Cerebral venous thrombosis in systemic lupus erythematosus

We describe six cases of cerebral venous thrombosis in patients with systemic lupus erythematosus. In one patient, cerebral venous thrombosis was the initial manifestation of lupus; in the five others, it occurred 1-33 years after the diagnosis of lupus. The main clinical features of cerebral venous thrombosis were persistent headache in all six patients, focal symptoms in four, and seizures in three; papilledema was present in only one patient. Cerebral venous thrombosis was diagnosed based on angiography or magnetic resonance imaging. Both the transverse (in five patients) and the superior sagittal (in three) sinuses were involved. Extracranial arterial and/or venous thrombosis were present in three patients, abortion in two, thrombocytopenia in four, and lupus anticoagulant in three. The neurologic symptoms resolved rapidly in five patients treated with steroids and heparin. Cerebral venous thrombosis should be suspected in patients with lupus who complain of persistent headache, especially in the presence of neurologic symptoms.     

Expectant management of deep cerebral venous thrombosis with intraventricular hemorrhage

Limited randomized clinical trial data favor the use of anticoagulation in patients with cerebral venous thrombosis. We present a patient with deep cerebral venous thrombosis in whom anticoagulation was withheld because of coexistent intraventricular hemorrhage. She had a benign clinical course without anticoagulation, suggesting that close observation may be a management option in these patients.     

自发性低颅压并发静脉血栓形成和出血性梗死一例临床分析

目的自发性低颅压(SIH)多以与体位相关的剧烈头痛为主要症状,误诊率高。SIH并发静脉和(或)静脉窦血栓形成非常罕见。方法我们报道1例SIH并发静脉血栓形成和出血性梗死的患者,并复习相关文献。结果本例患者自发性低颅压在前,静脉血栓形成和出血性梗死在后,同时经过检查排除了其他可导致静脉血栓形成的危险因素。所以我们考虑SIH是静脉血栓形成的危险因素。假如SIH患者与体位相关的间断的头痛变成持续性头痛时,应考虑到有静脉血栓形成的可能。结论因为SIH的患者有形成硬膜下血肿的风险,所以对于静脉血栓形成的抗凝治疗应谨慎。     

Cerebral venous thrombosis and submandibular lithotripsy: A case report

We present the case of a 38-year-old woman with a diagnosis of cerebral venous thrombosis that developed after lithotripsy of the submandibular gland. Few cases of vessel thrombosis after lithotripsy have been reported in other organs, this is the unique cerebral case reported.     

Cerebral Venous Thrombosis after BNT162b2 mRNA SARS-CoV-2 vaccine

The development of SARS-CoV-2 vaccines has raised several concerns regarding venous thromboembolism, namely cerebral venous thrombosis. Although cerebral venous thrombosis has been reported after administration of a viral vector vaccine, due to a possible auto-immune mechanism inducing thrombocytopenia, the same has not happened in mRNA vaccines. We report two cases of cerebral venous thrombosis, shortly after administration of mRNA vaccine. In both patients, there was no evidence of thrombocytopenia or antiplatelet antibodies, and alternative causes for cerebral venous thrombosis were found. As such, despite the temporal relation of both cases to vaccine administration, these types of cerebral venous thrombosis do not seem to be pathophysiological different from cerebral venous thrombosis not associated to SARS-CoV-2 vaccination. Continuous pharmacovigilance is necessary to monitor possible new events and clarify this association.     

Cerebral vein thrombosis as an exceptional cause of transient ischemic attack

We present a case of transient ischemic attack (TIA) as an extremely rare presentation of cerebral vein thrombosis. A 41-year-old man presented with a headache and episodes of hypoesthesia on his left side lasting approximately 30 minutes. Several vascular risk factors were remarkable in his medical history. The neurologic examination was normal. TIA of arterial origin was excuded by detailed investigations. Magnetic resonance imaging revealed acute thrombosis of the right superficial middle cerebral vein and the vein of Trolard. TIA-like episodes stopped with anticoagulation. Head trauma a few days earlier was the most likely mechanism of venous thrombosis. We suggest that the patients with TIA and headache should be carefully investigated in terms of cerebral venous thrombosis.     

Cerebral venous thrombosis with plasminogen deficiency

We describe a patient with inherited plasminogen deficiency who developed extensive cerebral venous thrombosis. Several other conditions that might have contributed to a hypercoagulable state, including mild thrombocytosis, thyrotoxicosis, and a chronic inflammatory lung disorder, were present. We also discuss the evidence linking plasminogen deficiency with a thrombophilic state. The diagnosis of cerebral venous thrombosis in this case was readily established by nuclear magnetic resonance imaging, a technique that is ideally suited for the evaluation and follow-up of patients with this condition.     

Cerebral venous thrombosis after lumbar puncture and intravenous steroids in two patients with multiple sclerosis

Two patients affected with a multiple sclerosis developed cerebral venous thrombosis after lumbar puncture and treatment with intravenous methylprednisolone. In one case, the course was favorable. The second patient died in spite of intracerebral thrombolysis. The autopsy confirmed the diagnosis of cerebral venous thrombosis and multiple sclerosis. We discuss the relationship between lumbar puncture, steroid treatment and cerebral venous thrombosis.     

Temporal Profile of CT and T2*-Weighted Gradient-Echo MRI in a Patient with Unilateral Thalamostriate Vein Thrombosis

Deep cerebral venous system thrombosis (DCVST) is an uncommon variety of thrombosis that accounts for 11% of cases of cerebral venous thrombosis. Thalamostriate vein (TSV) thrombosis is further rare among patients with DCVST. Although patients with cerebral venous thrombosis commonly have characteristic neurological deficits including headache, deterioration of consciousness, and seizures, patients with DCVST do not necessarily show such symptoms. Therefore, diagnose of DCVST is sometimes difficult. Here we report a case of TSV thrombosis with a unilateral basal ganglion lesion presenting with right-sided hemiparesis. A 61-year-old Japanese female was referred to our hospital. On neurological examination, she had no headache but presented with right facial paresis with dysarthria. Her right hemiparesis was present in the upper and lower extremities. We repeatedly performed brain computed tomography (CT) and T2*-weighted conventional gradient-echo (GRE) magnetic resonance imaging, and conclusively diagnosed as left TSV thrombosis. We firstly report a case of unilateral DCVST associated with TSV thrombosis in which a temporal profile of CT and T2*-weighted GRE images was obtained. Although DCVST is a rare clinical entity, physicians should be aware that repeated radiological observations can be useful for the diagnosis and early medical treatment for DCVST.     

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.     

Postpartum cerebral vein thrombosis

Pregnancy increases the risk of cerebrovascular accidents to approximately 13 times the expected rate. Most of the cerebrovascular accidents are due to cerebral venous thrombosis. Headache, seizures and focal neurological deficits are some of the symptoms of cerebral venous thrombosis. Headache can also be due to postural puncture, pre eclampsia and eclampsia in the postpartum period. Neuroimaging with MRI with contrast helps in the diagnosis of cerebral venous thrombosis and giving a definite diagnosis, which aids in the treatment and prognosis. The cases presented in this report highlight the importance of investigations such as MRI or CT in the diagnosis of cerebral venous thrombosis in pregnancy and puerperium.     

Intracranial venous thrombosis in the first trimester of pregnancy.

We describe a fatal case of intracranial venous thrombosis occurring in early pregnancy. Such thrombosis usually occurs in late pregnancy or the puerperium but rarely during the first trimester of pregnancy. Computerized axial tomography suggested massive cerebral venous infarction. Necropsy findings showed not only cerebral venous thrombosis but also extensive pelvic and iliac vein thromboses. The relationship of cerebral venous thrombosis and pregnancy is discussed and the literature reviewed.     

An unusual cause of cerebral venous sinus thrombosis. Paroxysmal nocturnal hemoglobinuria

Cerebral venous sinus thrombosis caused by paroxysmal nocturnal hemoglobinuria is uncommon. Our case is a 44-year-old woman who presented with a 2 day history of headaches, nausea, and seizures followed by a Todd`s paresis; she had been diagnosed as paroxysmal nocturnal hemoglobinuria for 4 years. A magnetic resonance venography revealed extensive thrombosis of the cerebral venous sinus. She received antithrombotic treatment with a good outcome. We highlight paroxysmal nocturnal hemoglobinuria as the reason for the cerebral venous sinus thrombosis. The treatment of cerebral venous sinus thrombosis caused by paroxysmal nocturnal hemoglobinuria should be individualized.     

外伤性脑静脉窦血栓的临床诊治

目的 探讨外伤性脑静脉窦血栓的诊治方法。方法 对近两年来临床工作中发现的27例外伤性脑静脉窦血栓患者资料进行回顾性分析。所有病例均采用核磁共振成像（MRV）证实，行尿激酶静脉溶栓治疗，首齐20万U，每日增加5万U。最大剂量达100万U。结果 19例再通，7例建立侧支循环，1例死亡。结论 对临床工作中出现的腰穿颅内压大于300mm H20的病例、跨窦骨折的病例、病情平稳后又出现反复（如头痛加剧、呕吐等）而复查头颅CT无异常的病例，应高度怀疑脑静脉窦或深静脉血栓形成。MRV成像是脑静脉安或深静脉血栓诊断的首选方法。     

Homocystinuria with transverse sinus thrombosis

A case of cerebral venous thrombosis caused by undiagnosed homocystinuria is reported. The pitfalls regarding the diagnosis of a potentially medically treatable condition are discussed. Cerebral venous thrombosis in children has a variable type of onset and a multiplicity of causes. This type of pathology, although not frequent, is more common than previously thought. Among the different etiologies, undiagnosed homocystinuria is not routinely considered. We report a case of venous thrombosis of the left transverse cerebral sinus in a girl with drug-resistant partial epilepsy and homocystinuria. This diagnosis was considered and confirmed after the appearance of acute cerebral symptoms caused by venous thrombosis.     

Prolonged direct catheter thrombolysis of cerebral venous sinus thrombosis in children: a case series

Cerebral venous sinus thrombosis is a rare condition with potentially devastating neurologic outcome--death and severe disability are common in advanced cases. In adults, protocols for mechanical clot disruption and direct thrombolysis are established; no guidance exists for children. We present our experience of 6 children with cerebral venous sinus thrombosis and ominous clinical progression. We found that effective thrombolysis required substantially longer infusion, more rounds of mechanical disruption, and higher doses of thrombolytics than are commonly practiced. Despite pervasive thrombosis, prethrombolysis hemorrhage, coma, and other predictors of death and disability, our patients survived and 4 of 6 had no functional deficits. One patient had moderate, and one had severe deficits. We report these cases to illustrate that hemorrhage may not be a contraindication to thrombolysis for cerebral venous sinus thrombosis, that prolonged infusion may be required to restore perfusion, and that good neurologic outcomes can be achieved despite dire clinical presentations and extensive sinus thrombosis.     

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。     

低颅压综合征合并颅内静脉血栓2例并文献回顾分析

目的总结分析低颅压综合征(SIH)合并颅内静脉血栓形成(CVT)的临床表现,诊断和治疗方法。方法报道2例低颅压综合征合并颅内静脉血栓形成,并回顾文献报道15例同类病例,分析其临床特征、影像学表现、治疗和病理生理机制。结果17例患者均以亚急性起病,其中14例患者表现为体位性头痛。常见的伴随症状有恶心、呕吐、复视、耳鸣等。所有患者的影像学检查都存在SIH和CVT的表现,头部核磁共振(MRI)加静脉成像(MRV)是诊断颅内静脉血栓形成的主要手段。结论低颅压综合征是颅内静脉血栓形成的危险因素之一,了解两者内在的病理生理联系和临床特征有利于对该病的早期诊断和及时治疗。