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.     

上矢状窦血栓形成早期大脑灰质CT特殊表现(附1例报道及文献复习)

目的分析上矢状窦血栓形成的早期大脑灰质CT特点,提高对上矢状窦血栓形成早期大脑灰质CT特点的认识。方法结合文献对1例上矢状窦血栓形成患者的早期脑CT影像学特点进行分析。结果患者发病后6h行脑CT平扫显示双侧额叶皮质高密度影;第3d时脑MRIT1显示上矢状窦前部血栓形成可疑,脑FLAIR-MRI显示右侧额叶高信号及左侧额叶弧形高信号,脑MRIT1相增强扫描显示双侧额叶皮层弧线型增强,MRV显示上矢状窦前1/3处见一无信号区,周围有侧枝循环形成;治疗第6d时复查脑CT和MRI均无出血征象。结论患者早期脑CT所显示的高密度影可能是皮层静脉和毛细血管血液淤积的显示,并不全是出血,对于该征象的认识并结合病理生理过程给予的合理解释对于及时正确的认识该病具有重要意义。     

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.     

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.     

Chronic superior sagittal sinus thrombosis with phlebosclerotic changes of the subarachnoid and intracerebral veins

An autopsy case of chronic thrombotic occlusion of the superior sagittal sinus (SSS) with an unusually protracted clinical course is reported. The patient was an 84‐year‐old man without any predisposing conditions for thrombosis. The clinical features were atypical and were characterized by recurrent cerebral subcortical hemorrhages. The autopsy revealed a large, organized thrombus in the SSS and extensive hemorrhagic infarction of the bilateral parasagittal region of the cerebral hemispheres. The hemorrhagic infarction was comprised of the aggregation of numerous minute foci of coagulation necrosis associated with petechial hemorrhages. Marked phlebosclerotic changes were observed in the subarachnoid and intracerebral veins, which were considered to be reactive changes of the venous walls against a persistent elevation in peripheral venous pressure caused by thrombosis of the SSS.     

尿激酶联合替罗非斑在颅内静脉窦血栓形成介入性治疗中的应用

目的 探讨尿激酶联合替罗非斑血管内溶栓治疗颅内静脉窦血栓形成(CVST)的疗效和安全性. 方法 广州军区武汉总医院神经外科自2009年1月至2011年1月行尿激酶联合替罗非斑经微导管选择性静脉窦内溶栓、机械性碎栓治疗9例重症CVST患者,分析其临床资料和疗效. 结果 出院前8例患者脑脊液(CSF)压力正常,DSA检查显示7例患者静脉窦主干通畅,皮层静脉和深静脉恢复正常.1例部分再通,皮层静脉部分代偿.术中和术后未发生手术操作相关的并发症;出院时8例患者症状、体征得到改善,其中头痛消失7例,遗留轻度头痛1例.1例死亡. 结论 尿激酶联合替罗非斑经微导管选择性静脉窦内溶栓治疗是CVST安全、有效的治疗手段.     

球囊扩张成型与取栓治疗颅内静脉窦血栓疗效分析

目的探讨球囊扩张成型与取栓治疗颅内静脉窦血栓的疗效和安全性。方法 24例经DSA检查证实颅内静脉窦血栓形成患者,采用颅内静脉窦内球囊扩张成型及取栓手术,使静脉窦即时再通,术后抗凝治疗6个月。结果 24例上矢状窦及横窦血栓患者,微导管与微导丝配合,逆行进至上矢状窦中前1/3处,直径5mm球囊沿微导丝进至该处。充盈球囊,透视监视下回拉球囊,使闭塞的静脉窦再通。临床症状均得到改善。术后随访12-48个月,平均随访30.3个月,随访期间,未见血栓再发者。结论颅内静脉窦球囊扩张成型与取栓治疗是颅内静脉窦血栓安全、有效的治疗手段。     

Sagittal sinus thrombosis associated with transient free protein S deficiency after L-asparaginase treatment: case report and review of the literature

Cerebral sinus thrombosis associated with acquired free protein S deficiency is very rare. We report the case of an adult patient with acute lymphoblastic leukemia who presented with repeated transient ischemic attacks followed by a seizure during consolidation treatment with L-asparaginase. Magnetic resonance of the brain showed a small cortical hemorrhagic infarct. Superior sagittal sinus thrombosis was demonstrated by cerebral angiogram. A marked decrease of the free form of protein S was documented. One month later, when the patient was free of symptoms, the follow-up free protein S antigen level was restored to the normal range. We suggest that the sagittal sinus thrombosis in this patient was caused by acquired, transient free protein S deficiency. This case also extends the clinical spectrum of cerebral sinus thrombosis to include recurrent transient ischemic attacks alternating with seizures.     

Endovascular treatment of dural sinus thrombosis with rheolytic thrombectomy and intra-arterial thrombolysis

BACKGROUND AND PURPOSE: Cerebral venous thrombosis is a rare entity that can be difficult to manage. Intrasinus thrombolysis is an increasingly applied intervention, but this modality carries an increased risk of hemorrhage. We describe for the first time an option with a potentially lower incidence of intracranial bleeding, the combination of the AngioJet rheolytic thrombectomy catheter with intra-arterial thrombolysis, in 2 patients with extensive dural sinus thromboses, preexisting intracranial hemorrhage, and severe progressive neurological deficits despite heparin therapy. METHODS: Four procedures were performed in 2 patients with thromboses in the superior sagittal and transverse sinuses (right in 1 patient and bilateral in 1 patient) and cortical veins. Rheolytic thrombectomy was performed in the sigmoid, transverse, straight, and superior sagittal sinuses; this technique involves the use of the Bernoulli effect to create a vacuum that fragments and aspirates thrombus. For associated persistent cortical vein thromboses, low-dose intra-arterial thrombolysis was used. RESULTS: Both patients had excellent angiographic results with sinus reopening after rheolytic thrombectomy and cortical vein reopening after intra-arterial thrombolysis. Follow-up CT showed no change in 1 patient and increased preexisting intracranial hemorrhage in the other. One patient had a negative hypercoagulable workup, and the other patient had probable anti-phospholipid antibody syndrome. At 6 months, both patients had excellent clinical outcome with no neurological deficits except mild short-term memory loss in 1 patient. CONCLUSIONS: The combination of rheolytic thrombectomy with intra-arterial thrombolysis is a treatment modality that allows accelerated recanalization of occluded dural sinuses and cerebral veins with lower doses of thrombolytic agents.     

颅高压对兔静脉窦血栓后脑梗死的影响

目的利用兔静脉窦血栓的动物模型,观察兔静脉窦血栓后颅内压对脑组织的影响。方法成年健康日本大耳白兔95只,随机分为5组,其中建模成功90只:A、B、C、D组为处理组,均为结扎上矢状窦后1/3及其回流静脉,其中B、C、D组为在结扎基础上分别放置0.2 ml、0.4 ml、0.6 ml的硬膜外球囊,E组为假手术组(6只)。各组均在术后8 h、24 h、48 h观察脑含水量、脑梗死范围及病理变化。结果与E组相比,A、B、C、D组在相应时间点的脑含水量、脑梗死范围均明显增加,且随着颅内压增高呈增高趋势。结论在兔静脉窦血栓模型中,颅内压增高可加重脑水肿的程度和增加脑梗死的范围,解除颅内高压则可减轻脑水肿和脑梗死范围。     

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.     

Thrombus propagation and venous drainage disturbance in cerebral sinus-vein thrombosis--38 autopsied cerebral sinus-vein thrombosis

38 autopsied cases of cerebral sinus-vein thrombosis (CSVT) in our institute were studied. In this study, special attention was paid for the evolution and fate of venous thrombus. 18 cases showed hemorrhagic infarction or intracerebral hematoma (group 1; G 1). In contrast, no cerebral parenchymal changes were observed in the other 20 cases (group 2; G 2). In 13 of 18 cases of G 1, superior sagittal sinus (SSS) were thrombosed. 10 of these 13 cases showed thrombosed cerebral cortical veins (CV) or deep cerebral veins (DV). In contrast, none of 16 cases of G 1 with thrombosed SSS showed thrombosed CV or DV. All cases of the solitary thrombosis of CV or DV (each 2 cases) belong to G 1. Venous thrombi were divided into three stages according to its process of organization; recent thrombus (R), hyalinized thrombus (H), organized thrombus (O). In the venous thrombi of G 1, 6 cases were R, 6 were partly H, 6 were partly 0. In addition to O, R and H were also observed in group O. Distribution of various stage of thrombus in same case suggested that gradual thrombus evolution had occurred before or after the clinical onset in CSVT. This study suggested: (1) CV or DV occlusion may play an important role for the advent of cerebral parenchymal changes in CSVT. (2) Gradual thrombus evolution after the onset is one of possible causes of slow clinical deteoration after the onset. Therefore, prevention of these thrombus propagation with anti-platelet drugs or fibrinolotic therapy should be recommended for the treatment of CSVT. On the contrary, hyperosmolar agents and diuretics may produce potential risk of dehydration, and as a result, accelerate secondary thrombus extension.     

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.     

血管内局部溶栓治疗颅内静脉窦血栓

目的评价局部溶栓与机械碎栓治疗静脉窦血栓的影像学资料与治疗效果。方法对7例静脉窦血栓病人经股静脉穿刺,将微导管选择性插入已闭塞的静脉窦内。6例行静脉窦局部灌注尿激酶或基因重组组织型纤溶酶原激活剂,1例采用球囊机械碎栓术;3例伴脑皮质或深部静脉血栓者同时经颈动脉给予溶栓药。结果造影示6例已闭塞的静脉窦部分再通,1例无明显再通;3例侧支静脉回流增多。1例溶栓后原有脑内血肿增大,导致偏瘫加重;1例术后发生无症状出血性静脉梗死。随访10个月~3年,6例mRankin评分0~1分,1例mRankin评分2分。结论血管内局部溶栓治疗静脉窦血栓,静脉窦完全再通率低,但血管部分再通及侧支静脉回流增多,可改善病人的临床预后。     

Urokinase treatment of sagittal sinus thrombosis with venous hemorrhagic infarction

Background: Selective catheterization of the dural venous sinuses with local infusion of urokinase may be beneficial in patients with venous sinus thrombosis, and has been reported to be safe in patients with venous infarction. However, information regarding safety in the presence of hemorrhage is sparse. Methods: Three patients presented with severe, progressive focal neurological symptoms (National Institutes of Health Stroke Scales: 14, 22, and 12) resulting from superior sagittal sinus thrombosis, with evidence of hemorrhage on computed tomographic scans (two intraparenchymal, one subarachnoid). Selective venous catheterization was performed and low-dose urokinase was delivered directly into the thrombus by continuous infusion at 60,000 U/h. Intravenous heparin was administered concurrently. Results: Angiographic patency was restored in all patients. The total duration of urokinase infusion ranged from 36 to 84 hours. There was no major morbidity or mortality related to the procedure. All patients had dramatic clinical improvement during and after the course of therapy, and none had worsening of pre-existing hemorrhage. All patients were independent at 3 months, with minimal or no deficit (National Institutes of Health Stroke Scales: 2, 0, and 2). Conclusion: In selected patients with superior sagittal sinus thrombosis associated with venous hemorrhagic infarction, urokinase appears to be safe and may reverse progressive neurological deterioration. Future prospective study is warranted to further investigate this treatment option, and patients with severe deficits or pre-existing hemorrhages should not be excluded.     

Magnetic resonance imaging in lateral sinus hypoplasia and thrombosis

Lateral sinus thrombosis may be difficult to differentiate angiographically from lateral sinus hypoplasia, which mainly affects its proximal transverse portion. Using magnetic resonance imaging, we evaluated six patients who demonstrated poor filling or lack of filling of one or both lateral sinuses at angiography. In each patient, magnetic resonance imaging unambiguously demonstrated either lateral sinus thrombosis or lateral sinus hypoplasia. The latter was characterized by a frank asymmetry in size (surface of section) of the transverse portion of the lateral sinuses on parasagittal images without any abnormal signal in the course of the sinus. Lateral sinus thrombosis was indicated by increased intraluminal signal on all planes and with all pulse sequences. By virtue of its freedom from bone-related artifact, its multiplanar imaging capability, and its sensitivity to both blood flow and thrombus formation, magnetic resonance imaging is an excellent tool for the evaluation of lateral sinus thrombosis or hypoplasia.     

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.     

Evolution of venous cerebral infarction and brain edema--an experimental study of sagittal sinus obstruction

Superior sagittal sinus with/without neighboring venous system of 36 mongrel cats were occluded by cyanoacrylate polymer after i.v. administration of Evans-blue (EB). Thereafter, the cats were sacrificed 1, 3, 6, 12, 24, 72 or 120 hours after sinus-vein occlusion. According to the degree of occluded region, the cats were divided into two groups; group A (GA) and B (GB). GA showed only superior sagittal sinus occlusion, while many cortical veins were also occluded in GB. No EB nor histological changes were found in 13 cats of GA and 4 sham operated cats, while EB distribution was observed in all 20 cats of GB. The other 3 cats of GA showed a little EB in gyrus lateralis. EB distribution of GB were divided into two types. Type 1 showed EB mainly in the cortical gray matter, while type 2 showed massive EB extravasation in the white matter as well. Edematous changes with gliosis in its resolution phase, were observed in type 1. In addition, EB free zone was formed along with U-fiber zone (cortico-medullary junction) in the cats of later phase (72 hours after occlusion). The findings of EB extension in the type 1 means the existence of blockage against edema evolution from cortex toward subcortical white matter. The cats of type 2 showed fulminant hemorrhagic changes which appeared depend on time interval. Although occurrence of pathological changes were rather earlier than that of cases of clinical cerebral sinus thrombosis, this pathological findings demonstrated the typical character of venous hemorrhagic infarction. In this paper, the similarity and difference between this model and clinical case of sinus-vein thrombosis were discussed. And the possible function of the U-fiber zone in the corticomedullary junction against edema evolution was suggested.     

Cerebral cortical and deep venous thrombosis without sinus thrombosis: clinical MRI correlates

BACKGROUND: Cortical and/or deep vein thrombosis (CDVT) without dural sinus involvement is uncommon and presents diagnostic difficulty for many reasons. Our aim is to determine the relationship between magnetic resonance imaging (MRI) findings and clinical findings in patients with CDVT. METHODS: Forty-six patients with venous stroke proved on MRI included in our Registry, corresponding to 0.1% of 4650 patients with stroke, were studied. Magnetic resonance angiography (MRA) was performed in all patients, and 18 of them had follow-up MRA. Outcome was evaluated by using the Glasgow Outcome Scale at the time of discharge and during follow-up. RESULTS: Thirty-two patients presented cortical venous stroke; 21 of them had involvement of the dorsomedial venous system, six had a defect in the posteroinferior venous group, and five had a defect in the anteroinferior venous group. Thirteen patients presented simultaneous involvement of the superficial and deep venous system; seven with a defect in the parietal and internal cerebral veins (three with involvement of vein of Gallen), four with a defect in the temporooccipital (vein of Labbé) and basal vein of Rosenthal, two with a deficit in the anterior frontotemporal and uncal-pterygoid venous system. One patient had deep venous thrombosis primarily localized to the thalami bilaterally and the basal ganglia on the right because of occlusion of the thalamostriate veins. The main presenting symptoms of CDVT were headache, focal neurologic signs, partial complex or secondary generalized seizures, and consciousness disturbances in those with deep venous thrombosis, presented alone or in combination at onset. CDVT was more than twofold more frequent in women than in men. Pregnancy, puerperium, oral contraceptive use, and infections were the most common predisposing factors. CONCLUSION: Computerized tomography, conventional MRI and diffusion-weighted imaging showing ischemic and/or hemorrhagic lesion that does not follow the boundary of classical arterial boundaries without signs of sinus thrombosis, and partial or generalized seizures followed by focal neurologic signs may predict CDVT. The outcome of patients with cortical venous stroke was good, but not in those with cortical plus deep venous infarction.     

Dural sinus thrombosis: a mechanism-based classification and review of 42 cases

Cranial venous outflow obstruction due to dural sinus thrombosis may result in venous hypertension, cerebral infarction, cerebral haemorrhage or impaired cerebrospinal fluid (CSF) absorption with consequent pseudotumour syndrome. We propose a mechanism based classification of dural sinus thrombosis from these four outcomes. Forty two cases of dural sinus thrombosis presenting to Royal Prince Alfred Hospital between 1986-1997 were retrospectively reviewed. These cases were classified according to mechanism of presentation and relevance of this to site of thrombosis, treatment and prognosis. This study shows that the superior sagittal sinus and transverse sinus are the commonest sites of thrombosis, and multiple sites of thrombosis (69%) are more frequent than a single site. Magnetic resonance imaging (MRI) with venous flow studies is the investigation of first choice for diagnosis but angiography remains the gold standard. A pseudotumour syndrome is the commonest presentation (43%) followed by cerebral haemorrhage (31%). The overall prognosis for sinus thrombosis is good, with 71% of cases recovering to normal function.