少见原因引起的颅内静脉窦血栓形成

Natural history of cerebral vein thrombosis: a systematic review,MortalityThe mortality rate during the acute phase was assessed in 12Studies,Of 1180 evaluated patients, 66 died within the first month (mean, 5.6%; range, 0%-15.2%).However, the mortality rate varied highly between the studiesand was 0 in 5 studies. Of 45 evaluated patients, 32 died as aconsequence of CVT (most of them for cerebral herniation),whereas most of 13 died as a consequence of an underlyingdisease. The overall mortality rate at the end of follow-up was9.4% (122 deaths in 1303 total patients). However, it was highly variable between the studies (range, 0%-39%). Noteworthy,most of the deaths that occurred during follow-up were relatedto an underlying condition such as cancer (24 of 38 evaluateddeaths during follow-up), and they were not a direct consequenceof cerebral vein thrombosis,Disability Outcome information was available for 8 studies at 3 to 6 months of follow-up and 11 studies at one or more years of follow-up . Eight hundred and fortythree patients provided data at 3 to 6 months of follow-up; 735 surviving patients (87.2%; range, 58.9%-96.4%) had a good outcome with complete or partial recovery, and 73 patients (8.7%;range, 3.6%-29.4%) had a poor outcome with permanent neurologic deficits. At 12 or more months, 12 studies evaluated 1100 patients. Among these, 972 (88.3%; range, 54.2%-97.1%) had a complete or partial recovery, and only 107 (9.7%; range, 0%-45.8%) had a poor outcome,Recanalization Five studies investigated the recanalization of cerebral venous thrombosis. Unfortunately, all these studies presenting data on recanalization have a relatively small sample size (ranging from 16 to 40 patients) and, in total, enrolled 154 patients. Four of these defined recanalization as complete (blood flow without any interruption), partial (small interruption of continuous blood flow and/or narrowing of the venous lumen), or absent (interrupted blood flow), and the other did not distinguish between partial and complete recanalization.14 Recanalization was evaluated at 3 to 6 months in 4 studies, at 1 year in 3 studies, and at both time points in 2 studies. If the rates of recanalization presented in these studies are combined numerically, there was no difference in the rate of recanalization at 3 months and at one year of follow-up (59 of 70 84% at 3 months and 60 of 7085% at 1 year). These results suggest that recanalization occurs only within the first months following CVT and not thereafter, irrespective of anticoagulation.,少见原因引起的颅内静脉窦血栓形成,常见脑静脉和静脉窦血栓的病因有:(1)局限性的血栓形成,常由头面部、耳部的感染所致;如颌面疖肿引起的海绵窦、耳部感染引起乙状窦或横窦的炎性血栓等。(2)多发和广泛性脑静脉和静脉窦血栓，一般认为与静脉血管壁的损伤、血液的高凝状态、感染及妊娠和免疫反应等因素有关。多数学者认为,有三方面因素可促进脑静脉血栓的发展,血液凝固性增强;血液淤滞;血管壁异常。但有些罕见的病因也能引起颅内静脉窦血栓形成如，甲状腺机能亢进，结核性脑膜炎，肾病综合征，使用雄激素，系统性红斑狼疮，闭合性颅脑损伤，伤寒和白塞病，这些疾病通过治病因素改变全身或局部的血流或机体的免疫状态，致使颅内静脉窦血栓形成，有些发病原因现在还不太清楚,1991年Schutta等在Stroke上首先报道了1例遗传性纤溶酶原缺乏症且伴有甲状腺毒症的患者发展成,由此提出甲状腺毒症可能在的发生中起一定的作用。本组中1例甲亢为少年男性,在出现静脉窦血栓之前,一直服用抗甲亢药物。入院前6个月自主停药导致甲亢复发，甲亢可导致血液的凝固性增加,促使发生。本例有三个血栓形成因素：甲亢患者有房颤病史，心房动作不协调不规律导致心内血流异常，可促使凝血酶原形成。血浆纤维蛋白原增高使血液粘稠度增高，使血小板容易聚集形成血栓。血栓形成过程中重要的抑制因子C 蛋白降低，增加血栓形成的危险性。甲状腺机能亢进能使静脉血液停滞，也是脑静脉血栓形成的原因。另外,甲亢是免疫性疾病,是否通过免疫机制造成血管壁的损伤而致血栓形成,有待于进一步探讨,本例结核性脑膜炎合并静脉窦血栓病例为年轻患者，首发症状主要为发热，头痛呕吐，查体有颈抵抗及明显的视乳头水肿，CSF蛋白明显增高，糖与氯化物降低，白细胞中等程度增高，淋巴细胞为主，结核菌DNA实验阳性，结核性脑膜炎的诊断成立，头MRI，MRV证实有静脉窦血栓，联合抗痨的同时，应用皮质激素，甘露醇及低分子右旋糖酐，抗血小板治疗取得满意效果。本病例结脑合并脑静脉窦血栓，推测在发病机制上有一定的因果关系，一方面炎症病变累及硬脑膜，影响静脉窦血液回流，另一方面发热呕吐全身衰竭导致血液浓缩，呈高凝状态，易于血栓形成。本例病例提示，对于结脑病人尤其是难以完全解释的高颅压，及定位体征，宜及时进行MRI，MRV检查，以证实是否和并脑静脉窦血栓,2例肾病综合征患者均为中年男性，以肾病综合征入院，入院后出现头痛，呕吐，视物模糊，等高颅压症状，及神经系统损害体征，给予MRI，MRV，DSA检查，显示上矢状窦后部及双侧枕窦闭塞，给与颈动脉溶栓治疗，即肝素抗凝治疗，病情好转。由于肾病综合征患者血液中的凝血因子改变,包括、因子减少,、因子、纤维蛋白原、-血栓