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1、Natural history of cerebral vein thrombosis: a systematic review,Mortality The mortality rate during the acute phase was assessed in 12 Studies,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 studies and
2、was 0 in 5 studies. Of 45 evaluated patients, 32 died as a consequence of CVT (most of them for cerebral herniation), whereas most of 13 died as a consequence of an underlying disease. The overall mortality rate at the end of follow-up was 9.4% (122 deaths in 1303 total patients). However, it was hi
3、ghly variable between the studies (range, 0%-39%). Noteworthy,most of the deaths that occurred during follow-up were related to an underlying condition such as cancer (24 of 38 evaluated deaths during follow-up), and they were not a direct consequence of cerebral vein thrombosis,Disability Outcome i
4、nformation 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 re
5、covery, 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,R
6、ecanalization 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
7、 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
8、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 70
9、85% 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
10、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
11、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
12、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，DS
13、A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、-血栓球蛋白、和血小板增高。血小板的粘附和凝集力增强。抗凝血酶和抗纤溶酶活力降低。当血浆白蛋白2025/,血栓形成的危险性明显增加。个别病例极易误诊,把颅内静脉窦血栓患者症状中的性格改变归于使用激素的副作用,从而延误了正确治疗的时机。因而对此类患者如出现颅内压增高及神经系统损害体征应及时给予MRI，MRV，检查以确定是否和并脑静脉窦血栓。,本例病人男，22岁，健身训练5年，5月前开始雄激素肌肉注射，以头痛呕吐入院，查体最明显变现为使神
14、经乳头水肿，经MRI，MRV检查确诊为上矢状窦，横窦血栓。给予肝素抗凝和降颅压治疗，患者痊愈出院。由于雄激素已经被证明的肌营养作用，而使雄激素经常被运动员使用，许多数据表明睾酮能减低纤维蛋白原和PAI-1，然而与它的抗凝作用和纤溶作用相比，它激活血小板的功能更强。雄激素通过减低环氧化酶的活性来增加血小板的功能，进而导致血栓形成，这是近期大家较为认同的解释。,本例病人为系统性红斑狼疮（SLE）并发脑静脉血栓形成，SLE病人可以通过以下机制导致脑静脉血栓形成，脑血管的炎症改变是主要原因，在中枢神经系统受累的SLE患者中血液中出现狼疮抗凝物和抗磷脂抗体者高达40，其中抗磷脂抗体通过抑制蛋白C和蛋白S
15、的作用来促进血栓的形成，这也与血栓的形成密切相关，此外，纤维蛋白溶解缺乏。抗凝血酶功能的改变，高纤维蛋白血症和血凝状态改变导致的血液高凝状态是血栓形成的又一原因。本例给予羟氯喹，皮质激素，环磷酰胺和肝素抗凝治疗，患者病情得到控制，好转出院,本例患者为2岁男孩，由1米高处落下，为枕部受力导致的闭合性脑损伤，当时未发生症状，次日早晨出现呕吐来院就诊，查体肌力减弱，共济失调步态，CT,MRI检查显示左侧乙状窦闭塞，给予抗凝治疗，患者痊愈。闭合性颅脑损伤导致脑静脉窦血栓的原因包括：静脉窦内皮损害激活凝血系统，脑损伤后大量凝血酶释放，凝血酶会导致血小板和红细胞破坏而使机体处于高凝状态等，此患者脑外伤未合
16、并颅骨骨折而导致血栓形成十分罕见，在出现症状后早期诊断有助于提高患者预后,伤寒病发脑炎国内外均有报道，但伤寒导致脑静脉血栓形成实数罕见，本例患者以畏寒，发热头痛起病，体表特征，实验室检查均支持伤寒诊断，头颅MRI见脑膜强化，DSA证实上，下矢状窦，双侧横窦，乙状窦血栓形成，故确诊为伤寒性脑膜炎并颅内静脉窦血栓形成，本病例可能是伤寒杆菌透过血脑屏障导致脑膜静脉炎性改变，血管壁受到损害致使凝血物质释放增多，导致血栓形成，此外伤寒患者全身状态较差，对静脉血栓形成也有一定的作用。本病例针对原发病治疗的同时给予甘露醇及抗凝治疗，患者病情好转,Behcet病(BD)引起神经系统受累时,则称为神经白塞病(NBD)。NBD是BD的严重并发症之一,颅内小血管炎是其基本病变。NBD一般在BD基本症状出现数月或数年后发病,NBD的DSA多呈现大、中、小血管的广泛狭窄和血栓形成。本病例特点:(1)青年男性;(2)BD发病前有感染史,BD发病后3年出现上矢状窦闭塞的症状与体征;(3)皮肤、口腔、龟头处可见溃疡,反复发作,针刺反
