神经白塞病的临床及影像学特点

Clinical and imaging features of Neuro-Behcet's disease

目的探讨神经白塞病(NBD)的临床及影像学特点。方法对本院1例NBD患者的临床资料及国内文献报道的250例NBD患者的资料进行回顾性分析。结果 251例NBD患者男∶女=1.89∶1,平均发病年龄37.7岁,NBD出现在白塞病发病后的2个月~39年(平均7.8年),最常见的全身表现为复发性口腔溃疡(95.2%),其次为生殖器溃疡(73.3%),皮肤损害(50.2%),眼部损害(44.2%)。肢体无力(44.2%)为最常见的神经系统症状,其次为头痛(36.3%),感觉障碍(25.5%),球麻痹(15.9%),仅5.6%以神经系统症状首发。所有行血沉、C-反应蛋白、WBC、髓鞘碱性蛋白检测的患者结果均升高,行寡克隆区带检测的患者绝大多数为阳性。MRI可见长T_1或等T_1、长T_2的异常信号,Flair像呈高信号。病灶可累及半球(45.8%)、脑干(36.7%)、小脑(9.2%)、脊髓(7.2%)等多个部位。糖皮质激素或联合免疫抑制剂治疗有效,56.3%的患者症状完全或明显缓解。结论 NBD临床表现多样且缺乏特异性,容易误诊。加强对患者复发性口腔溃疡病史的询问,有助于早诊断、早治疗。

Objective To investigate the clinical and imaging features of Neuro-Behcet＇s disease （NBD）. Methods The clinical data of 1 NBD patient who treated in our hospital and other 250 NBD patients reported in domestic literatures were analyzed retrospectively. Results Among all the 251 NBD patients, male： female = 1.89： 1, neurological involvement occurred after the onset of Behcet＇s disease from 2 months to 39 years （ average 7.8 years） with average age of onset was 37.7 years. The most common manifestations of systemic were recurrent aphthous ulcer （95.2%）, followed by genital ulcer （73.3%）, skin lesion （50.2%）, eye damage （44.2%）. The most common form of NBD nervous system damage was headache （36.3%）, followed by sensory disturbance （25.5%）, bulbar paralysis （15. 9%）, neurological symptoms in the first episode just 5. 6%. Erythrocyte sedimentation rate, C-reactive protein, WBC, myelin basic protein were rised in all tested patients, and oligoclonal band were negative in most tested patients. MRI showed long T1 or equal T1 and long T2 abnormal signal, Flair high signal lesions. Lesions may involve the cerebral hemisphere （45.8%）, brainstem （36.7%）, cerebellum （9.2%）, and spinal cord （7.2%）, and so on. Glucocorticoids or combined with immunosuppresive was effective, and 56.3% patients were completely or obviously improved. Conclusions The clinical features of NBD are complex and no specificness, easy to be misdiagnosed. It is important to inquire about the history of recurrent oral ulcers that could help for early diagnosis and early treatment.