Fujiwara Shunya, Manabe Yasuhiro, Nakano Yumiko, Omote Yoshio, Yunoki Taijun, Kono Syoichiro, Narai Hisashi, Abe Koji
Department of Neurology, National Hospital Organization Okayama Medical Center, Okayama, Japan.
Department of Neurology, Graduate School of Medicine, Dentistry and Pharmaceutical Sciences, Okayama University, Okayama, Japan.
Case Rep Neurol. 2021 Feb 8;13(1):78-83. doi: 10.1159/000512323. eCollection 2021 Jan-Apr.
We report 2 cases of probable neuro-Behçet's disease (NBD) with longitudinally extensive transverse myelitis (LETM). In both cases, the patients presented paraplegia, as well as sensory, bladder, and rectal disturbances. Magnetic resonance imaging (MRI) of patient 1 showed continuous high signal intensity extending from the midbrain to the entire spinal cord in the central part of the cord on T2-weighted imaging (T2WI). Spinal MRI of patient 2 revealed high signal intensity extending from Th2 to Th10 in the central part of the cord on T2WI. Both patients received high-dose methylprednisolone. A continuous lesion from the midbrain to the entire spinal cord as in patient 1 has not been previously reported. Patient 2 dramatically improved by infliximab therapy. The present cases suggest that NBD should be considered as a differential diagnosis in patients with LETM.
我们报告了2例可能患有神经白塞病(NBD)并伴有纵向广泛横贯性脊髓炎(LETM)的病例。在这两个病例中,患者均出现截瘫,以及感觉、膀胱和直肠功能障碍。患者1的磁共振成像(MRI)在T2加权成像(T2WI)上显示,脊髓中央部分从脑干到整个脊髓呈连续高信号强度。患者2的脊髓MRI在T2WI上显示脊髓中央部分从胸2到胸10呈高信号强度。两名患者均接受了大剂量甲基强的松龙治疗。像患者1那样从脑干到整个脊髓的连续性病变此前尚未见报道。患者2经英夫利昔单抗治疗后显著改善。目前的病例表明,对于LETM患者应考虑将NBD作为鉴别诊断。