A case of cerebral deep venous and venous sinus thromboses with anti-cardiolipin antibody]

We describe a case of cerebral deep venous and venous sinus thromboses with anti-cardiolipin antibody. A 62-year-old male with no previous illness of thrombosis but with alcohol abuse was admitted with acute onset unconsciousness. He recovered two days after with no severe sequela. Laboratory findings suggested the preceding conditions of dehydration and inflammation. X-ray CT of the head revealed symmetrical low density areas in the thalami and basal ganglia, high density signs in the cerebral deep veins, and dilation of the lateral ventricles. MRI on the second hospital day showed abnormal intensities in the thalami and basal ganglia (high signal on T 2-weighed and FLAIR image, low signals on T 1-weighed image, but almost isointensity on diffusion weighed image) and acute to subacute phase thrombus in the superior sagittal sinus. Abnormal intensities observed on MRI disappeared gradually in the following studies. Venous phase images of cerebral angiography performed in chronic phase disclosed occlusion of the superior sagittal sinus and stenosis of the vein of Galen. These radiological findings support the diagnosis of cerebral deep vein and venous sinus thromboses. Hematological examination revealed positive anti-cardiolipin IgG antibody. Several cases of cerebral deep venous thrombosis with anti-cardiolipin antibody have been reported. In our case, dehydration induced by alcohol abuse would have been the trigger of thrombosis, while the existence of anti-cardiolipin antibody might contribute to the risk of thrombosis as an underlying condition.     

Thrombosis of the superior cerebral vein with hemorrhagic cerebral infarction--serial MRI and pathological study of a case

We reported an autopsy case of thrombotic occlusion of the superior cerebral vein with hemorrhagic laminar necrosis of the right parietal cortex. A 68-year-old woman was admitted to our hospital because of a severe headache and left hemiplegia of acute onset. There was a past history of hypertension, fever of unknown origin, leukocytopenia and nasal dermatitis. Magnetic resonance images (MRI) disclosed thrombosis of the superior sagittal sinus and of the right parietal cortical vein as well as right parieto-occipital cerebral infarction. Although she improved with mild sequelae, the subsequent MRI showed a recurrent thrombosis of the superior sagittal sinus. Ten months after the onset she died suddenly, presumably due to acute myocardial infarction. Pathologically, thrombotic occlusion of the right parietal cortical vein, recurrent thrombosis of the superior sagittal sinus and old hemorrhagic cortical laminar necrosis of the right parietal region were revealed. Moreover, intracranial arteritis and phlebitis were observed, as well as arteriolitis in the peripheral nerves. In our case, MRI was useful for the diagnosis and following the course of cerebral venous thrombosis. Cerebral noninfective vasculitis may well have caused the venous thrombosis.     

颅内表皮样囊肿自发破裂并发静脉窦血栓1例及文献复习

目的 探讨颅内表皮样囊肿自发破裂并发静脉窦血栓的临床、影像学表现及诊疗经验.方法 回顾性分析1例颅内表皮样囊肿自发破裂并发静脉窦血栓患者的临床资料,并对相关文献进行复习.结果 患者,男,51岁,因表皮样囊肿破裂后出现头痛症状就医,入院后病情进展迅猛,考虑并发静脉窦血栓.急诊行双侧去骨瓣减压术,术中于中线钻孔并切开上矢状...     

Superior sagittal sinus thrombosis as a rare complication of spontaneous intracranial hypotension syndrome: a case report and review of the literature

In addition to an orthostatic headache, spontaneous intracranial hypotension syndrome can lead to subdural hematoma and diffusion, subarachnoid hemorrhage, and brain sag. However, cerebral venous sinus thrombosis is rarely reported in patients with spontaneous intracranial hypotension. We present the case of a 35-year-old male who developed an orthostatic headache, nausea, vomiting, and photophobia for 5 days. An enhanced brain magnetic resonance image showed extensive linear pachymeningeal enhancement in the bilateral cerebral hemispheres. Lumbar puncture showed that cerebrospinal fluid pressure was 80 mmH₂O. Subsequent magnetic resonance scans demonstrated subdural effusion of the bilateral frontoparietal lobes, hyperintense T1-weighted images, and T2WI lesions within the superior sagittal sinus in 17?days. The patient was given low molecular weight heparin and adverse events occurred. Head computed tomography showed cerebral external fluid accumulation in the bilateral frontoparietal lobes. Then, digital subtraction angiography was performed at 22?days, which confirmed superior sagittal sinus thrombosis, and the patient recovered fully after therapy. The evolution of the disease and radiological findings support the diagnosis of spontaneous intracranial hypotension with superior sagittal sinus thrombosis. To the best of our knowledge, there are very few case reports describing superior sagittal sinus thrombosis as a complication of spontaneous intracranial hypotension. When spontaneous intracranial hypotension and cerebral venous thrombosis occur together, difficult practical questions arise regarding the treatment of these two conditions.     

Dural Sinus Thrombosis with Nonsymptomatic Persistent Falcine Sinus: A Case Report

A 24-year-old woman was admitted to our hospital after convulsive status epilepticus. A cerebral magnetic resonance venography revealed a persistent fetal falcine sinus. Additionally, the posterior third of the superior sagittal sinus was hypoplastic and the abnormal deep venous drainage was accompanied. These abnormalities had already been detected by magnetic resonance imaging several years ago. In the present scan, we discovered a sinus thrombosis in the hypoplastic superior sagittal sinus. In the cerebral angiography, we observed delayed venous return in the left parieto-occipital lobe and hypothesized that cerebral venous stasis due to the thrombus caused the convulsive status epilepticus. The patient was treated with intravenous administration of heparin along with an antiepileptic drug, and she recovered with no neurological defects. In the present case, the falcine sinus and the anomalous venous return were likely congenital while the status epilepticus was derived from thrombosis in the hypoplastic superior sagittal sinus. Although the falcine sinus functioned as an alternative pathway for the superior sagittal sinus, the hypoplastic superior sagittal sinus itself may also play an important role as a venous drainage channel.     

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.     

An unusual case of superior sagittal sinus thrombosis accompanied with dural AV fistula]

Benign intracranial hypertension or pseudotumor cerebri is an collective term for a number of diverse syndromes characterized by increased intracranial pressure. Neither intracranial mass nor ventricular dilatation is observed in this disorder. Moreover, the pathogenesis of this syndrome has yet to be determined. We report a case of 36-year-old female diagnosed as benign intracranial hypertension, who has developed superior sagittal sinus thrombosis and dural AV fistula during the follow up period. The patient was pointed out to have papilledema and elevated intracranial pressure six years ago. Although she was examined by both DSA and CT scan, no abnormal intracranial lesions were observed. Consequently, she was diagnosed as the benign intracranial hypertension and had been followed as an out patient. Three years later, lumboperitoneal shunting was performed because of severe headache and visual impairment. Postoperatively, the patient had been well for two years. Recently, occipital headache recurred and she was readmitted to our hospital. MRI studies demonstrated dilated vessels in the right occipital area. Additionally, angiograms revealed not only the superior sagittal sinus thrombosis but also the rich network of dural AV fistula adjacent to the occlusion. According to those results, the superior sagittal sinus was supposed to have the incomplete occlusion or delayed blood flow that were not observed by DSA, MRI and CT scan performed previously. Those occlusive change in the superior sagittal sinus impeded the CSF absorption and elevated the pressure of venous inflow, then the arterio-venous communication has been developed.(ABSTRACT TRUNCATED AT 250 WORDS)     

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。     

Appearance and disappearance of empty delta sign in superior sagittal sinus thrombosis

The diagnostic value of the empty delta sign on post-infusion CT films was investigated in five patients with superior sagittal sinus thrombosis subsequently verified angiographically and/or pathologically. The empty delta sign, which has been considered to be unique and reliable in the diagnosis of cerebral venous sinus occlusion, was observed only on CT films taken one to four weeks after onset, and was not seen in the extremes of the acute or the chronic stage of the illness. These observations may explain why this sign has not been apparent in some reports concerning the CT findings of superior sagittal sinus thrombosis. Recanalization within the thrombus may be the reason why this sign was no longer apparent in the chronic stage of the patients with superior sagittal sinus thrombosis.     

Diabetic ketoacidosis presenting as a cerebral venous sinus thrombosis

Cerebral venous sinus thrombosis is an uncommon condition with a variable clinical presentation, often resulting in a delayed diagnosis. The most common risk factors are pregnancy and puerperium, oral contraceptive use, head injury, dehydration, blood dyscrasias, malignancies, and systemic diseases. We present a nineteen-year-old female in whom a superior sagittal sinus thrombosis was caused by dehydration during diabetic ketoacidosis and led to the diagnosis of new-onset type 1 diabetes mellitus. To our knowledge this is the first published report of a cerebral venous sinus thrombosis in association with diabetic ketoacidosis in an adult.     

Cerebral venous thrombosis associated with epoetin alfa therapy

BACKGROUND: Cerebral venous thrombosis is a rare complication of polycythemia. To our knowledge, epoetin alfa-induced polycythemia has not previously been reported in association with cerebral venous thrombosis. CASE DESCRIPTION: A 37-year-old patient who was receiving peritoneal dialysis and epoetin alfa (Epogen) therapy presented with a several-day history of worsening headache, and a neuroimaging scan demonstrated thrombosis of the sagittal and transverse sinus. Epoetin alfa therapy, which had been initiated 3 months earlier according to an institutional protocol, was associated with a problematic increase in hematocrit values. CONCLUSIONS: Headache should raise the suspicion of cerebral venous thrombosis in patients who are being treated with epoetin alfa, particularly in the presence of elevated hematocrit values. Monitoring hematocrit parameters in accordance with "standard guidelines" is recommended.     

Superior sagittal sinus thrombosis associated with Evans' syndrome of haemolytic anaemia

Summary A case of superior sagittal sinus thrombosis associated with Evans' syndrome of immune haemolytic anaemia is reported. The neurological symptoms and signs were headaches, right quadrant hemianopia, dyslexia without agraphia, motor aphasia, numbness in and weakness of the right upper extremity, papilloedema and coma. The cerebral venous sinus thrombosis, involving cerebral veins, superior sagittal sinus and straight sinus, was diagnosed by cerebral angiography. It is noteworthy that the superior sagittal sinus thrombosis occurred during a haematological recovery period, with rapid responses to treatment with 6-mercaptopurine and high doses of adrenocorticosteroids. Following a reduction in the doses of these drugs, the symptoms and signs related to the superior sagittal sinus thrombosis gradually subsided, and the haematological pictures remained in remission.     

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.     

Reversible cerebral vasoconstriction syndrome: a rare entity in children presenting with thunderclap headache

Reversible cerebral vasoconstriction syndrome is characterized by a reversible segmental and multifocal vasoconstriction of cerebral arteries, and severe headaches with or without focal neurologic deficits or seizures. A 15-year-old boy presented with thunderclap headache. He had severe hypertension, although his neurologic examination was normal. Initial workup for thunderclap headache to exclude subarachnoid or intracranial hemorrhage, meningitis, pituitary apoplexy, or venous sinus thrombosis was negative. Brain magnetic resonance angiography and cerebral angiography demonstrated bilateral anterior and posterior circulation diffuse, multifocal, vascular irregularities (beading and stenosis) suggestive of underlying vasculopathy or vasculitis. He was started on verapamil. There was complete reversal of the vascular abnormalities in 6 weeks evident by magnetic resonance angiography, with resolution of headache and normalization of blood pressure. Reversible cerebral vasoconstriction syndrome has been rarely reported in children. This case report highlights the diagnostic dilemma and management of the rare childhood presentation of this condition.     

Protein S deficiency, epileptic seizures, sagittal sinus thrombosis and hemorrhagic infarction after ingestion of dimenhydrinate

Congenital protein S deficiency is associated with an increased risk of venous thrombosis. A14-year-old boy presented with epileptic seizures and thrombosis of the superior sagittal sinus and frontal hemorrhagic venous infarction after ingestion of 50 mg of dimenhydrinate, an antiemetic drug. The patient was found to be heterozygous for the factor V Leiden mutation and had a functional protein S deficiency. He recovered completely within a month after conservative treatment. Dimenhydrinate may have disrupted a subclinical pre-existing condition in this case.     

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。     

颅内静脉系统血栓形成的临床和影像学分析

目的探讨颅内静脉系统血栓形成的临床和影像学表现以及治疗和预后,为该病的临床诊断和治疗提供依据。方法选取我院就诊的颅内静脉和静脉窦血栓形成患者71例,收集人口学资料、临床症状和体征、影像学表现、生化等实验室检查、病因,以及治疗方案和预后,并进行统计学分析。结果 71例患者中,常见的临床表现为颅高压症状(头痛、恶心、呕吐)、癫痫、视物模糊或视力下降等。D-二聚体升高67例(94.4%)。横窦血栓形成34例(47.9%),乙状窦血栓形成25例(35.2%),上矢状窦血栓形成44例(62.0%),深部脑静脉血栓形成(直窦或Galen静脉)9例(12.7%),同时累及2个或以上静脉窦者52例(73.2%)。结论 CVST的临床表现缺乏特异性,临床上对于头部CT未见明显异常的不明原因的头痛患者、产褥期或口服避孕药或合并自身免疫性疾病的女性头痛患者,需考虑CVST可能性,并积极行影像学检查,早期识别,早期诊断。     

脑静脉窦血栓形成并发癫痫的常见临床表现及其发病的预测因素

目的调查脑静脉窦血栓形成(CVST)后癫痫的发病率、临床表现形式和预测因素。方法2010年1月到2015年1月本院神经科住院CVST的患者90例。按有无继发癫痫,将90例CVST患者分为两组,通过病例报道表收集患者的人口学信息,症状和体征,以及辅助检查结果脑电图(EEG)、磁共振(MRI)和全脑血管造影(DSA)和诊断治疗方法,对是否并发癫痫进行单因素分析和多因素分析。应用受试者工作曲线(ROC)的曲线下面积(AUC)评价多因素模型,并和CVST预后量表的预测价值相比较。结果 CVST继发癫痫的发病率为34.4%,单因素分析显示,孕妇或产褥期(χ~2=5.517,P=0.019)、视物模糊(χ~2=6.103,P=0.013)、意识障碍(χ~2=32.505,P0.001)、脑出血(χ~2=4.822,P=0.028)、上矢状窦血栓形成(χ~2=5.908,P=0.015),以及横窦血栓形成(χ~2=6.669,P=0.010),在是否并发癫痫两组中比较差异有统计学意义。多因素分析显示,孕妇或产褥期(OR=3.64,95%CI 1.65~8.53)、脑出血(OR=3.02,95%CI 1.43~6.18),以及上矢状窦血栓形成(OR=1.98,95%CI 1.23~4.36)是CVST继发癫痫的独立危险因素。本研究预测模型的预测准确性AUC为0.73(95%CI 0.63~0.81,P0.05),CVST预后量表AUC为0.78(95%CI 0.65~0.86,P0.05),两模型的预测价值差异无统计学意义(P0.05)。结论癫痫是CVST患者的常见临床表现,孕妇或产褥期,脑出血和上矢状窦血栓形成是CVST并发癫痫的独立危险因素。本研究预测模型和CVST预后量表能较好的预测是否继发癫痫。     

Cerebral venous sinus thrombosis in a patient with ulcerative colitis

We report a 30-year-old man who developed superior sagittal sinus thrombosis during the course of ulcerative colitis. Cerebral venous thrombosis that concurrently develops with ulcerative colitis is extremely rare. Only 31 such patients have been reported. Hematologic examination showed abnormalities in the coagulation system in 47% of these patients. Steroids were administered to 69% of these patients. Outcomes were poor: 50% of these patients had neurologic sequelae or died. The risk of cerebral venous thrombosis should always be considered during the course of ulcerative colitis.     

Cerebral Venous Sinus Thrombosis Score and its Correlation with Clinical and MRI Findings

Background: It is plausible that extent of cerebral venous sinus thrombosis (CVST) may determine clinical severity, magnetic resonance imaging (MRI) lesion, and outcome, therefore this study was undertaken. Methods: A total of 160 CVST patients were included and their clinical details, risk factors, Glasgow Coma Scale score, and parenchymal lesion on MRI were noted. The extent of venous sinus thrombosis on magnetic resonance venography was assessed by using CVST score which was computed giving 1 point for each thrombosed sinus and 3 points to superior sagittal sinus (SSS). Death and outcome at 6 months were assessed using modified Rankin Scale (mRS) as good (≤2) and poor (mRS 3-5). Results: Their median age was 29.5 years, and 76 (47%) were females. The median CVST score was 3 (range 1-9). CVST score did not correlate with clinical severity and risk factors. Insignificantly higher proportion of patients had parenchymal lesion with a CVST score of more than 2 (76.5% versus 64.2%). Superficial venous system thrombosis, however, correlated with seizure, papilloedema, and frontal lobe lesion. Frontal, temporal lesion correlated with SSS thrombosis, frontal and temporal with transverse sinus, temporal and cerebellar involvement in sigmoid, and basal ganglia and thalamus in straight sinus thrombosis. Seventeen patients (11%) died, and at 6 months, 132 (82%) had good and 11 (7%) poor recovery. Death and 6 months outcomes were not related to CVST score. Conclusions: Extent of CVST does not determine clinical severity, MRI lesion, and outcome. The location of parenchymal lesion however is related to thrombosis of draining sinus.