Uygunoğlu Uğur, Pasha Maarya, Saip Sabahattin, Siva Aksel
J Spinal Cord Med. 2015 Jan;38(1):111-4. doi: 10.1179/2045772314Y.0000000209. Epub 2014 Mar 28.
Spinal cord involvement is not common, but can be seen in neuro-Behçet's syndrome (NBS). The major site of involvement is the cervical spinal cord with the myelitis-like inflammatory lesions continuing more than two segments, and extending to the brainstem.
A 30-year-old male patient who has been followed with a diagnosis of Behçet's syndrome admitted to our neurology department clinically and radiologically suggestive of recurrent and extensive longitudinal myelitis. His anti-aquaporine antibody was negative. Because of insufficient effect of azathioprine, cyclophosphamide, and corticosteroids, infliximab was started. His clinical and radiological status is stationary for 3 years under infliximab treatment.
Myelitis such as that occurring in our patient may have a similar presentation like neuromyelitis optica (NMO), which should therefore be included in differential diagnosis. Myelitis observed in both NMO and NBS shows spinal cord lesions longer than three or more vertebrae. Anti-aquaporine antibody must be evaluated in all patients presenting with longitudinal myelitis. Anti-tumor necrosis factor agent infliximab might be an alternative treatment in severe form of NBS such as myelitis.
In our case, successful treatment of recurrent and extensive longitudinal transverse myelitis in NBS with infliximab was demonstrated.
脊髓受累并不常见,但可见于神经白塞病(NBS)。主要受累部位是颈髓,脊髓炎样炎性病变持续超过两个节段,并延伸至脑干。
一名30岁男性患者,诊断为白塞病,临床及影像学表现提示复发性广泛性纵向脊髓炎,入住我院神经内科。其抗水通道蛋白抗体阴性。由于硫唑嘌呤、环磷酰胺及皮质类固醇疗效欠佳,开始使用英夫利昔单抗治疗。在英夫利昔单抗治疗下,其临床及影像学状态稳定3年。
我们患者所发生的脊髓炎可能有类似视神经脊髓炎(NMO)的表现,因此应列入鉴别诊断。NMO和NBS中观察到的脊髓炎均显示脊髓病变长度超过三个或更多椎体。所有表现为纵向脊髓炎的患者均必须评估抗水通道蛋白抗体。抗肿瘤坏死因子药物英夫利昔单抗可能是NBS严重形式如脊髓炎的一种替代治疗方法。
在我们的病例中,证实英夫利昔单抗成功治疗了NBS中的复发性广泛性纵向横贯性脊髓炎。