓炎确实罕见。该病例为NBD患者纵向脊髓炎的诊断和治疗提供了重要启示。