MRI and SPECT studies of dural arteriovenous fistulas presenting as pure progressive dementia with leukoencephalopathy: a cause of treatable dementia

We report two patients with dural arteriovenous fistulas (DAVFs) who presented with pure progressive dementia. Both patients showed only slowly progressive dementia, without headache, papilledema and other neurologic signs associated with diffuse white matter changes in MRI. MR cerebral angiography showed sigmoid sinus DAVFs that were mainly supplied by the occipital artery, together with retrograde filling of the superior sagittal and straight sinus and dilated cortical veins. SPECT studies showed extensive blood flow reduction in the occipital and parieto-occipital areas and right temporal lobe in one patient. Selective embolization for treatment of the DAVF improved cognitive function associated with the abnormal white matter MRI signal. MRI and SPECT showed that severity of dementia correlated with diffuse white matter changes and regional cerebral blood flow. Our cases suggest that gradually impaired cerebral circulation due to venous hypertensive encephalopathy could be involved in slowly progressive dementia with leukoencephalopathy resulting from a DAVF. DAVFs may be particularly important for differential diagnosis in elderly patients with pure progressive dementia. Thus, early diagnosis of DAVFs and treatment by endovascular surgery is important as treatable or reversible dementia.     

Intracranial dural arteriovenous fistula presenting as parkinsonism and cognitive dysfunction

There are few syndromes of parkinsonism with dementia which can be treated. We report two patients with dural arteriovenous fistula (DAVF) who presented with rapidly progressive parkinsonism and cognitive dysfunction. DAVF are rare lesions resulting from abnormal connections between meningeal arteries and dural sinuses. Angiography in both patients revealed DAVF associated with multiple occlusive changes in the dural venous sinus. This report emphasizes the need for a high level of clinical suspicion to diagnose DAVF and consider it as one of the causes of rapidly progressive cognitive dysfunction in patients with parkinsonism.     

Intracranial dural arteriovenous fistula (DAVF) presenting progressive dementia and parkinsonism.

We studied three patients with dural arteriovenous fistula (DAVF). Major symptoms were progressive dementia and parkinsonism, both of which progressed in step-wise fashion. Two of the three patients showed diffuse cerebral white matter lesions on brain CT and MRI. Progressive dementia and parkinsonism in our patients could be caused by diffuse cerebral parenchymal disturbance: impaired cerebral circulation due to severe venous hypertension. DAVF is important for the differential diagnosis in patients with progressive dementia and parkinsonism.     

Clinical picture of patients with cerebral venous thrombosis and patterns of dural sinus involvement

BACKGROUND/OBJECTIVES: Cerebral venous thrombosis may cause focal brain lesions or an isolated intracranial hypertension without focal signs. We investigated whether these different clinical pictures correspond to different patterns of dural sinus thrombosis. METHODS: Forty-eight patients with cerebral venous thrombosis were classified in 2 groups based on the clinical and neuroradiological picture at admission: one group had clinical signs of intracranial hypertension, without focal neurological signs; the other group had focal neurological signs. The extension of thrombosis in dural sinuses was compared between the groups. RESULTS: The amount of thrombosed sinuses was significantly different in the two groups, the involvement of the dural sinuses being greater in patients with isolated intracranial hypertension. CONCLUSIONS: The clinical picture of cerebral venous thrombosis is related to the pattern of dural sinus involvement. The presence of isolated intracranial hypertension is more frequent in patients with a more extended thrombosis of the dural sinuses.     

rCBF study with 123I-IMP SPECT of dural arteriovenous fistula]

It is important for dural arteriovenous fistula (DAVF) to evaluate venous ischemia in the brain related to venous hypertension, but only a few such studies have been performed. In this study, regional cerebral blood flow(rCBF) in DAVF was examined for venous ischemia by 123I-IMP SPECT. The subjects were eighteen patients with DAVF. Of the eighteen patients, nine had DAVF with low perfusion areas and venous ischemia. The factors affecting rCBF in DAVF are: 1) the presence of retrograde leptomeningeal venous drainage, 2) sinus occlusion, and 3) DAVF with high flow. The presence of retrograde leptomeningeal venous drainage was observed in nine patients, sinus occlusion in four patients, high flow in three patients. In two patients, pure leptomeningeal venous drainage was formed by patent sinus, and blood regurgitated from DAVF on the sinus wall to cortical vein. When DAVF was associated with LMVD, most patients had venous hypertension and concomitant venous congestion in the same areas due to reduced venous circulation, resulting in a decrease in rCBF and an increase in regional cerebral blood volume. These hemodynamics suggest venous ischemia in the brain. 123I-IMP SPECT was useful for evaluating rCBF and as a parameter of the treatment.     

硬脑膜动-静脉瘘合并髓静脉扩张的临床及影像学表现(附1例报告及文献复习)

目的报道1例硬脑膜动-静脉瘘(DAVF)合并髓静脉扩张病例的临床资料,探讨DAVF合并髓静脉扩张的临床及影像学表现。方法回顾性收集1例DAVF合并髓静脉扩张患者的临床及影像学资料,并结合文献复习进行分析。结果患者以症状性癫痫起病。头颅MRI表现为脑白质内迂曲、扩张血管影,并继发性脑梗死和脑水肿;全脑数字减影血管造影(DSA)提示右侧颈内动脉造影静脉期上矢状窦前1/3不显影,静脉晚期颅内多发髓静脉迂曲增粗,经室管膜下静脉-大脑内静脉回流,右侧颈外动脉造影示DAVF形成,大脑上静脉逆向回流,左侧颈内动脉造影示左侧发育性静脉畸形。经血管内介入栓塞DAVF治疗后患者病情改善。结论DAVF合并髓静脉扩张少见,可继发脑实质病变进而出现神经功能缺损症状与体征;影像学表现为头颅MRI提示髓静脉扩张涉及的疾病谱较多,需仔细鉴别;MRI和DSA联合检查能更好评价脑实质和血管情况。     

Endovascular angioplasty and stent placement in venous hypertension related to dural arteriovenous fistulas and venous sinus thrombosis

Venous hypertension in lateral sinuses resulting from dural arteriovenous fistula (DAVF) and venous sinus thrombosis (VST) can manifest with severe neurological deficits, such as infarction or intracerebral hemorrhage. It has been proved that venous hypertension plays a significant role in the evolution and progression of DAVF and VST. The definite treatment in complicated conditions such as multiple DAVFs or multiple sinus occlusions is still unknown. Traditional transarterial embolization, transvenous embolization or radiosurgery alone has limited effects on these conditions. We reported one case with venous hypertension presenting with severe neurological symptoms. The case had quick clinical recovery after correction of venous hypertension by endovascular angioplasty and stent placement in occluded lateral sinuses. Accordingly, we propose this method can be an ideal treatment option either in single or staged therapy of venous hypertension related to DAVFs and VST.     

A case of membranous lipodystrophy (Nasu) with diffuse cerebral white matter involvement and cerebellar atrophy on brain CT and MRI

A 37-year-old female was admitted to our hospital because of progressive dementia and gait disturbance. She was healthy until 34 years of age when she had difficulty in walking and memory disturbance with personality changes. At age 36, she developed urinary incontinence and dementia. The neurological examination demonstrated euphoric mental state, emotional incontinence, severe dementia, paraplegia, dysmetria, choreic movements in both arms and urinary incontinence. Diffuse hyperreflexia and bilateral Babinski signs were observed. Routine laboratory examination showed slightly increased erythrocyte sedimentation rate and alkaline phosphatase. Electroencephalogram revealed diffuse irregular slow waves. X-ray film of the ulnar bone revealed osteoporotic and cystic lesions. The biopsy of the left tibial bone showed a specific membranous cystic structure. Computerized tomography (CT) of the brain showed symmetric, diffuse low density areas in the cerebral white matter and severe atrophy of the cerebellum. T2-weighted magnetic resonance imaging (MRI) revealed diffuse high intensity areas in the cerebral white matter. The present case is characterized by diffuse changes in cerebral white matter and cerebellar atrophy, which have been never reported in Nasu-Hakola disease. The diffuse cerebral white matter changes shown by CT and MRI appear to indicate that this patient is the first case of leukodystrophy of sudanophilic type since the original case reported by Nasu et al.     

MRI和MRV在脑静脉和静脉窦血栓诊断中的价值

目的探讨MRI和MRV对脑静脉和静脉窦血栓(CVST)形成的诊断价值。方法回顾性分析16例CVST患者的临床和影像学资料。结果受累的脑静脉和静脉窦有上矢状窦9例,横窦5例,直窦3例,乙状窦2例,大脑大静脉合并大脑内静脉2例。常规MRI可见受累静脉窦流空信号消失,静脉窦内信号高低不一,11例脑实质内出现静脉性脑梗死表现,其中4例合并出血。增强扫描15例静脉窦内出现充盈缺损及静脉窦壁强化表现,9例脑实质内出现脑回样强化。MRV表现为受累静脉窦部分或广泛高血流信号缺失、狭窄。结论MRI和MRV是诊断和随访CVST形成的最佳检查方法。     

脑静脉窦血栓形成的影像学表现及脑实质病变预测

目的总结脑静脉窦血栓形成(CVST)患者的临床和MRI表现特征并探讨继发脑实质病变的预测因素。方法回顾57例CVST患者的临床及头颅MRI资料,分析患者MRI表现特征,并对临床及影像学指标进行对比分析。结果57例患者中男22例,女35例,年龄15~72岁(平均35岁)。临床症状头痛29例,癫痫发作11例,感觉变化8例,局灶性神经功能缺损9例;头颅MRI示静脉窦血栓形成部位上矢状窦9例,横窦2例,乙状窦1例,直窦1例,其中同时累及2个或2个以上静脉窦44例。静脉窦血栓伴发脑实质病变36例,其中出血性静脉梗死20例,非出血性静脉梗死12例,脑实质内出血4例。对伴和不伴脑实质病变2组患者的对比分析显示,伴脑实质病变组女性比例明显高于不伴脑实质病变组;伴脑实质病变组癫痫、感觉异常及局部神经功能缺损症状发生率均明显高于不伴脑实质病变组;伴脑实质病变组直窦血栓的发生率明显高于不伴脑实质病变组(P<0.05)。结论MRI可清楚显示CVST的直接及间接征象,通过分析CVST患者的临床和静脉窦血栓表现特征还可帮助预测严重的脑实质病变发生风险。     

表现为神经功能缺损的3例硬脑膜动静脉瘘误诊分析

目的探讨表现为神经功能缺损的硬脑膜动静脉瘘(DAVF)的临床及影像特点,以提高对该病的诊断率。方法回顾性分析3例临床表现为神经功能缺损的DAVF患者的病例及影像资料。结果例1:发作性言语不能,右侧肢体活动不灵,误诊为TIA;例2:痴呆、双下肢行走不稳、尿失禁,既往有头部伤史,误诊为正常颅压脑积水;例3:左侧动眼神经麻痹,既往糖尿病史9年,误诊为糖尿病动眼神经麻痹。3例均经DSA证实为DAVF。前两例头部MRI重新仔细阅片,发现两侧大脑皮层存在不对称的血管流空信号,提示DAVF,第3例左侧动眼神经麻痹患者头MRI和CTA均未见异常,行全脑DSA进一步除外动脉瘤时,意外发现病因为颈外动脉系统供血的海绵窦区DAVF。结论当患者有神经功能缺损、临床表现与影像不一致、对治疗不佳时,应考虑到DAVF的可能,注意MRI上有无细微的血管形态改变,以减少误诊。     

Incidence of hereditary thrombophilia in patients with cranial dural arteriovenous fistulae

Dural arteriovenous fistulas (DAVFs) can develop as consequence of prior venous sinus thrombosis. However, the prevalence of prothrombotic conditions in patients with intracranial DAVFs is unknown and there is no consensus on the indications to screen for procoagulable conditions in these patients. We performed a retrospective review of patients presenting to our institution for management of cranial DAVF. Patient and DAVF characteristics were noted, in particular the incidence of hereditary thrombophilia (HT) detected by laboratory evaluation. Respective comparisons of patients with and without HT were performed using comparative statistics. There were 165 patients presenting for management of 171 DAVFs. A large proportion of DAVF were located at a large dural sinus (42.7%), which included superior sagittal, transverse, and sigmoid sinuses. On multivariate logistic regression analysis cortical venous drainage was independently associated with hemorrhagic presentation (OR 8.23, 95% CI 1.78–38.08; p = 0.007). A HT was detected in 18 of 61 patients (29.5%) who underwent a thrombophilia work-up. Location at a large dural sinus was not more common in patients with a HT (55.0% vs 54.5%; p = 0.97). There was also no difference in the presence of cortical venous drainage (56.5% vs 38.6%; p = 0.22) or hemorrhage on presentation (15.9% vs 5.0%; p = 0.22) in patients with and without a HT. In HT were found to be highly prevalent in our cohort, though our results do not suggest their presence greatly influences DAVF pathophysiology. Screening for procoagulable states is indicated in selected patients with an intracranial DAVF.     

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.     

硬脑膜动静脉瘘的影像特征分析

目的 通过分析硬脑膜动静脉瘘（dural arteriovenous fistula,DAVF）患者的影像学检查结果,探索DAVF在不同影像学检查中的特征性表现,为临床诊断治疗提供依据。方法 对自2005年以来在我院治疗的32例DAVF患者的影像学资料进行回顾性分析。结果 32例DAVF患者,颅脑CT和磁共振成像（magnetic resonance imaging,MRI）检查能较好地显示因DAVF所致的颅内继发性病变,如扩张的引流静脉,继发性出血及静脉窦狭窄、闭塞等改变。数字减影血管造影（digital subtraction angiography,DSA）检查则能准确地显示瘘口部位、供血动脉、静脉引流情况及颅内血流分布情况,但无法显示继发的脑实质病变。 结论 DSA检查是诊断DAVF的金标准。CT、MRI检查亦能为诊断DAFV提供依据。     

Dural sinus thrombosis in spontaneous intracranial hypotension

Dural sinus thrombosis (DST) is rarely associated with spontaneous intracranial hypotension (SIH). Engorgement of the venous system, caused by the CSF loss that occurs in SIH, is considered to favour the thrombosis, although signs of both SIH and DST are usually seen simultaneously at the first diagnostic MRI. We observed two patients with SIH and DST. Changes in pattern of headaches and MRI findings demonstrated that DST followed SIH. In SIH, the velocity of the blood flow in the dural sinuses may be reduced because of dilatation of the venous system which compensates the CSF loss. Other possible mechanisms seem unlikely on the grounds of both clinical presentation and MRI studies. Received in revised form: 6 December 2005     

颅内静脉窦血栓形成的临床及影像学诊断25例报道

目的 研究颅内静脉窦血栓形成的病因、临床特点、特别是影像学的表现。方法 回顾性分析25例已确诊的静脉窦血栓形成患者（男2例,女23例）的临床资料及影像学特点,包括发病原因、临床表现、实验室检查及影像学头部CT、磁共振成像（MRI）和磁共振血管成像（MRA）的异常表现。结果 25例本病患者除2例男性外,余23例均有明确病因,如妊娠期、产褥期、口服避孕药等,临床表现以亚急性颅压高症状为主;部分头部CT可见条索征及空三征,MRI特征性表现为T1、T2加权像窦内异常高信号,MRA示栓塞的静脉窦未显影。结论 颅内静脉窦血栓形成患者多可找到明确病因,临床表现多样,无特异性,MRI/MRA可帮助确诊。     

Thrombosis of deep cerebral veins in form adults: clinical features and diagnostic approach

Thrombosis of deep cerebral veins is a rare condition, and is associated with a poor prognosis. We report four new cases observed between 1994 and 1997. All four cases were women, aged less than 45 years. Initial symptoms associated alteration of consciousness, change in mental status, progressive headache and vomiting. We observed also uni or bilateral signs of long tract injury. In three cases, diagnosis initially suspected by CT scan was confirmed with encephalic MRI. For the last patient, conventional angiography was needed. Thrombosis affected straight sinus, vein of Galien and internal cerebral veins in all patients. Basilar veins were also affected in one patient, without dural sinuses extension. Lateral sinus was involved in two others cases, and superior sagittal in the last patient. Etiology remains undetermined in one patient, associated with post-partum, use of oral contraceptive pill, and familial protein S deficiency, one case each. Outcome was favorable in all four cases with anticoagulation therapy. Precocity of diagnosis is determinant and MRI is usefull in this issue. These observations show that evolution of deep veins thrombosis can be favorable, without needing fibrinolytic therapy.     

Dural arteriovenous malformation with abnormal parenchymal vessels: an autopsy study

Summary A 48-year-old man with dural arteriovenous malformation (AVM) is reported. Radiologically, the dural AVM was demonstrated mainly in the region of the left transverse sinus. Postmortem examination revealed dural AVM involving the bilateral transverse, superior sagittal and straight sinuses. In addition, numerous malformed venous vessels and extensive necrosis were observed mainly in the parenchyma of the brain stem and cerebellum. The present case suggests that dural AVMs may be associated with malformed venous vessels in the brain parenchyma.     

Combined Surgical and Endovascular Management of a Complex Posttraumatic Dural Arteriovenous Fistula of the Tentorium and Straight Sinus

Diagnosis and management of a complex tentorial dural arteriovenous fistula (DAVF) of the straight sinus with vein of Galen aneurysmal dilatation is presented. A 65-year-old woman with remote history of cranial gunshot lapsed into coma after months of progressive neurological decline. Computed tomography brain scan showed cerebellar hemorrhage while arteriography demonstrated a complex arteriovenous fistula of an isolated straight sinus and tentorium with extensive arterial supply. First extracranial then intracranial arterial feeders to the fistula were occluded in separate procedures following initial presentation. Eleven days after presentation, an occipital burr-hole craniotomy was performed, the isolated straight sinus was cannulated under fluoroscopic guidance, and the fistula eradicated with multiple thrombogenic fibered platinum and Gugliemi detachable coils. Staged embolization now represents the standard of care for many complex DAVFs. A multi-disciplinary surgical and endovascular approach is a valuable combination to cure deep lesions with limited surgical or transvascular access.     

Diffusion-Weighted Magnetic Resonance Imaging of Spontaneous Venous Infarction and Cortical Hemorrhage Complicating Dural Arteriovenous Fistula

Dural arteriovenous fistula (DAVF) is an abnormal arteriovenous shunt that occurs in the dura matter within or near a dural sinus. The clinical manifestations vary. The authors report herein a rare case of type III DAVF presenting with coexisting intracranial hemorrhage (ICH) and venous infarction evidenced by computed tomography (CT) and diffusion-weighted magnetic resonance imaging. Conventional angiography proved that the right middle meningeal artery and the occipital artery supplied the DAVF, with direct drainage into the cortical veins and superior sagittal sinus.