神经白塞病六例临床特点分析并文献复习

Characteristics of Neuro-Behcet disease： six cases case report and literature review

对2000-2014年我院收治的6例神经白塞病（NBD）患者及同期文献报道的344例NBD患者共350例患者的临床资料进行回顾性分析显示NBD患者平均发病年龄为36.7岁,从出现白塞病（BD）症状到发生NBD,平均间隔时间为5.6年.男性多发,男∶女为1.94∶1.NBD全身表现最常见的症状为口腔溃疡（94.9％,277/292）,其次为外阴溃疡（69.1％,215/311）、发热（42.1％,131/311）.神经系统常见症状为肢体无力（36.9％,129/350）、头痛（34.0％,119/350）、感觉障碍（21.1％,74/350）、球麻痹症状（11.4％,40/350）,常见体征为锥体束征（27.1％,95/350）.NBD以中枢神经系统（CNS）受累最常见,其中CNS实质型受累占88.0％（308/350）,非实质型受累占5.7％（20/350）,周围神经系统（PNS）受累少见（6.9％,24/350）.糖皮质激素是治疗NBD的首选药物,疗效不佳者可同时合用免疫抑制剂或抗肿瘤坏死因子单克隆抗体.NBD多为单相病程,但32.0％（49/153）的患者存在复发、缓解的情况,7.7％（27/350）的患者预后不良.分析结果提示NBD是一种以CNS受累为主,以肢体无力、头痛、感觉障碍为主要症状的疾病,具有复发、缓解的特点,病情进展、残余症状重者预后不佳,临床诊治过程中需多加关注.

Six patients with Neuro-Behcet disease （NBD） were admitted in Beijing Hospital from 2000 to 2014,during the same period 344 NBD cases were reported in the literature.The clinical data of 350 Chinese NBD patients,including the symptoms,signs,laboratory tests,imaging,treatment and prognosis were retrospectively analyzed.The average onset age was 36.7 years old.The average interval time from Behcet disease （BD） to NBD was 5.6 years.NBD often occurred in male,the male/female ratio was 1.94：1.The most common manifestations were oral ulcer 94.9％ （277/292）,followed by genital ulcer 69.1％ （215/311） and fever 42.1％ （131/311）.The most common symptoms in neurology were limb weakness （36.9％,129/350）,headache （34.0％,119/350） and sensory disturbance （21.1％,74/350）,followed by bulbar symptoms （11.4％,40/350）,pyramidal sign was most often （27.1％,95/350）.The central neurology system （CNS） was the major target of NBD,parenchymal CNS was affected most （88.0％,308/350）,non-parenchymal CNS was affected less （5.7％,20/350）,peripheral neurology system （PNS） was rarely affected （6.9％,24/350）.Steroids were the first-line drug for NBD,if it failed,immunosuppressive agents or biologic agents could be used.The most NBD patients had single episodes,32.0％ （49/153） had relapses with remission,7.7％ （27/350） had adverse prognosis.Analysis showed that NBD should be paid more attention.