白塞病伴发神经系统受累临床特点

发布时间：2018-03-14 14:41

  本文选题：白塞病　切入点：神经系统　出处：《北京协和医学院》2017年硕士论文　论文类型：学位论文

【摘要】：目的:研究白塞病(Behcet's disease,BD)伴发神经系统受累的临床特点。方法:回顾性分析2000年1月至2016年6月间北京协和医院收住院的BD神经系统受累患者的临床表现、脑脊液及影像学检查、治疗及预后。数据采用SPSS 21.0软件处理,正态分布用均值±标准差表示,非正态分布用中位数及范围表示,两组间比较采用独立样本t检验或Mann-Whitney U检验;相关性分析采用Pearson或Speraman相关系数检验。p0.05认为差异具有统计学意义。结果:BD伴发神经受累者62例(男37例,女25例),占同期BD住院患者的6.1%(62/1009)。BD 起病年龄(29.0±10.8)岁,神经受累年龄(33.9±11.7)岁,BD起病到神经症状出现的平均时间4.85年,出现神经系统症状到NBD确诊的中位时间为3(0-50)月,其中10例(16.1%)以神经受累为首发表现。59例患者伴发中枢神经系统受累,42例(67.7%)为实质受累(pNBD),主要累及脑干、大脑半球,脊髓、小脑亦可受累,13例存在多发病灶。17例伴发非实质受累,均为颅内静脉窦血栓,以上矢状窦、横窦最多见(分别58.8%、52.9%)。3例伴发周围神经病变。pNBD患者以锥体束征、头痛、精神行为异常为主要表现,非实质受累BD患者最常见表现为头痛、视力下降、视乳头水肿。57例行腰穿检查,pNBD患者中80%(32/40)脑脊液压力正常,55%(22/40)蛋白轻度升高(0.51±0.24)g/L,细胞学检查可见淋巴细胞性炎症(9/20)或中性粒细胞性炎症(2/20)。非实质受累患者93.3%(14/15)存在高颅压,3例脑脊液蛋白轻度升高,细胞学均未见异常。76.2%(32/42)pNBD患者头MRI病变位于中线结构,如脑干、大脑半球侧脑室旁、半卵圆中心,以T2高信号为主。所有pNBD患者均接受≥1 mg/Kg/d糖皮质激素治疗,54.8%(23/42)行激素冲击;CTX为最常用的免疫抑制剂,10例联合多种免疫制剂(环孢素/硫唑嘌呤);生物制剂治疗6例(英夫利西单抗4例,托珠单抗1例,重组人干扰素α2al例),鞘内注射28例。非实质受累患者予激素、免疫抑制剂、抗凝、脱水降颅压治疗。随访中位时间36(0.3-156)月,pNBD中23例好转,8例病情反复,3例死亡,非实质受累患者2例病情反复,治疗后改善,所有非实质受累患者预后良好,无死亡病例。结论:BD伴发神经系统受累多见于男性,其中实质受累多见,且以脑干常见,病死率较高,非实质受累以颅内静脉血栓多见,多数预后良好,均需早期识别并积极治疗。
[Abstract]:Objective: to study the clinical features of Behcetsdisease (BDD) associated with nervous system involvement. Methods: the clinical manifestations, cerebrospinal fluid (CSF) and imaging findings of BD patients admitted to Peking Union Hospital from January 2000 to June 2016 were retrospectively analyzed. The data were treated with SPSS 21.0 software, the normal distribution was expressed as mean 卤standard deviation, the non-normal distribution was expressed as median and range, and the comparison between the two groups was performed by independent sample t test or Mann-Whitney U test. Results there were 62 cases (37 males and 25 females) of BD with neuropathic involvement, accounting for 6.1g / 1009. BD onset age of 29.0 卤10.8years in BD patients in the same period, and the difference was statistically significant by using Pearson or Speraman correlation coefficient test (p0.05), the age of onset of BD was 29.0 卤10.8 years old, and the age of onset of BD was 29.0 卤10.8 years old. The average time from onset of BD to onset of neurological symptoms was 4.85 years, and the median time from neurological symptoms to NBD diagnosis was 3-0-50) months, the age of nerve involvement was 33.9 卤11.7years. Among them, 10 cases (16. 1) with nerve involvement as the first manifestation. 59 cases with central nervous system involvement (42 cases with central nervous system involvement) as the parenchymal involvement, mainly involving brain stem, cerebral hemisphere, spinal cord, cerebellar involvement, 13 cases with multiple lesions. 17 cases with non-parenchymal involvement. Most of them were intracranial venous sinus thrombosis, superior sagittal sinus and transverse sinus (58.9%, 52.9%, respectively) with pyramidal sign, headache and abnormal mental behavior. Loss of vision, Papillary Edema. 57 cases with PNBD: 80 / 32 / 40) normal cerebrospinal fluid pressure 55 and 22 / 40) protein slightly increased by 0.51 卤0.24g / L, cytological examination showed lymphocytic inflammation (9 / 20) or neutrophil inflammation (2 / 20). There was high intracranial pressure in patients with non-parenchymal involvement (93.314r-1 / 15). Cerebrospinal fluid protein increased slightly in 3 cases. No cytological abnormality was found in 32 / 42pNBD patients with head MRI lesions located in midline structures, such as brain stem, paraventricular hemispheres, semicircular center, brain stem, brain stem, cerebral hemispheric lateral ventricle, hemispNBD, and hemispNBD. All patients with pNBD were treated with glucocorticoid 鈮，

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