Cerebral venous sinus thrombosis in Beh(c)et's disease: clinical study and follow-up of 12 cases
10.3760/cma.j.issn.1007-7480.2015.01.004
- VernacularTitle:白塞病合并颅内静脉窦血栓12例临床分析
- Author:
Xinxiang HUANG
;
Guohua LI
;
Yan XU
;
Li WANG
;
Wenjie ZHENG
;
Yan ZHAO
- Publication Type:Journal Article
- Keywords:
Beh(c)et's disease;
Thrombosis
- From:
Chinese Journal of Rheumatology
2015;19(1):16-20
- CountryChina
- Language:Chinese
-
Abstract:
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.
