脑静脉窦血栓形成的诊断

目的探讨诊断脑静脉窦血栓形成(CVST)的方法。方法对54例CVST患者的发病诱因、起病形式、临床表现、影像学特征以及血液和脑脊液检查等资料进行回顾性分析。结果 54例CVST患者平均年龄(33.33±12.20)岁,其中45岁以下45例。在妊娠和产褥期(31.5%)、脱水(22.2%)、上呼吸道感染(18.5%)、乳突炎(9.3%)等诱因下急性或亚急性起病,首发出现头痛(87.0%)、恶心(50.0%)、性发作(7.4%)、意识障碍(5.5%)以及肢体无力(3.7%)等症状,同时有D-二聚体水平增高,影像学表现为静脉窦闭塞及脑组织水肿或梗死,伴有出血性病变。多数脑脊液压力增高、白细胞数正常。结论在妊娠、产褥、脱水、感染等诱因下,出现头痛、恶心、意识障碍、性发作等症状的青年患者,要高度怀疑CVST,应及时行头颅CT或MRI检查;D-二聚体增高的情况下,脑静脉造影和脑脊液检查有助于进一步确诊。     

Latent Cerebral Venous and Sinus Thrombosis

The incidence of cerebral venous and sinus thrombosis (CVST) is still unknown. Several assumptions of the incidence have been made. In the one and only series of consecutive brain autopsies series by Towbin in1973 CVST was found in 10,9 % of patients aged 60 years or more. These findings suggest that the true incidence of CVST is higher than generally thought, but there are no other reports supporting these findings. Therefore in order to determine the incidence of latent CVST we conducted a new prospective post mortem study. All brain autopsies performed in our hospital between 1996 and 1998 on patients 60 years of age or older were examined for the presence of CVST in a prospective way. We examined the sagittal sinus, the torcula, the straight sinus, both transverse sinuses, and both sigmoideal sinuses for the presence of thrombosis. The clinical condition, medication, brain-imaging results, clinical cause of death, general findings from the body autopsy and definite cause of death were recorded. Additionally we requested the annual mortality figures of CVST at the department of the National Agency for Statistics of the Netherlands (CBS). A consecutive and prospective series of 102 brain autopsies was performed in our general hospital during a period of two years. We found one case of latent CVST in our series of 102 patients. Comparison with the findings of Towbin using a chi-square test yielded a significant difference in the incidence of latent CVST (χ₂ = 7.65, p < 0.01) between the two studies. The present autopsy study demonstrates that latent CVST is rare. Received: 7 June 2002, Received in revised form: 14 October 2002, Accepted: 21 October 2002 Correspondence to H. P. Bienfait, MD     

妊娠相关颅内静脉窦血栓研究进展

颅内静脉窦血栓（cerebral venous sinus thrombosis,CVST）是指由于多种病因引起的脑静脉 回流受阻、颅内高压为特征的特殊类型的脑血管病,具有较高死亡率。成年女性则以口服避孕药物和 围产期多见。由于妊娠引起的CVST危险因素较多,发病机制复杂,早期对妊娠合并CVST的诊治具有一 定难度。本文结合近几年国内外对妊娠相关CVST的病因和发病机制、临床表现和辅助检查、治疗等 几个方面的研究进展进行阐述,以期早期发现、优化治疗方案,降低死亡率,为临床诊治提供依据。     

孕产妇合并颅内静脉窦血栓形成的诊疗策略

目的 探讨孕产妇合并颅内静脉窦血栓形成可能的病因、临床表现、影像学特征、诊断,总结诊疗策 略。 方法 回顾性分析北京协和医院神经内科1993-2013年连续收治的孕产妇合并危重颅内静脉窦血 栓形成患者12例,平均年龄（26±5）岁（19～34岁）。12例患者均在急性/亚急性期行计算机断层扫描 （computed tomography,CT）或磁共振成像（magnetic resonance imaging,MRI）检查;9例行磁共振静脉 成像（magnetic resonance venography,MRV）,6例行数字减影血管造影（digital subtraction angiography, DSA）检查诊断静脉窦血栓形成。分析可能的病因、临床表现、影像学特征、诊断及治疗策略。 结果 发病时期：孕早期7例,孕晚期1例,产后2周内4例,人工流产后1例;合并疾病包括：蛋白S缺乏 1例,高同型半胱氨酸1例,特发性血小板减少性紫癜1例,全身感染1例,贫血1例;临床特征：均为急性 或亚急性起病。头痛12例,痫性发作5例,其他局灶性神经系统体征5例（包括肢体无力、病理征、麻 木、共济失调、周围性面瘫等）,视力下降1例,不同程度意识障碍4例。12例患者中9例存在脑实质异 常,其中脑梗死或出血性梗死7例,脑出血1例,脑组织明显肿胀1例。受累静脉窦：横窦、乙状窦9例, 上矢状窦8例,直窦、下矢状窦2例,颈静脉2例。治疗：6例妊娠期发病患者均及时终止妊娠,1例因家 属不同意终止妊娠,病情加重,自动放弃回家。10例积极尽早给予肝素化抗凝治疗,所有患者均病情 好转出院。 结论 孕产妇出现急性/亚急性头痛、抽搐及其他局灶体征时,需警惕危重颅内静脉窦血栓形成可 能;影像学提示符合静脉窦引流区域的脑实质异常信号（出血性梗死为主）,可作为及时诊断的重要 线索;病情危重时及时选择终止妊娠去除病因可能与良好预后相关,同时需尽早开始抗凝治疗。     

特发性颅内高压与颅内静脉窦血栓形成

特发性颅内高压(idiopathic intracranial hypertension,IIH)以往曾称为假性脑瘤(pseudotumor cerebri,PTC)或良性颅内高压(benign intracranial hypertension,BIH),是指一组颅内压升高、但颅脑计算机断层扫描(computer tomography,CT)检查无脑积水或颅内占位、且脑脊液成分正常的综合征.     

Duration of Anticoagulation After Cerebral Venous Sinus Thrombosis

Cerebral venous sinus thrombosis (CVST) often occurs in young patients and is treated with acute and then long-term oral anticoagulation. It is important to decide when to discontinue anticoagulation, as lifelong anticoagulation exposes the patient to considerable cumulative risk. However, a lack of high quality studies makes choosing a duration of anticoagulation after CVST difficult. In this article, we review the evidence for different treatment durations in several common clinical scenarios of CVST. In addition, when no direct evidence is available, we discuss and extrapolate from the more comprehensively studied situation of systemic venous thromboembolic disease. Recommendations are graded using standard criteria for the level of evidence.     

感染性脑静脉窦血栓的临床分析

目的 探讨感染性脑糠脉窦血栓（CVST）的特点及治疗策略。方法 对10例由感染所致的CVST患者的病因、诊断、治疗情况进行回顾性分析。结果 10例患者中，4例继发于乳突感染，2例继发于头面部疖肿感染，3例继发于中耳炎，1例由单纯疱疹病毒性脑炎导致。患者最初主要为头痛、发热等局部感染表现，进而出现颅内压增高及神经系统症状。实验室检查白细胞升高，头部CT、MRI、DSA等检查显示患者有不同程度的脑静脉窦闭塞。10例患者均行抗感染治疗，其中6例给予抗凝治疗，4例给予局部溶栓治疗。治疗后患者痊愈7例，好转2例，死亡1例。结论 头面部感染及全身性菌血症患者，如出现颅内压增高等神经系统症状时应考虑CVST。早期诊断及治疗可以明显改善患者预后。     

脑静脉窦血栓的综合血管内治疗

目的评价综合应用颈动脉溶栓、静脉窦置管溶栓及静脉窦支架置入术治疗脑静脉窦血栓的疗效。方法对我科1997年7月～2005年6月收治的99例脑静脉窦血栓患者进行综合血管内治疗,其中5例患者接受静脉窦置管溶栓合并静脉窦支架置入术(方案1),38例患者接受颈动脉溶栓合并静脉窦置管溶栓(方案2),56例患者接受单纯颈动脉溶栓(方案3),观察3组患者的临床疗效。结果经治疗,99例患者中有94例颅内压降至300 mm H_2O, 75例患者静脉窦实现再通,93例患者循环时间降至11 s以下。结论综合应用颈动脉溶栓、静脉窦置管溶栓及静脉窦支架置入术等方法治疗脑静脉窦血栓是一种安全、有效的手段。     

重症脑静脉窦血栓形成的血管内溶栓抗凝治疗

目的探讨重症脑静脉窦血栓形成血管内溶栓抗凝治疗的效果与安全性。方法选择传统治疗方法无效的重症脑静脉窦血栓形成病例2例,采用在数字减影血管造影(DSA)下静脉窦接触性溶栓抗凝与颈内动脉抗凝溶栓联合治疗。结果2例病人临床症状全部缓解,闭塞静脉窦开通,无严重并发症发生。结论血管内介入溶栓抗凝治疗可能是治疗重症脑静脉窦血栓形成的一种安全有效的方法。     

Cerebral Venous Sinus Incision for Surgical Thrombectomy Combined with Thrombolysis During Decompressive Craniectomy for Malignant Cerebral Venous Sinus Thrombosis Complicated with Cerebral Hernia

For malignant cerebral venous sinus thrombosis (CVST) complicated with cerebral hernia, decompressive craniectomy may be life-saving, and thrombectomy combined with thrombolysis may obtain better outcomes. This report describes an approach performed on 2 patients diagnosed with CVST combined both thrombectomy and thrombolysis with decompressive craniectomy through incising the superior sagittal sinus. The general procedure of the operation is as follows. The anterior part of the superior sagittal sinus was exposed firstly. After cutting the dura matter for decompression, a superior sagittal sinus incision was taken to detect sinus thrombus. In order to facilitate hemostasis during detecting the sagittal sinus, 2 silk sutures were sutured along the incision. The incision was 5 millimeters long approximately along the middle line of the front third of the superior sagittal sinus. A silicone intubation was inserted in the sinus through the incision. Thrombus was seen in the suction tube. At a depth of about 10 cm, while it is difficult to penetrate the tube, we used the gelatin sponge to cover the sinus incision and fixed the suture lines after cross-knotting. The silicone intubation was drawn out through the forehead and connected to external micro pump for injecting anticoagulant drugs, then cut the dura mater into star-shaped and discard bone flap for decompression. Absorbable artificial dura mater was used to repair bilateral dura mater, respectively. At last, connect the catheter to the micro pump for pumping anticoagulant. After operation, the 2 patients received thrombolysis through the catheter placed in the sinus. Both of them recovered well. There was no incision-related bleeding occurred after surgery. Both the patients achieved incredibly good outcomes. For patients with malignant cerebral venous sinus thrombosis, acute cerebral hernia or cerebral hernia tendency, it may be an effective approach combined both thrombectomy and thrombolysis with decompressive craniectomy through incising the superior sagittal sinus.     

脑静脉窦血栓形成24例分析

目的：探讨脑静脉窦血栓形成（CVST）的诊断及治疗策略。方法：对24例确诊为CVST患者的病因、临床表现、影像学特征、治疗方法及预后进行回顾性分析。结果：24例患者中,经各种方法治疗后4例痊愈,14例好转出院,1例死亡,5例自动出院。结论：CVST病因复杂,临床表现无特异性,确诊需行MRI／MRV或DSA检查;早期给予抗凝、溶栓及病因的对症治疗可获得较好效果。     

颅内静脉窦血栓形成的头痛特点分析

目的 通过分析颅内静脉窦血栓形成（cerebral venous sinus thrombosis,CVST）的病例,总结CVST患者 头痛的特点,提高神经科医师对该病的认识。 方法 收集2007年1月-2016年12月在北京大学第三医院就诊的CVST病例,回顾其头痛特点、临床表 现、影像学检查及化验结果,并复习相关文献进行分析总结。 结果 共入组18例CVST患者,本组CVST最常见的部位是横窦（77.8%,14/18）,其次为乙状窦（61.1%, 11/18）和上矢状窦（50%,9/18）;14例（77.8%）存在多个颅内静脉窦血栓。所有患者均以头痛为首 发症状,其中7例（38.9%）为单纯性头痛;11例合并其他临床症状,包括癫痫（38.9%,7/18）、呕吐 （44.4%,8/18）、肢体麻木（11.1%,2/18）、肢体无力（16.7%,3/18）、眼部症状（22.2%,4/18）以及 意识障碍（11.1%,2/18）。9例（50%）为偏侧头痛,其中8例（88.9%,8/9）存在同侧CVST;头痛视觉 模拟评分法（visual analogue scale,VAS）评分平均7.0分（3～10分）;头痛发病形式5例（27.8%）为急性, 12例（66.7%）为亚急性,1例（5.6%）为慢性。 结论 头痛是CVST最常见的首发症状,偏侧头痛CVST患者血栓以头痛同侧多见;头痛亚急性起病的 患者,尤其需注意CVST的可能性。     

脑静脉窦血栓形成的诊断与血管内治疗

目的 探讨脑静脉窦血栓形成的诊断和血管内溶栓治疗。方法 对8例以顽固性头痛、呕吐伴或不伴局灶性神经功能缺损或癫发作为主要表现的患者，采用颅脑MRI和脑血管造影检查确定诊断，并施行经颈内动脉、静脉窦内接触性溶栓或静脉窦内支架成形术协同抗凝治疗。结果 MRI显示相应脑静脉窦区出现长T2WI，短T1WI信号，DSA显示脑静脉窦闭塞、静脉侧支循环建立和脑动静脉循环时间延长。治疗后静脉窦完全或部分再通，脑循环时间接近正常，临床病情显著缓解。结论 血管内介入治疗可能是脑静脉窦血栓形成最有效的治疗方法之一，有待大样本研究证实。     

Acquired and Congenital Risk Factors associated with Cerebral Venous Sinus Thrombosis

The mechanistic paradigm underlying venous thrombosis at atypical locations stems from the observation that most events occur as a result of pathology of the organ system drained by the involved venous segment. Cerebral venous sinus thrombosis stands apart as an exception to this general rule. Although brain and sinus pathology are well established causes, these combined variables account for approximately one third of cases. The marked female preponderance and strong association with gender specific risk factors including hormonal manipulation, pregnancy and the puerperium are particularly notable. Factor V Leiden and prothrombin G20210A mutations and hyperhomocysteinemia represent important risk factors particularly when combined with acquired variables. The association with oral contraception use and the prothrombin G20210A gene mutation may offer insights into the anatomic predilection for cerebral venous sinus involvement as compared to venous thrombosis of the lower extremities. The intent of this review is to summarize the corporate literature of both acquired and congenital risk factors associated with cerebral venous sinus thrombosis in order to assist clinicians in their search for underlying mechanisms and to risk stratify patients for anticoagulation treatment duration and risk of recurrent thrombosis.     

颅内静脉窦血栓形成的遗传性易栓危险因素研究现状

超过20%的颅内静脉窦血栓形成患者可以检测到易栓症。常见的遗传性易栓症有凝血因 子缺陷、抗凝蛋白缺乏、纤溶异常、高同型半胱氨酸血症、凝血因子水平增高等。本文介绍颅内静脉 窦血栓形成相关的遗传性易栓危险因素。     

白塞病相关颅内静脉窦血栓形成9例分析

目的 研究白塞病相关颅内静脉窦血栓形成（cerebral venous sinus thrombosis,CVST）的临床表现、血 栓好发部位、治疗及预后。 方法 回顾性分析2014年1月-2016年8月于首都医科大学附属北京同仁医院神经内科住院的白塞病 相关CVST患者的临床资料。 结果 共收集白塞病相关CVST患者9例,其中男性2例（22.2%）,女性7例（77.8%）,年龄21～47岁,中 位数是35.9岁;急性起病1例（11.1%）,慢性起病8例（88.9%）;头痛4例（44.4%）、头晕1例（11.1%）、 阵发性黑蒙3例（33.3%）、双眼视力下降5例（55.6%）、视盘水肿9例（100%）、复视3例（33.3%）、耳 鸣1例（11.1%）;乙状窦血栓形成7例（77.8%）、横窦血栓5例（55.6%）、直窦血栓1例（11.1%）、下矢状 窦血栓1例（11.1%）、颈内静脉血栓2例（22.2%）;仅1处静脉窦受累的4例（44.4%）,同时有2处及2处 以上静脉窦受累的5例（55.6%）;所有患者均予醋甲唑胺50 mg 2次/日口服,3例予糖皮质激素冲击 治疗,其中1例合并华法林抗凝治疗、2例进行了腰大池-腹腔分流手术。随访1～32个月,所有患者治疗 后均好转。 结论 白塞病可引起颅内静脉窦血栓形成,可累及单处或多处静脉窦,CVST患者需除外白塞病可能。     

跨横窦硬膜外血肿并脑静脉窦血栓形成的诊治

目的探讨跨窦硬膜外血肿并脑静脉窦血栓形成的诊疗方法。方法对近三年来临床工作中发现的9例跨窦硬膜外血肿并脑静脉窦血栓形成患者的临床资料进行回顾性分析。所有病例诊断均经MRI、MRV证实,并静脉使用尿激酶溶栓治疗,首剂20万U,每日增加5万U。最大剂量40万U。结果9例跨窦硬膜外血肿并脑静脉窦血栓形成经治疗后4例再通,5例建立侧支循环;无1例死亡,无原有血肿扩大及再出血病例。结论对临床工作中发现腰穿颅内压大于300mmH2O,跨窦骨折合并硬膜外血肿的病例,应警惕静脉窦血栓形成。