Borhani-Haghighi Afshin, Kardeh Bahareh, Banerjee Shubhasree, Yadollahikhales Golnaz, Safari Anahid, Sahraian Mohammad Ali, Shapiro Lee
Clinical Neurology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran.
Clinical Neurology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran.
Mult Scler Relat Disord. 2020 Apr;39:101906. doi: 10.1016/j.msard.2019.101906. Epub 2019 Dec 23.
Neuro-Behcet's disease (NBD) is defined as a combination of neurologic symptoms and/or signs in a patient with Behcet's disease (BD). Relevant syndromes include brainstem syndrome, multiple-sclerosis like presentations, movement disorders, meningoencephalitic syndrome, myelopathic syndrome, cerebral venous sinus thrombosis (CVST), and intracranial hypertension. Central nervous involvement falls into parenchymal and non-parenchymal subtypes. The parenchymal type is more prevalent and presents as brainstem, hemispheric, spinal, and meningoencephalitic manifestations. Non-parenchymal type includes CVST and arterial involvement. Perivascular infiltration of polymorphonuclear and mononuclear cells is seen in most histo-pathologic reports. In parenchymal NBD, cerebrospinal fluid (CSF) generally exhibits pleocytosis, increased protein and normal glucose. In NBD and CVST, CSF pressure is increased but content is usually normal. The typical acute NBD lesions in brain magnetic resonance imaging (MRI) are mesodiencephalic lesions. The pattern of extension from thalamus to midbrain provides a cascade sign. Brain MRI in chronic NBD usually shows brain or brainstem atrophy and/or black holes. The spinal MRI in the acute or subacute myelopathies reveals noncontiguous multifocal lesions mostly in cervical and thoracic lesions. In chronic patients, cord atrophy can also be seen. Brain MRI (particularly susceptibility-weighted images), MR venography (MRV) and computerized tomographic venography (CTV) can be used to diagnose CVST. Parenchymal NBD attacks can be treated with glucocorticoids alone or in combination with azathioprine. For patients with relapsing-remitting or progressive courses, shifting to more potent immunosuppressive drugs such as mycophenolate, methotrexate, cyclophosphamide, or targeted therapy is warranted. For NBD and CVST, immunosuppressive drugs with or without anticoagulation are suggested.
神经白塞病（NBD）被定义为白塞病（BD）患者出现神经症状和/或体征。相关综合征包括脑干综合征、多发性硬化样表现、运动障碍、脑膜脑炎综合征、脊髓病综合征、脑静脉窦血栓形成（CVST）和颅内高压。中枢神经系统受累分为实质型和非实质型。实质型更为常见，表现为脑干、半球、脊髓和脑膜脑炎表现。非实质型包括CVST和动脉受累。大多数组织病理学报告显示多形核细胞和单核细胞的血管周围浸润。在实质型NBD中，脑脊液（CSF）通常表现为细胞增多、蛋白质增加和葡萄糖正常。在NBD和CVST中，CSF压力升高但内容物通常正常。脑磁共振成像（MRI）中典型的急性NBD病变是中脑间脑病变。从丘脑延伸至中脑的模式呈现出一种级联征。慢性NBD的脑MRI通常显示脑或脑干萎缩和/或黑洞。急性或亚急性脊髓病的脊髓MRI显示大多在颈段和胸段病变的不连续多灶性病变。在慢性患者中，也可见脊髓萎缩。脑MRI（尤其是磁敏感加权成像）、磁共振静脉血管造影（MRV）和计算机断层静脉血管造影（CTV）可用于诊断CVST。实质型NBD发作可单独使用糖皮质激素或与硫唑嘌呤联合治疗。对于复发缓解型或进行性病程的患者，有必要换用更强效的免疫抑制药物，如霉酚酸酯、甲氨蝶呤、环磷酰胺或靶向治疗。对于NBD和CVST，建议使用免疫抑制药物，可联合或不联合抗凝治疗。
