Interobserver agreement in the magnetic resonance location of cerebral vein and dural sinus thrombosis

The interobserver variation in the magnetic resonance (MR) location of cerebral vein and dural sinus thrombosis (CVT) has not been previously reported. Four independent observers rated a convenience sample of 40 MR/MR angiographies to assess whether or not each dural sinus and major cerebral veins were occluded. Interobserver reliability was measured using κ statistics. Interobserver agreement was comparable between the six pairs of raters. Agreement was excellent for thrombosis of the deep cerebral venous system ( κ  = 1.00), cerebellar veins ( κ  = 1.00), superior saggital sinus ( κ range: 0.82–1) and right jugular vein ( κ range: 0.84–0.95); good to excellent for the right transverse/sigmoid sinus ( κ range: 0.75–0.90) and the left jugular vein ( κ range: 0.65–0.85); moderate to excellent for the left lateral sinus ( κ range: 0.59–0.78) and the straight sinus ( κ range: 0.59–0.92); poor to good for the cortical veins ( κ range: 0.02–0.65). Agreement between observers varies with the location of CVT. It is good or excellent for most of the occluded sinus and veins, except for the cortical veins. This study suggests that information on the location of CVT can be reliably collected and used in multicentre studies.     

Abducens nerve palsy due to inferior petrosal sinus thrombosis

Isolated unilateral abducens nerve palsy is usually due to ischemia, trauma or neoplasm. Dorello’s canal is the space between the petrous apex and superolateral portion of the clivus, bound superiorly by Gruber’s ligament. The abducens nerve travels with inferior petrosal sinus (IPS) though the Dorello’s canal before entering the cavernous sinus. A 31-year-old man presented with neck pain, and binocular horizontal diplopia, worse looking towards left and at distance. He had a history of intravenous drug abuse but no history of hypertension or diabetes. On examination, he had complete left 6th nerve palsy with normal fundi, pupils, and other cranial nerves. Methicillin-resistant Staphylococcus aureus bacteremia was detected with naïve tricuspid valve endocarditis and multiple septic emboli to lungs with infarcts. His cerebrospinal fluid was normal. MRI of the brain was normal. MRV of head and neck showed thrombosis of the left internal jugular vein, left sigmoid sinus and left inferior petrosal sinus with normal cavernous sinus and no evidence of mastoiditis. He was treated with broad spectrum antibiotics. He was not anticoagulated for fear of pulmonary hemorrhage from pulmonary infarcts. Although cerebral venous sinus thrombosis commonly presents with elevated intracranial pressure, isolated ipsilateral 6th nerve palsy from its compression in Dorello’s canal due to thrombosis of the ipsilateral inferior petrosal sinus is extremely rare. To our knowledge, only two patients have been reported with isolated abducens palsy due to IPS thrombosis; one caused by septic emboli and the other developed it during IPS cortisol level sampling.     

Thrombus of the left lateral sinus spreading to the internal jugular vein

Cerebral venous thrombosis is an uncommon event which presents a wide spectrum of sometimes extraneurological signs different from the classical clinical presentation. We report the cases of two middle-aged women who developed thrombosis of the left lateral sinus spread-ing to the internal jugular vein from the sigmoid sinus. The time course of the symptoms suggested that intracranial thrombosis occurred first. No infectious or neoplastic local disease could be found but both women were taking oral contraceptives. Medical treatment led to good reperfusion of the intracranial sinuses but occlusion of the jugular vein persisted despite prolonged oral anticoagulants. Long-term outcome was favorable with residual benign epilepsy in one patient, and occurrence of an arteriovenous fistula in the other.     

An unusual case of neuro-Behçet’s disease presenting with co-occurence of cerebral venous sinus thrombosis with basilar artery occlusion

Non-parenchymal neuro-Behçet disease generally affects cerebral venous sinuses, whereas intracranial intracerebral arterial involvement has been rarely reported. But co-involvement of both intracranial intracerebral artery and venous vascular systems in a patient at the same time has not been mentioned before. To the best of our knowledge, this case involving a 25-year-old male with a 7-year history of Behçet disease is the first reported of this type of involvement. He developed occlusion of the basilar artery together with thrombosis of the left sigmoid sinus, distal internal jugular vein, and straight sinus. He was successfully treated with a combination of high-dose steroid and cyclophosphamide. Cranial magnetic resonance angiography demonstrated the resolution of these abnormalities.     

Early Spontaneous Recanalization of Sigmoid Sinus Thrombosis Following a Closed Head Injury in a Pediatric Patient : A Case Report and Review of Literature

Cerebral venous sinus thrombosis (CVST) following a closed head injury in pediatric patients is a rare condition, and an early spontaneous recanalization of this condition is extremely rare. A 10-year-old boy was admitted with a mild, intermittent headache and nausea five days after a bicycle accident. The brain computed tomography showed an epidural hematoma at the right occipital area with pneumocephalus due to a fracture of the occipital skull bone. The brain magnetic resonance imaging and the magnetic resonance venography demonstrated a flow signal loss from the right sigmoid sinus to the right jugular vein. The diagnosis was sigmoid sinus thrombosis, so close observations were selected as a treatment for the patient because of his gradually improving symptoms; however, he complained of vomiting 14 days the after conservative treatment. The patient was readmitted for a further examination of his symptoms. The laboratory and the gastroenterological examinations were normal. Due to concern regarding the worsening of the sigmoid sinus thrombosis, the brain magnetic resonance venography was rechecked and it revealed the recanalization of the venous flow in the sigmoid sinus and in the jugular vein.     

改良溶栓方案治疗脑静脉窦合并颈内静脉血栓形成

目的探讨改良溶栓方案【静脉窦内微量持续泵点尿激酶(100,000u/24h)】治疗脑静脉窦合并颈内静脉血栓形成的疗效。方法对11例患者进行机械性破栓、静脉窦内留置微导管行最低量尿激酶100,000u/24h静脉窦直接泵点滴48～96h治疗。术后积极治疗原发病,抗凝治疗6个月。术后随访6～12个月,平均10个月。结果 11例脑静脉窦合并颈内静脉血栓形成患者,脑静脉窦均获得再通(其中8例患者应用尿激酶100,000u/24h效果良好,3例患者在应用尿激酶100,000u/24h,48h复查后增量至250,000u/24h),1例颈内静脉未通,预后良好。结论改良溶栓方案可有效治疗脑静脉窦合并颈内静脉血栓形成,有待大宗病例进一步证实。     

Can anticoagulation therapy in cerebral venous thrombosis associated with Behçet's disease be stopped without relapse?

There is as yet no consensus on the treatment of cerebral venous thrombosis (CVT) in Behçet's disease, and the place of anticoagulation is also still being debated. This report is of a series of seven patients with Behçet's disease (BD)-associated CVT, for which anticoagulation was stopped, and discusses the possibility of stopping anticoagulation during follow-up while receiving optimal treatment for BD. The diagnosis of BD was established during follow-up, which lasted a median of 120 [range: 60–1490] days after CVT diagnosis. The median duration of anticoagulation therapy was 29.5 months. On stopping anticoagulation, concomitant treatment then included colchicine, steroids and azathioprine, all introduced after BD was diagnosed. With a median follow-up of 25 months after anticoagulation interruption, only one relapse of CVT was observed. No relapse of CVT or other venous thrombosis was observed in the six patients treated by steroids associated with an immunosuppressant or colchicine. Our results emphasize that corticosteroids are essential for the treatment of BD-associated CVT, and that anticoagulant therapy may be safely stopped during follow-up in the presence of optimal BD treatment (steroids alone or with immunosuppressive drugs).     

Superior Sagittal Sinus Thrombosis in the Presence of Idiopathic Bilateral Internal Jugular Vein Stenosis

Cerebral venous outflow obstruction and anomalies in cerebral venous circulation predispose to dural sinus thrombosis. This case report illustrates the magnetic resonance and angiographic findings in a patient who had superior sagittal sinus thrombosis secondary to idiopathic bilateral internal jugular vein stenosis, a previously unrecognized entity. The findings suggest that bilateral stenosis of the internal jugular veins at their junction with the innominate veins causes obstruction to cerebral venous outflow leading to dural sinus thrombosis.     

Spontaneous Epidural Hematomas due to Cerebral Venous Thrombosis in a Patient with Immune Thrombocytopenic Purpura

IntroductionIntracerebral haemorrhage in patients suffering from cerebral venous thrombosis (CVT) is relatively uncommon. CVT typically occurs in hypercoagulable state of various causes. Some drugs play a causative role in CVT and thrombopoietin receptor agonists are among them.Case summaryWe present a female patient with refractory immune thrombocytopenic purpura (ITP) treated with romiplostim, suffering from severe thrombosis of jugular vein expanding intracranially. Despite being treated with adequate anticoagulation, she developed spontaneous bilateral epidural and subdural hematomas with devastating outcome.DiscussionTo our knowledge, this is the first reported case of spontaneous atraumatic epidural hematomas due to CVT in adult patient. We support that in our patient, blood stasis leading to the dissection of dura mater, platelet dysfunction, and anticoagulation treatment contributed to the formation of the intracranial, extracerebral haemorrhages.     

闭合性颅脑损伤后乙状窦栓塞的诊断与治疗

目的讨论闭合性颅脑损伤后乙状窦栓塞的诊断方法及治疗原则。方法采用扩大版的科技引文索引检索系统获得1995年1月至2010年1月关于闭合性颅脑损伤后乙状窦栓塞的7项研究中的15名患者,结合本组1例,共16例。通过分析,明确其临床概况、诊断方法、治疗原则及预后。结果 16例闭合性颅脑损伤后乙状窦栓塞患者平均年龄20.9岁,伤后GCS评分13~15分,剧烈头痛、呕吐等颅内压增高症状明显。12例栓塞在右侧,4例在左侧。12例合并有横窦或颈静脉栓塞。13例有颅骨骨折表现,且骨折与乙状窦栓塞均为同侧。10例仅对症治疗,5例接受抗凝治疗(其中2例还接受了手术治疗),另有1例仅接受了手术治疗。所有患者预后良好。结论闭合性颅脑损伤后出现无法解释的颅内压增高表现时应高度怀疑乙状窦栓塞,CTA、磁共振静脉造影以及DSA可以明确乙状窦栓塞的诊断。大部分患者经观察对症治疗可取得满意效果,部分患者可在监测凝血功能条件下行抗凝治疗。     

Cerebral venous thrombosis associated with tentorial subdural hematoma during oxymetholone therapy

Androgen was reported to cause cerebral venous thrombosis (CVT) during replacement therapy for aplastic anemia. Oxymetholone, a synthetic androgen analogue, has been widely used in the treatment of aplastic anemia. A 40-year-old woman with aplastic anemia visited our hospital because of severe headache, nausea, vomiting, blurred vision and diplopia for a period of 1 month. She had taken oxymetholone for 2 years. Neurological examination revealed bilateral papilledema and bilateral sixth nerve palsies. Brain magnetic resonance imaging (MRI), performed at the time of admission, demonstrated left-sided tentorial SDH, and focal cerebral thrombosis of the left superficial sylvian vein and sigmoid sinus. MR venography revealed multiple irregularities in the superior sagittal sinus and left transverse sinus. CVT with tentorial subdural hematoma (SDH) caused by oxymetholone was strongly suggested. Oxymetholone was immediately discontinued, and her symptoms and signs disappeared. Because of the thrombocytopenia, anticoagulation was not started. She was discharged and visited the outpatient clinic without neurological symptoms for 6 months. This report supports the cautions given about the risk of CVT with oxymetholone supplementation in aplastic anemia. To the best of our knowledge, this is the first report of CVT associated with tentorial SDH that was probably caused by oxymetholone.     

单发和多发脑静脉血栓形成的临床特征分析

目的 探讨单发和多发脑静脉血栓形成(CVT)患者的临床特征和短期预后. 方法 总结分析136例CVT患者的血栓部位及受累静脉窦/静脉数目,依受累颅内静脉窦/静脉数目将患者分为单发CVT组和多发CVT组,采用单变量分析比较2组患者的临床特点及预后. 结果 单发CVT组44例,多发CVT组92例(其中累及2个部位45例,3个部位35例,4个部位9例,5个部位3例).最常受累的静脉窦/静脉为横窦/乙状窦(86.8%),其后依次为上矢状窦(58.1%)、直窦(18.4%)、大脑深静脉系统(7.4%)和皮层静脉系统(2.9%).单变量分析显示单发CVT组患者平均发病年龄大于多发CVT组,但短期预后好于多发CVT组,比较差异有统计学意义(P＜0.05);多发CVT组患者出现颅内实质病变及合并颅外静脉血栓的几率高于单发CVT组,同时治疗前颅内压≥300mm H2O的患者比例高于单发CVT组,比较差异有统计学意义(P＜0.05). 结论 CVT中以多发CVT所占比例较高,最容易受累的部位为横窦/乙状窦和上矢状窦.与单发CVT相比,多发CVT患者颅内压较高,多合并颅外静脉血栓,出现静脉性脑梗死和脑出血的几率较高,临床病情重,预后相对较差.     

MRI和MRV在脑静脉和静脉窦血栓诊断中的价值

目的探讨MRI和MRV对脑静脉和静脉窦血栓(CVST)形成的诊断价值。方法回顾性分析16例CVST患者的临床和影像学资料。结果受累的脑静脉和静脉窦有上矢状窦9例,横窦5例,直窦3例,乙状窦2例,大脑大静脉合并大脑内静脉2例。常规MRI可见受累静脉窦流空信号消失,静脉窦内信号高低不一,11例脑实质内出现静脉性脑梗死表现,其中4例合并出血。增强扫描15例静脉窦内出现充盈缺损及静脉窦壁强化表现,9例脑实质内出现脑回样强化。MRV表现为受累静脉窦部分或广泛高血流信号缺失、狭窄。结论MRI和MRV是诊断和随访CVST形成的最佳检查方法。     

Facial palsy in cerebral venous thrombosis : transcranial stimulation and pathophysiological considerations

BACKGROUND: Cranial nerve palsy in cerebral sinovenous thrombosis (CVT) is rare, its pathophysiology remains unclear, and data from electrophysiological examinations in such patients are missing. CASE DESCRIPTION: We report the case of a 17-year-old woman with familial protein S deficiency who was admitted with extensive multiple CVT. Two weeks after onset of symptoms, she developed isolated right peripheral facial palsy, and MR venography showed segmental occlusion of the ipsilateral transverse sinus. Complete recovery of facial palsy occurred concomitant with recanalization of the transverse sinus. Facial neurography, including transcranial magnetic stimulation of the facial nerve and related motor cortex, ruled out a coincidental idiopathic palsy and revealed conduction block proximal to the facial canal. CONCLUSIONS: Facial palsy in our patient was caused by transient neurapraxia in the intracranial segment of the nerve. We suggest that elevated venous transmural pressure in the nerve's satellite vein, which belongs to the affected drainage territory of the transverse sinus, might have caused venous blood-brain barrier dysfunction in the intrinsic vascular system of the nerve, with leakage of fluids and ions into the endoneurial space and thus an increase in interstitial resistance.     

Neuropsychological manifestations in a case of bilateral thalamic infarction.

Neuropsychological manifestation has been reported with lesions of the anterior and non-specific thalamic nuclei and mammilothalamic tract (MMT). These have been reported in the setting of arterial infarction and/or haemorrhage. Cerebral venous sinus thrombosis (CVT) is a rare cause of brain infarction. It occurs in the setting of oral contraceptive administration or pregnancy. Inherited thrombophilias are documented risk factors. The most frequent being heterozygous factor V Leiden mutation. We report a single case of bilateral thalamic infarction due to cerebral vein and sinus thrombosis. Clinically the case manifested with memory impairment and dysexecutive symptoms. Predisposing factor for venous thrombosis was a homozygous factor V Leiden mutation. The patient was treated with anticoagulation and made a good recovery.     

乙状窦沟及周围骨性结构CT观察的临床意义

目的通过CT测量了解乙状窦的优势引流、位置变异以及周围骨性结构的方法,以探讨桥小脑角和上、中斜坡区病变手术入路的选择。方法对54具成年人颅骨标本进行CT扫描,测量其眶耳线平面,并对比观察乙状窦的优势引流、位置的变异,乳突的气化程度、范围,颈静脉球窝的位置、大小,以及中耳、内耳门的结构。结果54具标本中乙状窦沟右侧大于左侧者45具(83.3%);左侧大于右侧者6具;双侧近似者3具。乙状窦沟前壁至外耳道后壁距离平均为13.5mm。颈静脉球窝于双侧眶耳线平面上的总出现率为37.0%(40/108)。结论头部CT测量可较为完整地了解乙状窦沟及其周围骨性结构,对桥小脑角和上、中斜坡区病变手术入路的选择具有参考价值。当一侧乙状窦前移时,上、中斜坡区中线的病变可考虑于非优势引流侧施行手术,而桥小脑角病变则可选择乙状窦后入路手术。     

脑静脉系统血栓形成的临床特点

目的探讨脑静脉血栓形成(CVT)的临床特点。方法回顾性分析27例CVT患者的临床资料。结果本组患者中25例有发病诱因(包括妊娠或产褥期18例,高热、腹泻5例等),平均年龄(29.7±9.3)岁;多以急性(7例,25.9%)、亚急性(19例,70.4%)起病。以头痛为首发症状23例(91.7%),伴呕吐21例,12例出现意识障碍,均出现不同部位的神经功能缺损表现。腰穿CSF压力增高21例。血常规检查异常22例(81.5%),凝血功能异常13例(48.2%)。影像学检查[CT、MRI、MR静脉血管成像(MRV)]显示,均有不同程度的颅内静脉或静脉窦受累,其中上矢状窦受累最多(77.8%)。24例采用肝素抗凝治疗,2例用溶栓治疗。17例痊愈,4例好转,6例死亡(其中4例并发出血性梗死,2例伴大脑大静脉血栓形成)。结论 CVT继发于循环血液不足及血液高凝状态,以急性或亚急性起病,主要表现为颅内压增高综合征,死亡率较高;MRI和MRA是早期诊断的有效手段,早期肝素抗凝治疗可取得良好疗效。     

硬脑膜静脉窦血栓形成的血管内介入治疗

目的 探讨硬脑膜静脉窦血栓形成(DVST)的血管内介入治疗。方法 11例由CT、MRI、DSA确诊的硬脑膜静脉窦血栓形成患者经皮股动、静脉穿刺给予介入治疗；围手术期规范抗凝、抗血小板聚集等综合治疗。结果 2例患者临床症状戏剧性改善，10例患者临床症状消失或好转，1例无变化；术后影像学均有不同程度的改善；1例机械辅助溶栓患者介入治疗时发生导丝断裂；1例机械辅助溶栓后置人支架未成功；术中、术后无其它相关并发症发生。出院时临床痊愈5例，显效3例，好转2例，无效1例。随访9例患者3～6月无复发。结论 多种介入方法联合治疗DVST的方法是可行的且安全有效。是值得在临床推广的治疗DVST的方法，其远期效果尚需观察。     

Neurological complications in two children with Lemierre syndrome

Lemierre syndrome is a distinct clinical syndrome comprising oropharyngeal sepsis and fever, internal jugular vein thrombosis and remote septic metastases caused by Fusobacterium species. The mortality rate was historically high and although use of antibiotics led to a dramatic fall in incidence, a resurgence has been seen recently. A 14‐year‐old male developed Lemierre syndrome after tonsillitis. There was extensive leptomeningitis, especially over the clivus, causing 6th and 12th cranial nerve palsies, a clinical feature termed the ‘clival syndrome’. He also developed an epidural abscess in the cervical spine, which was unsafe for surgical drainage. Conservative treatment with an extended course of antibiotics and anticoagulation for jugular vein thrombosis led to a good recovery. A 15‐year‐old female developed Lemierre syndrome after a persistent sore throat lasting 7 weeks. She had palsy of the 12th cranial nerve from clival osteomyelitis. She was treated with a 6‐week course of antibiotics and anticoagulants leading to almost full recovery at 3‐month review. Awareness of the potential neurological complications of Lemierre syndrome and prompt management are crucial in reducing morbidity and mortality in this ‘forgotten disease’.     

静脉侧支回流在多发性脑静脉窦血栓病中的作用(附89例分析)

目的 探讨静脉侧支回流在多发性脑静脉窦血栓病程中的作用。方法对我科收治的89例多发性脑静脉窦血栓病人采用经颈动脉溶栓、静脉窦内留置微导管连续溶栓等治疗方法，同时辅以全身抗凝治疗，观察临床效果和侧支循环的变化。结果所有病例治疗后颅内压均明显下降，DSA示残留静脉窦有回流63例，海绵窦代偿性引流75例，椎静脉丛引流35例，头皮静脉代偿引流14例。结论血管内溶栓和抗凝是治疗脑静脉血栓较为可靠和安全的方法：静脉侧支回流的建立和加强对于多发性脑静脉窦血栓的治疗和预后判断意义重大。