CAJ | 학술논문

目的研究白塞病合并颅内静脉窦血栓(CVST)的临床特点.方法回顾性分析北京协和医院2004年1月至2014年3月白塞病合并CVST患者的临床表现、血栓部位、治疗及预后.用SPSS19.0软件行统计学分析,正态分布资料用x±s表示;非正态分布用中位数及范围表示.结果白塞病合并CVST患者12例,占同期白塞病住院患者(601例)的2.0％.其中男性6例,女性6例,诊断CVST时的年龄15～52岁,平均(28±10)岁.92％(11例)患者神经系统症状出现在白塞病其他系统首发表现之后平均(56±35)个月.神经系统症状多为亚急性或慢性起病(9例,82％),以头痛(11例,92％)、视乳头水肿(9例,75％)、恶心呕吐(8例,67％)和视力下降(6例,50％)最为常见.10例患者行腰椎穿刺检查,8例存在脑脊液压力升高(80％).CVST最常出现的部位依次为横窦(9例,75％)、上矢状窦(8例,67％)和乙状窦(7例,58％),并且同时合并2个及以上静脉窦血栓形成的占多数(8例,67％).除CVST外,8例患者合并有其他部位血栓,7例为下肢静脉血栓,1例为肺动脉血栓形成.此外,同时合并的神经系统其他病变还有脑梗死3例,蛛网膜下腔出血1例,右侧颞叶出血1例.经过糖皮质激素、免疫抑制剂、抗凝、脱水降颅压等治疗,平均中位随访时间8个月(0.7～88.8个月),好转10例(83％),病情反复2例(17％).结论 CVST是白塞病的相对少见并发症,临床常表现为头痛、视乳头水肿、恶心呕吐和脑脊液压力升高,需早期识别、及时诊断,积极治疗后,大多预后良好.
목적 연구백새병합병로내정맥두혈전(CVST)적림상특점.방법 회고성분석북경협화의원2004년1월지2014년3월백새병합병CVST환자적림상표현、혈전부위、치료급예후.용SPSS19.0연건행통계학분석,정태분포자료용x±s표시;비정태분포용중위수급범위표시.결과 백새병합병CVST환자12례,점동기백새병주원환자(601례)적2.0％.기중남성6례,녀성6례,진단CVST시적년령15～52세,평균(28±10)세.92％(11례)환자신경계통증상출현재백새병기타계통수발표현지후평균(56±35)개월.신경계통증상다위아급성혹만성기병(9례,82％),이두통(11례,92％)、시유두수종(9례,75％)、악심구토(8례,67％)화시력하강(6례,50％)최위상견.10례환자행요추천자검사,8례존재뇌척액압력승고(80％).CVST최상출현적부위의차위횡두(9례,75％)、상시상두(8례,67％)화을상두(7례,58％),병차동시합병2개급이상정맥두혈전형성적점다수(8례,67％).제CVST외,8례환자합병유기타부위혈전,7례위하지정맥혈전,1례위폐동맥혈전형성.차외,동시합병적신경계통기타병변환유뇌경사3례,주망막하강출혈1례,우측섭협출혈1례.경과당피질격소、면역억제제、항응、탈수강로압등치료,평균중위수방시간8개월(0.7～88.8개월),호전10례(83％),병정반복2례(17％).결론 CVST시백새병적상대소견병발증,림상상표현위두통、시유두수종、악심구토화뇌척액압력승고,수조기식별、급시진단,적겁치료후,대다예후량호.
Objective To investigate the clinical characteristics of Beh(c)et's disease (BD) complicated with cerebral venous sinus thrombosis (CVST).Methods In patients data at Peking Union Medical College Hospital between February 2004 and April 2013 were reviewed retrospectively to identify patients who were diagnosed as BD complicated with CVST.SPSS 19.0 software was used for statisticals analysis.Data of normal distribution was expressed by x±s (standard deviation) and that of abnormal distribution by median and range.Results Of 601 inpatients with BD during that period,12 patients (2％,6 male and 6 female) developed CVST,and their age was (28±10) years old (range 15 to 52 years old).Most of patients (92％,11/12)suffered neurological symptoms after other initial systemic symptoms of BD in mean (56±35) months.The most frequent neurological signs were headache (11 patients,92％),papilledema (9 patients,75％),nausea/vomiting (8 patients,67％) and blurred vision (6 patients,50％),with progression as the main mode of onset (9/11,82％).Eight out of 10 patients (80％) were found elevated cerebrospinal fluid (CSF) pressure by lumbar puncture.Occlusions of multiple sinuses (8 patients,67％) were more frequent than single occlusion (4 patients,33％).The most frequent locations of CVST were the transverse sinus (9 patients,75％),superior sagital sinus (8 patients,67％) and sigmoid sinus (7 patients,58％).Other than CVST,8 patients were complicated with other sites of thrombosis (7 cases in lower extremity veins and 1 in pulmonary artery).In addition,cerebral infarction was found in 3 patients,subarachnoid hemorrhage and right temporal lobe hemorrhage in 1 patient,respectively.After glucocorticoid,immunosuppressant,as well as anticoagulant and dehydration therapy,with a median follow-up of 8 months (0.7 to 88.8 months),10 patients achieved clinical improvements (83％),2 patients relapsed (17％).Conclusion CVST is a relatively rare complication of Beh(c)et's disease,with headache,papilledema,nausea/vomiting and blurred vision as the common clinical manifestations.By early identification,timely diagnosis and active treatment,the prognosis of most patients is good.