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.     

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.     

Endoscopic sphenoid sinus drainage in Lemierre syndrome

Lemierre syndrome is a rare condition arising from an invasive oropharyngeal infection, which leads to septic thrombophlebitis of the internal jugular vein and multi-organ septic embolization. Intracranial complications are rare but serious, including subdural empyema, cavernous sinus thrombosis, and internal carotid artery aneurysms. We report a patient with Lemierre syndrome with multiple intracranial complications despite aggressive antimicrobial therapy. The patient eventually required transsphenoidal endoscopic drainage of the sphenoid sinus to help eradicate the infectious source. We postulate that in patients with Lemierre syndrome with evidence of infection in the paranasal sinuses, endoscopic sinus drainage can be an adjunct to antimicrobial therapy in achieving infection control.     

Emergency endovascular treatment of cerebral sinus thrombosis with a rheolytic catheter device.

Severe thrombosis of the superior sagittal, transverse, and straight sinuses developed in a 53-year-old woman. This resulted in extensive multifocal hemorrhagic venous infarction and severe intracranial hypertension refractory to intensive management. Endovascular therapy using a rheolytic catheter device in combination with a small amount of fibrinolytic agent led to rapid normalization of the intracranial pressure, allowing optimization of the cerebral perfusion pressures and was followed by steady, albeit protracted, clinical improvement. The patient not only survived but also left the hospital with minimal neurologic deficit. The rheolytic catheter endovascular treatment is, in the opinion of the authors, the treatment of choice for patients with life-threatening cerebral sinus thrombosis.     

兔颈静脉血栓形成对颅内压的影响

目的 研究颈静脉血栓形成对颅内压的影响.方法 40只日本大耳兔随机分为左侧颈静脉血栓(LJVT)组、右侧颈静脉血栓(RJVT)组、双侧颈静脉血栓(BJVT)组、假手术(Sham)组,通过阻断相应血管血流并注入凝血酶建立血栓模型.通过颈部彩超测量造模前后各组动物颈静脉直径,并监测72 h内颅内压变化.造模后1周观察脑组织形态学改变.结果 与造模前比较,LJVT和RJVT造模后颅内压即升高(P<0.05),12h后降至造模前水平(P>0.05);BJVT造模后颅内压显著升高(P<0.01),72h仍处于较高水平(P<0.01); Sham组造模前后颅内压无明显差异(P> 0.05).结论 颈静脉血栓形成可引起颅内静脉回流受阻,进而导致颅内压升高.一侧颈静脉血栓可导致短暂颅内压升高,双侧颈静脉血栓颅内压升高严重而持久.     

Unusual Intracranial Parasagittal Meningioma Extending into the Internal Jugular Vein through the Sinuses

Meningiomas frequently invade cerebral venus sinuses, especially parasagittal meningioma to superior sagittal sinus. However, most invasions do not reach internal jugular vein. We present a case of parasagittal meningioma extending into the internal jugular vein through the sinuses. Radiological investigation revealed that the tumor was invading the sagittal, transverse, sigmoid sinus and junction of the internal jugular vein to subclavian vein, which was filled with tumor. The histopathological examinations revealed that both the cerebral tumor and mass in the internal jugular vein contributed to the transitional meningioma. This is a rare case of a meningioma extending into the internal jugular vein through the sinuses. According to this case, the frontal parasagittal meningioma could invade directly the internal jugular vein. The significance of this association to cerebral venus sinuses and internal jugular vein are discussed.     

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.     

Major cerebral arterial and venous disease in osteopetrosis

Two patients with osteopetrosis were studied in whom severe stenosis of one or both internal carotid arteries was demonstrated. One patient had autosomal dominant osteopetrosis and the other patient had the autosomal recessive form of the disease. In one patient, probable occlusion of one internal jugular vein and retrograde thrombosis of the contributing dural venous sinuses was present. Venous drainage of parts of the brain occurred through dilated emissary and scalp veins. It appears that major extracranial vessels may be impinged upon by dysplastic bone in osteopetrosis, although this is the first report of such an occurrence. A posterior fossa aneurysm was present in one case, and may have been related to abnormal intracranial hemodynamics.     

Bithalamic infarcts: embolism of the top of basilar artery or deep cerebral venous thrombosis?

Bithalamic infarcts are usually attributed to thromboembolism of the top of the basilar artery. However, in some cases, deep cerebral venous thrombosis and thrombosis of cerebral venous sinuses was proved to be the cause. The case of a 47-year-old female with ischemic thalamic and mesencephalic lesions is reported, that was attributed to thrombosis of internal cerebral veins. In cases of bithalamic infarcts, apart from the top of the basilar artery syndrome, deep cerebral venous thrombosis should be taken into consideration. Neuroimaging findings such as generalized cerebral edema, multiple infarcts or hemorrhages, hyperdense appearance of cerebral sinuses or veins and filling defects in the cerebral venous sinuses in contrast-CCT, can lead to the proper diagnosis.     

Anomalous venous drainage in a case of non-syndromic craniosynostosis

The authors describe the clinical and radiological findings in a case of non-syndromic craniosynostosis affecting multiple sutures, in which the intracranial venous drainage was grossly anomalous. Investigation by magnetic resonance imaging and angiography revealed that almost all of the intracranial venous blood was draining from the dural sinuses transosseoussly via enlarged emissary veins to the external jugular veins and the vertebral veins. Although present, both internal jugular veins were small. This discovery represented a contraindication for the vault expansion surgery that had been recommended as treatment for the patient's raised intracranial pressure, and it has important implications for the management of all types of craniosynostosis. Received: 2 October 1995     

Spontaneous intracranial hypotension followed by dural sinus thrombosis: A case report

SIH is an underdiagnosed condition with variable clinical and radiological presentations that leads to engorged dural sinuses and promotes stasis predisposing for dural sinus thrombosis (DST). We describe a young female patient who presented with clinical and imaging signs of SIH alone followed by a superimposed DST 10 days later. We discuss the pathophysiological mechanisms that may have led to this constellation, and review the literature to-date along with diagnostic and management implications.     

Endovascular intervention for repositioning the distal catheter of ventriculo-atrial shunt

Displacement of distal ventriculo-atrial (VA) shunt is not uncommon. However, misplacement of the distal catheter of VA shunt in the internal jugular vein is a possibility, especially when conducted without intraoperative monitoring. We describe a patient in whom a VA shunt was performed due to failure of ventriculo-peritoneal shunt and the distal catheter of the shunt was found to be misplaced in the left internal jugular vein. Endovascular intervention via femoral vein was used to retrieve the distal catheter.     

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。     

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.     

A case with cerebral thrombosis receiving tamoxifen treatment.

Cerebral sinus thrombosis (CST) is known to be related to a number of underlying aetiologies including otitis media, trauma, pregnancy, birth control pills, tumours, malnutrition, dehydration, haematologic disorders and malignancy (Fishman, 2000; Raizer and Abbott, 2000). We present the case of a patient with breast cancer receiving the antioestrogen drug tamoxifen who developed CST. A 40-year-old female presented as an emergency with a 10-day history of headache and left sided weakness. On questioning her past medical history included a diagnosis of breast cancer 3 years ago treated by radical mastectomy and tamoxifen 20 mg daily. At the time of admission, neurologic examination revealed a mild left sided hemiparesis and a present Babinksi sign. Non-contrast enhanced tomography was normal. Magnetic resonance imaging (MRI) showed thrombosis in the superior sagittal sinus, right lateral sinus and jugular vein in addition venous infarction in the right temporal lobe was present (Figs 1a and b). Routine haematology and biochemistry was normal. Anticoagulation tests, antithrombin III, protein S and C levels were also found to be normal. She was treated with anticoagulation therapy and her hemiparesis improved within 3 days. Control MRI showed the resorption of the venous infarction and resolution of the thrombosis (Fig. 1c).     

Importance of Jugular Valve Incompetence in Contrast Transcranial Doppler Ultrasonography for the Diagnosis of Patent Foramen Ovale

Transcranial Doppler (TCD) ultrasound with the intravenous injection of agitated saline as contrast (cTCD) is an effective method for detecting right-to-left intracardiac and extracardiac shunt (RLS); however, the sensitivity of cTCD in the diagnosis of RLS remains slightly less than that of transesophageal echocardiography, even in patients with adequate transtemporal ultrasonic bone windows. The authors present a case with cTCD underestimating RLS because of jugular valve incompetence in a 42-year-old man presenting with an episode of transient aphasia. Three weeks after transcatheter closure of a patent foramen ovale associated with an atrial septal aneurysm, he experienced 2 episodes of amaurosis fugax. Following a negative 45-minute embolus detection study with power M-mode TCD, the patient underwent a cTCD study with monitoring of the left middle cerebral artery (MCA), the anterior cerebral artery, and the submandibular extracranial internal carotid artery. A single microbubble (MB) was detected in the left MCA in only 1 of 5 studies; the remaining runs all failed to detect an RLS. Significant MB reflux was noted in the left internal jugular vein because of jugular valve incompetence. The authors conclude that incompetence of the jugular vein valve can result in a false negative cTCD study for RLS detection.     

以头痛为唯一临床表现的脑静脉窦血栓形成患者的头痛特征

目的 探讨以头痛为唯一临床表现的脑静脉窦血栓形成（cerebral venous thrombosis,CVT）患者的头 痛特征。 方法 回顾性纳入2012年1月-2018年12月北京航天总医院神经内科连续收治的以头痛为唯一临床 表现的CVT患者。以头痛为唯一表现指除头痛外无提示颅内压升高的体征,且脑脊液压力正常,影像 或脑脊液检测显示无蛛网膜下腔出血。分析这类患者的头痛特征（部位、发作模式、演变和强度）。 结果 共8例患者纳入本研究,中位年龄40岁,女性6例（75%）。最常受累的静脉窦是侧窦（7例, 87.5%）。4例患者累及左侧侧窦,头痛均位于左侧;4例患者累及中线静脉窦（上矢状窦或直窦）,头痛 均为双侧性。头痛发作模式：进展性头痛7例,闪电样头痛1例;演变：持续性7例,间歇性1例;强度：严 重头痛7例,中等程度头痛1例。 结论 对于仅表现为头痛并且颅内压正常以及无蛛网膜下腔出血的CVT患者,侧窦受累很常见,尤 其是左侧。头痛部位与受累静脉窦位置一致。     

Cerebral sinovenous thrombosis in children: another reason to treat iron deficiency anemia

Iron deficiency anemia is a rare cause of cerebral sinovenous thrombosis in children. We report three cases of cerebral sinovenous thrombosis and iron deficiency anemia treated at Primary Children's Medical Center in Salt Lake City, Utah, between 1998 and 2001. The children were 9, 19, and 27 months old at the time of admission. Hemoglobin levels ranged from 6.6 to 7.0 g/dL, mean corpuscular volume levels from 45 to 56 fL, and platelet counts from 248,000 to 586,000/microL. Magnetic resonance imaging and magnetic resonance venography revealed thrombosis of the straight sinus and internal cerebral veins in all three children, with the addition of the vein of Galen, left transverse and sigmoid sinuses, and upper left internal jugular vein in one child. Recovery ranged from excellent to poor in 3 months to 3 years of follow-up. Four additional cases, ages 6 to 22 months, were found in the English-language literature. Evaluation for prothrombotic disorders was negative in all children, including the current cases. Treatments have included thrombectomy, corticosteroids, mannitol, heparin, low-molecular-weight heparin, warfarin, aspirin, blood transfusion, and iron supplementation, but there is no consensus regarding therapy, other than to correct the anemia and treat iron deficiency. Iron deficiency anemia, a preventable cause of cerebral sinovenous thrombosis, deserves consideration when cerebral sinovenous thrombosis is detected in young children.     

Venous Infarction of Brainstem and Cerebellum

The authors describe 2 cases of posterior fosa venous infarction. A 56-year-old woman with essential thrombocytemia presented with fluctuating complaints of headache, nausea, vomiting, left-sided numbness-weakness, and dizziness and became progressively stuporous. Cranial magnetic resonance imaging (MRI) showed bilateral parasagittal fronto-parietal and left cerebellar contrast-enhancing hemorrhagic lesions. On magnetic resonance venography, the left transverse and sigmoid sinuses were occluded. The second patient, a 39-year-old woman, presented with acute onset of diplopia, numbness of the tongue, vertigo, and right-sided weakness following a gestational age stillbirth. MRI revealed lesions in the right half of midbrain and pons and in the superior part of the right cerebellar hemisphere. Digital subtraction angiography showed right transverse and sigmoid sinus occlusion. The authors suggest that one should investigate the possibility of venous infarction in the presence of posterior fossa lesions that are often hemorrhagic and are not within any arterial territory distribution but respect a known venous drainage pattern. Recognition of the observed clinical and neuroimaging features can lead to earlier diagnosis and, potentially, more effective management.     

Ischaemic stroke,factor V Leiden heterozygosity and left atrial thrombosis in sinus rhythm: a case report

Abstract We describe a 63-year-old man in sinus rhythm (SR) with an ischaemic stroke involving basal ganglia region on the right side. The patient was known to be heterozygous for factor V Leiden (FVL) mutation. On diagnostic work-up, no arterial sources of embolism were found. Transoesophageal echocardiography evidenced a left atrial (LA) thrombosis without relevant cardiopathies. LA thrombosis is generally associated to atrial fibrillation, atrial enlargement, mitral valve stenosis and left ventricular dysfunction, whereas mitral regurgitation is considered protective. To our knowledge, this is the first report of cardioembolic stroke related to a LA thrombosis in a patient in SR without risk factors for thrombus formation except for FVL heterozygosity.