神经系统白塞综合征的研究进展

The research progress of the nervous system Behcet’s syndrome

白塞综合征(Behcet’s syndrome,BS)是一种少见的自身免疫病,神经白塞综合征(NBS)是兼有神经系统损害的白塞综合征,通常见于炎症性和血管性中枢神经系统疾病的鉴别诊断,NBS的临床和神经影像主要有两种变现形式:脑实质型NBS(p-NBS)和实质外脑静脉窦血栓形成(CVST)。最近研究发现,人类和小鼠神经纤维抗体显示NBS链球菌和结核分枝杆菌热休克蛋白(HSP)之间存在交叉反应,脊髓磁共振成像(MRI)有“百吉饼征”和“运动神经元”类型表现,英夫利昔单抗能有效预防复发及稳定残疾症状。原发性NBS通常累及脑干-间脑区部位,头痛、肌无力、共济失调和偏瘫是其主要临床特征。

Behcet’s syndrome(BS)a rare autoimmune disease,and nervous system Beh et’s syndrome(NBS)is a combination of neurological damage and Besser's syndrome,commonly included in the differential diagnosis of many inflammatory and vascular central nervous system(CNS)disorders.Clinical and neuroimaging findings support two major forms of NBS:parenchymal NBS(p-NBS)and an extra-parenchymal form that presents with cerebral venous sinus thrombosis(CVST).Recent research findings that the antibodies to human and mouse neurofibrils showing cross-reactivity with Streptococcus spp.and Mycobacterium tuberculosis heat shock proteins(HSP)in NBS.Two distinct magnetic resonance imaging(MRI)patterns of spinal cord involvement have been observed were“Bagel sign”and“motor neuron”patterns.Therapeutic studies have shown that infliximab effectively prevents further Relapses and stabilizes the symptoms of patients experiencing ongoing clinical relapses.Primary NBS commonly affects the brain stem-diencephalic region,and headache,dysarthria,ataxia,and hemiparesis are the main clinical features of NBS.