神经白塞病临床特点分析并文献复习(附12例报告)

Analysis of clinical characteristics of neuro-Behcet's disease and literature review:a report of 12 cases

目的分析神经白塞病(neuro-Behcet's disease,NBD)的临床特点及治疗。方法回顾分析航天中心医院2006年1月至2018年1月12例NBD患者的临床表现、实验室检查、影像学资料、治疗及预后。结果 12例患者,男7例,女5例;诊断NBD时的年龄16~55岁,平均(30±12)岁;均有白塞病(Behcet's disease,BD)的基本临床表现;口腔溃疡11例,生殖器溃疡7例,眼部病变5例,针刺反应阳性5例,结节红斑5例,发热4例。83%(10例)患者神经系统症状出现在BD其他系统首发表现之后平均(52±31)个月。神经系统症状以肢体无力(7例,58%)、头痛(6例,50%)、肢体活动障碍(5例,42%)、感觉障碍(4例,33%)、视力下降(3例,25%)等最为常见。9例患者行腰椎穿刺检查,3例存在脑脊液压力升高(33%)。MRI可见长T1或等T1、长T2的异常信号,Flair像呈高信号。病灶可累及半球(50%)、脑干(25%)、小脑(8.3%)、脊髓(8.3%)、颅内静脉窦(8.3%))等多个部位。糖皮质激素联合免疫抑制剂治疗有效,好转9例(75%),病情反复3例(25%)。结论 NBD临床表现多样,以累及中枢神经系统为主,肢体无力、头痛、感觉障碍为主要症状,需早期识别、及时诊断,经积极治疗后,大多预后良好。

Objective To investigate the clinical characteristics of neuro-Behcet＇s disease（NBD）. Methods The clinical data of 12 NBD patients, in Aerospace Central Hospital from January 2006 to January 2018, including signs and symptoms, laboratory tests, imageological examination, treatment and prognosis, were retrospectively analyzed. Results Twelve NBD patients included 7 males and 5 females, and their age ranged from 16 to 55 years old, with an average of（30±12） years old. The most common manifestations were oral ulcer（92%, 11/12）, followed by genital ulcer（58%, 7/12）,ocular involvement（42%, 5/12）, positive acupuncture reaction（42%,5/12）, erythema nodule（42%,5/12） and fever（33%,4/12）. 83% patients（10/12） suffered neurological symptoms after other initial systemic symptoms of BD in mean of（52±31） months. Most frequent neurological signs were limb weakness（7 patients, 58%）, headache（6 patients, 50%）, dyskinesia（5 patients, 42%）, sensory disturbance（4 patients, 33%）, decline of vision（3 patients, 25%）. Three out of 9 patients（33%） were found elevated cerebrospinal fluid（CSF） pressure by lumbar puncture. MRI showed long T1 or equal T1 and long T2 abnormal signal, Flair high signal lesions. Lesions might involve the cerebral hemisphere（50%）, brainstem（25%）, cerebellum（8.3%）, spinal cord（8.3%）, intracranial venous sinus（8.3%） and so on. After glucocorticoid combined with immunosuppressant therapy, 9 patients（75%） achieved clinical improvements, 3 patients（25%） relapsed. Conclusion NBD may be present with various clinical manifestations. Limb weakness, headache and sensory disorders are the main symptoms. By early identification, timely diagnosis and active treatment, the prognosis of most patients is good.