本文選題：白塞病　切入點：神經(jīng)系統(tǒng)　出處：《北京協(xié)和醫(yī)學院》2017年碩士論文　論文類型：學位論文

【摘要】：目的:研究白塞病(Behcet's disease,BD)伴發(fā)神經(jīng)系統(tǒng)受累的臨床特點。方法:回顧性分析2000年1月至2016年6月間北京協(xié)和醫(yī)院收住院的BD神經(jīng)系統(tǒng)受累患者的臨床表現(xiàn)、腦脊液及影像學檢查、治療及預后。數(shù)據(jù)采用SPSS 21.0軟件處理,正態(tài)分布用均值±標準差表示,非正態(tài)分布用中位數(shù)及范圍表示,兩組間比較采用獨立樣本t檢驗或Mann-Whitney U檢驗;相關性分析采用Pearson或Speraman相關系數(shù)檢驗。p0.05認為差異具有統(tǒng)計學意義。結果:BD伴發(fā)神經(jīng)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 起病年齡(29.0±10.8)歲,神經(jīng)受累年齡(33.9±11.7)歲,BD起病到神經(jīng)癥狀出現(xiàn)的平均時間4.85年,出現(xiàn)神經(jīng)系統(tǒng)癥狀到NBD確診的中位時間為3(0-50)月,其中10例(16.1%)以神經(jīng)受累為首發(fā)表現(xiàn)。59例患者伴發(fā)中樞神經(jīng)系統(tǒng)受累,42例(67.7%)為實質受累(pNBD),主要累及腦干、大腦半球,脊髓、小腦亦可受累,13例存在多發(fā)病灶。17例伴發(fā)非實質受累,均為顱內靜脈竇血栓,以上矢狀竇、橫竇最多見(分別58.8%、52.9%)。3例伴發(fā)周圍神經(jīng)病變。pNBD患者以錐體束征、頭痛、精神行為異常為主要表現(xiàn),非實質受累BD患者最常見表現(xiàn)為頭痛、視力下降、視乳頭水腫。57例行腰穿檢查,pNBD患者中80%(32/40)腦脊液壓力正常,55%(22/40)蛋白輕度升高(0.51±0.24)g/L,細胞學檢查可見淋巴細胞性炎癥(9/20)或中性粒細胞性炎癥(2/20)。非實質受累患者93.3%(14/15)存在高顱壓,3例腦脊液蛋白輕度升高,細胞學均未見異常。76.2%(32/42)pNBD患者頭MRI病變位于中線結構,如腦干、大腦半球側腦室旁、半卵圓中心,以T2高信號為主。所有pNBD患者均接受≥1 mg/Kg/d糖皮質激素治療,54.8%(23/42)行激素沖擊;CTX為最常用的免疫抑制劑,10例聯(lián)合多種免疫制劑(環(huán)孢素/硫唑嘌呤);生物制劑治療6例(英夫利西單抗4例,托珠單抗1例,重組人干擾素α2al例),鞘內注射28例。非實質受累患者予激素、免疫抑制劑、抗凝、脫水降顱壓治療。隨訪中位時間36(0.3-156)月,pNBD中23例好轉,8例病情反復,3例死亡,非實質受累患者2例病情反復,治療后改善,所有非實質受累患者預后良好,無死亡病例。結論:BD伴發(fā)神經(jīng)系統(tǒng)受累多見于男性,其中實質受累多見,且以腦干常見,病死率較高,非實質受累以顱內靜脈血栓多見,多數(shù)預后良好,均需早期識別并積極治療。
[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 鹵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 鹵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 鹵10.8 years old, and the age of onset of BD was 29.0 鹵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 鹵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 鹵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，

本文編號：1611660