Department of Neurology, Istanbul University Cerrahpaşa School of Medicine, Istanbul, Turkey.
Curr Opin Rheumatol. 2019 Jan;31(1):32-39. doi: 10.1097/BOR.0000000000000562.
Neurological involvement in Behçet's syndrome is defined as 'the occurrence of neurological symptoms and signs in a patient who meets the International Diagnostic Criteria for BS not otherwise explained by any other known systemic or neurological disease or treatment, and in whom objective abnormalities consistent with neuro-Behçet's syndrome (NBS) are detected either on neurological examination, neuroimaging studies (magnetic resonance imaging [MRI]), and/or on cerebrospinal fluid (CSF) examination'. Given that the neurological involvement of Behçet's syndrome carries a poor prognosis, we aimed to describe the differential diagnosis of NBS and highlight the different radiological patterns together with the treatment options.
Two distinct MRI patterns of spinal cord involvement in Behçet's syndrome according to T2-weighted axial images were described: 'Bagel Sign' pattern: a central lesion with hypointense core and hyperintense rim with or without contrast enhancement; and 'Motor Neuron' pattern: a symmetric involvement of the anterior horn cells. Infliximab prevents patients from having further attacks and even led to improvement in the neurological examination.
As the treatment options completely differ, a NBS diagnosis should be carefully made in patients with clinical and MRI features mimicking other central nervous system inflammatory disorders.
Behcet 综合征的神经系统受累被定义为“在符合国际 BS 诊断标准的患者中出现神经系统症状和体征，这些症状和体征不能用任何其他已知的系统性或神经系统疾病或治疗来解释，并且在神经系统检查、神经影像学研究（磁共振成像[MRI]）和/或脑脊液（CSF）检查中发现与神经 Behcet 综合征（NBS）一致的客观异常”。鉴于 Behcet 综合征的神经系统受累预后不良，我们旨在描述 NBS 的鉴别诊断，并强调不同的影像学模式以及治疗选择。
根据 T2 加权轴位图像，描述了 Behcet 综合征脊髓受累的两种不同的 MRI 模式：“Bagel Sign”模式：中央病变，核心低信号，边缘高信号，有或无对比增强；和“运动神经元”模式：前角细胞对称受累。英夫利昔单抗可防止患者再次发作，甚至导致神经系统检查改善。
由于治疗选择完全不同，对于具有类似于其他中枢神经系统炎症性疾病的临床和 MRI 特征的患者，应仔细做出 NBS 诊断。
