Vernet's Syndrome Associated with Internal Jugular Vein Thrombosis

Our objective is to present a case of Vernet's syndrome (cranial nerve (CN) IX, X, and XI palsy) associated with cerebral venous thrombosis (CVT) in an internal jugular vein. The patient presented with acutely developed dysphagia. The weakness of the left sternocleidomastoid and trapezius muscles was observed. The initial magnetic resonance imaging and computed tomography (CT) with contrast enhancement showed contrast-filling defect in the left internal jugular vein inside the jugular foramen. The magnetic resonance venography with contrast enhancement revealed a partial filling defect in the left sigmoid sinus and total occlusion of the left internal jugular vein. Under the diagnosis of CVT associated with CN IX, X palsy, anticoagulation therapy with low-molecular-weighted heparin was initiated. Despite the continued anticoagulation therapy for 3 months, neither the burden of thrombosis in the left sigmoid sinus and internal jugular vein on neck CT nor dysphagia symptoms improved. Clinicians need to be aware of internal jugular venous thrombosis as one of the differential diagnoses in Vernet's syndrome in patients in a hypercoagulable state. Further reporting of similar cases is needed to confirm the association between CVT and Vernet's syndrome.     

Case of transient cortical blindness due to thrombosis of the transverse sinus]

An 62-year-old man presented visual impairment and generalized seizure. Brain CT performed on the day of admission showed thrombus in the right transverse sinus, and DWI showed high intensity areas in the bilateral occipital and parietal lobes. According to bilateral occipital lobe lesions, we considered his visual impairment as cortical blindness. He was diagnosed as venous sinus thrombosis and intravenous heparin, edaravone and osmotic diuretics were administered. MR venography performed after starting of intravenous treatment showed flow gap in the left transverse sinus but no abnormalities in the right transverse sinus. On the second day of hospitalization, his cortical blindness showed improvement and thrombus in the right transverse sinus were disappeared. This indicated that his left transverse sinus originally hypoplastic, thrombus and hemostatis in the right transverse sinus (his dominant side) caused his cortical blindness and generalized seizure. There was a recanalization in the right transverse sinus after heparin therapy.     

Alterations in the intracranial venous sinuses in spontaneous nontraumatic chronic subdural hematomas

Occlusion of the cerebral venous system is frequently associated with intraparenchymal or subarachnoid hemorrhage. There are few reports of cerebral venous thrombosis associated with nontraumatic chronic subdural hematoma (CSH). We aimed to evaluate the intracranial venous system in patients with spontaneous nontraumatic CSH and to identify alterations in their venous sinuses. In this study, eight patients with spontaneous, nontraumatic CSH were followed for a 7-year period (mean: 2.2 years, range: 1–7 years). Neuroradiological images were taken at the onset of symptoms and during follow-up. Venous angiography or magnetic resonance venography was used to evaluate the cerebral venous system of patients. In all patients, medical or surgical treatments were based on clinical symptoms. In seven of the eight patients, the CSH was in the left hemisphere, whereas one patient had bilateral CSH. In one of the eight patients, thrombosis of the transverse sinus and sigmoid sinus was found, but recanalization was observed at follow-up. In a further three patients, the superior sagittal sinus, the straight sinus, and the vein of Galen were markedly reduced in diameter compared to normal or were not visible at presentation, but normal flow was observed at follow-up. In the remaining four patients, the transverse sinus was not observed at symptom onset or during long-term follow-up. A nontraumatic CSH can occur secondary to a venous thrombosis or it can be associated with the inability to image some of the venous sinuses. Venous flow was restored at long-term follow-up. Increased intravenous pressure is thought to be the pathogenic factor that causes a nontraumatic CSH.     

Alexia without agraphia following cerebral venous thrombosis associated with protein C and protein S deficiency

A 26-year-old right handed female was admitted to hospital with right homonymous hemianopia associated with alexia without agraphia. Her cranial magnetic resonance imaging and magnetic resonance angiography revealed a left occipital venous infarction due to thrombosis of the left transverse, sigmoid sinuses and the left internal jugulary vein. The underlying conditions were protein C and protein S deficiency associated with the use of oral contraceptives. To our knowledge, alexia without agraphia has never been described due to a venous infarction associated with hereditary thrombophilia in the literature.     

Alexia without agraphia following cerebral venous thrombosis associated with protein C and protein S deficiency

A 26-year-old right handed female was admitted to hospital with right homonymous hemianopia associated with alexia without agraphia. Her cranial magnetic resonance imaging and magnetic resonance angiography revealed a left occipital venous infarction due to thrombosis of the left transverse, sigmoid sinuses and the left internal jugulary vein. The underlying conditions were protein C and protein S deficiency associated with the use of oral contraceptives. To our knowledge, alexia without agraphia has never been described due to a venous infarction associated with hereditary thrombophilia in the literature.     

Imaging characteristics of two patients with isolated cortical venous thrombosis☆

Over the past twenty years, improvements in neuroimaging have greatly improved the ability to diagnose cerebral venous sinus thrombosis, as well as isolated cortical venous thrombosis. Neuroimaging allows for variations to be detected in the cortical vein and venous sinus. Diagnosis of thromboses in the venous system should not depend entirely on angiography of undeveloped veins or venous sinus. Currently, the combination of magnetic resonance imaging and magnetic resonance venography is the gold standard for diagnosing cerebral venous sinus thrombosis, rather than digital subtraction angiography. This article summarizes clinical manifestations, results from computed tomography and magnetic resonance imaging in two cases of isolated cortical venous thrombosis, analyzed relevant literature, and discussed the clinical and imaging characteristics of isolated cortical venous thromboses.     

Imaging characteristics of two patients with isolated cortical venous thrombosis

BACKGROUND:Over the past twenty years, improvements in neuroimaging have greatly improved the ability to diagnose cerebral venous sinus thrombosis, as well as isolated cortical venous thrombosis. Neuroimaging allows for variations to be detected in the cortical vein and venous sinus. Diagnosis of thromboses in the venous system should not depend entirely on angiography of undeveloped veins or venous sinus. Currently, the combination of magnetic resonance imaging and magnetic resonance venography is the gold standard for diagnosing cerebral venous sinus thrombosis, rather than digital subtraction angiography. This article summarizes clinical manifestations, results from computed tomography and magnetic resonance imaging in two cases of isolated cortical venous thrombosis, analyzed relevant literature, and discussed the clinical and imaging characteristics of isolated cortical venous thromboses.     

Idiopathic intracranial hypertension in a pediatric population: a retrospective analysis of the initial imaging evaluation

There are few studies in the pediatric population regarding the use of magnetic resonance venography to rule out cerebral venous sinus thrombosis in patients presenting with signs and symptoms of idiopathic intracranial hypertension. The purpose of this study was to compare the clinical characteristics of children with presumed idiopathic intracranial hypertension who did and did not undergo venography during their evaluation at a single children's hospital. The authors found that 45 of 68 (66%) patients underwent magnetic resonance venography. Five of 45 (11%) venography studies revealed a thrombosis. There were no significant clinical differences within patients based on the completion of venography or the presence of thrombosis. Larger studies are needed to identify risk factors for thrombosis in children with presumed idiopathic intracranial hypertension, as well as factors influencing physician decision making in the use of magnetic resonance venography in this evaluation.     

T2*-Weighted MRI Detected Dilated Cerebral Veins in a Patient with Acute-Phase Cerebral Venous Sinus Thrombosis—A Case Report

We describe a 45-year-old man who presented with nausea, vomiting, and strong occipital headache on the right side. Although no abnormalities on neurological examination or computed tomography imaging were found on admission, peripheral blood cell counts showed polycythemia (hemoglobin 20.6 g/dL) and electrocardiography demonstrated atrial fibrillation. Therefore, anticoagulant treatment with heparin was started immediately. On the following day, the occipital headache continued. Brain T2*-weighted (T2*WI) magnetic resonance imaging (MRI) and, to a lesser extent, susceptibility-weighted imaging showed dilation of numerous cortical veins, suggesting the possibility of cerebral venous thrombosis (CVT). MR venography (MRV) showed a deficit of the right transverse sinus. Contrast-enhanced MRI revealed partial defects of the right transverse sinus, and led to the definite diagnosis of CVT, and the anticoagulation therapy was continued. On day 7 the headache disappeared, and MRV on day 16 showed the recanalization of the right transverse sinus. There were no complications subsequent to the CVT. On day 25, the patient was discharged with no after-effect. We speculate that the dilation of cortical veins on T2*WI is a helpful sign in detecting acute-phase CVT.     

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.     

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.     

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.     

Cerebral venous thrombosis in a gentleman presenting with fever, convulsion and frontotemporal haemorrhages.

Cerebral venous thrombosis (CVT) is an uncommon but serious type of stroke. Thrombosis may involve the cortical or deep veins or the venous sinuses. The presenting clinical features are non-specific. We report a 48-year-old man with CVT who presented with fever, bitemporal throbbing headache, and generalised convulsion. Computed tomography (CT) of the brain revealed acute haemorrhages over right anterior frontal and posterior temporal regions with surrounding oedema and right anterior temporal subcortical oedema. The initial diagnosis was herpes simplex encephalitis. Absence of venous flow over the right transverse and sigmoid sinuses during the venous phase of digital subtraction angiography (DSA) revealed CVT. He was anti-coagulated for 6 months. An underlying cause of CVT was not detected. A high index of suspicion is required when risk factors of CVT are present. CT brain may be normal or showing non-specific findings. Magnetic resonance imaging plus venography, CT venography, or DSA is diagnostic.     

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。     

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.     

Dural sinus thrombosis following head injury: report of two cases and review of the literature

Dural sinus thrombosis (DST) usually involves the sagittal, transverse and sigmoid sinuses and is more common in women due to pregnancy, puerperium and oral contraceptive use. Other etiologies include coagulopathies, infection and head injury. We have present two DST cases following head injury. The first case was a 35-year-old man hospitalized because of one-week history of headache and repeated vomiting after a mild head injury. Thrombosis of the superior sagittal sinus, right transverse and sigmoid sinuses and right jugular vein was determined on angiography. The second case was a 25- year-old man operated on for epidural hematoma at the posterior fossa. Meningitis developed and an abducens palsy was determined. Magnetic resonance imaging demonstrated thrombosis of the right transverse and sigmoid sinus. Low molecular weight heparin was administrated for three months. Both cases had good recovery, but one had recanalisation of the thrombosis. Intracranial hematomas, depressed skull fracture or skull fracture that cross the sinus can obstruct the blood flow in the sinus. Moreover, closed head injury may cause to DST. Because of undefinitive pathophysiology, a consensus was not obtained on overall strategy concerning conservative, radiosurgical, or surgical therapy yet.     

Recanalisation of cerebral venous thrombosis

OBJECTIVE: To investigate recanalisation in the first 12 months after cerebral venous thrombosis. METHODS: 33 consecutive patients presenting with cerebral venous thrombosis were enrolled in the study. Diagnosis was made by magnetic resonance imaging (MRI) and magnetic resonance venography (MRV) or catheter angiography. Patients were initially treated with intravenous heparin. Warfarin was given for at least four months. Cerebral MRI and MRV were done at four months and repeated after 12 months if venous thrombosis persisted. Outcome was evaluated by the Rankin scale at 12 months. RESULTS: Outcome at 12 months was good, with a median modified Rankin scale score of 0 (range 0 to 2); 27 patients (82%) had no residual deficits. No patient suffered recurrent cerebral venous thrombosis, deep vein thrombosis, or pulmonary embolism during follow up. After four months, all deep cerebral veins and cavernous sinuses, 94% of superior sagittal sinuses, 80% of straight sinuses, 73% of jugular veins, 58% of transverse sinuses, and 41% of sigmoid sinuses had recanalised. No further recanalisation was observed thereafter. CONCLUSIONS: The results suggest that recanalisation only occurs within the first four months following cerebral venous thrombosis and not thereafter, irrespective of oral anticoagulation.     

儿童脑静脉窦血栓12例临床特征分析

【摘要】
目的 探讨儿童脑静脉窦血栓形成（cerebral venous sinus thrombosis,CVST）发病的危险因素、病因、临床表现、影像学表现及诊治方法。
方法 对2003年1月至2011年6月在北京天坛医院儿科及神经内科就诊的12例儿童脑静脉窦血栓进行回顾性分析。
结果 所有患儿均表现出颅高压症状。鼻窦炎（7例,58.3%）可能为儿童CVST主要发病病因。颅脑磁共振成像（magnetic resonance imaging,MRI）和磁共振静脉成像（magnetic resonance venography,MRV）显示最常见的受累静脉窦为乙状窦和横窦（各11例,占97.1%）。经降颅压、抗凝及对症支持治疗,8例痊愈,4例遗留神经功能缺损。
结论 CVST患儿常以颅高压症状就诊,感染为儿童CVST的最主要危险因素,神经影像学检查能为确诊提供依据,及时治疗可改善预后。     

Isolated Straight Sinus Thrombosis: Clinical and Neuroradiologic Correlates

A rare case of straight sinus thrombosis in a 36-year-old female with sudden onset of status migrainosus is presented. This condition was demonstrated by a linear density in the midline on a noncontrast computed tomography scan, as a filling defect at the location of straight sinus on magnetic resonance imaging, and by the inability to visualize blood flow in the straight sinus on magnetic resonance venography, w ith a similar demonstration on a 4-vessel digital subtraction angiography.     

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.