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.     

手术治疗硬脑膜动静脉瘘

目的探讨手术切除硬脑膜动静脉瘘的方法。方法2005年5月至2006年6月共收治7例难治性硬脑膜动静脉瘘患者，男性6例，女性2例，年龄25-42岁。病变部位以双侧大脑凸面硬脑膜为主3例，乙状窦小脑幕病灶2例，前颅窝和眶尖病灶1例，矢状窦双侧乙状窦1例。手术依据病灶部位，选择额颞、颞枕、额下、乙状窦前或联合入路，电凝、离断病灶供血动脉，切除受累的病变硬脑膜，沿动脉化静脉分流方向寻找瘘口，切断动静脉分流，必要时也可切断压力较高严重动脉化的静脉窦，改善静脉回流，恢复神经功能缺失。结果8例中5例病人术后症状消失或明显好转。硬脑膜动静脉瘘消失，1例手术中出血死亡。结果手术切除各种复杂硬脑膜动静脉瘘可作为一种有效的方法，但应防止术中较大的动静脉瘘和静脉窦撕裂产生的致使性出血。     

A tentorial dural arteriovenous fistula presenting progressive myelopathy: a case report

Abstract We report a case of a dural arteriovenous fistula (DAVF) at the tentorium cerebelli, which presented progressive myelopathy. A 68-year-old man with neurological deterioration of the cervical myelopathy visited our hospital. T₂ weighted magnetic resonance (MR) imaging showed high signal area and edema from the medulla to the upper thoracic spinal cord with flow voids on the dorsal surface of the cord. Angiography showed right tentorial DAVF, which was supplied by the right meningohypophyseal trunk, the middle meningeal artery, the accessory meningeal artery, and was drained into the posterior spinal veins. The patient underwent right retrosigmoid suboccipital craniotomy, then disruption of the fistula was performed by using micro Doppler sonography following endovascular obliteration of the main feeders. Postoperative angiography showed complete obliteration of the fistula. His daily functioning gradually improved up to 6 months after the surgery. Tentorial DAVFs with clinical manifestation of myelopathy are rare. Considering its aggressive nature, early surgical treatment could be necessary. (Received: November 17, 2010, Accepted: December 18, 2010).     

Endovascular treatment of vein of Galen dural arteriovenous fistula presenting as dementia

Dural arteriovenous fistula (DAVF) is an important cause of neurological dysfunction that is often misdiagnosed, especially in elderly population. Galenic DAVFs are a subtype of the rare falcotentorial DAVFs with a high risk of hemorrhage and aggressive clinical course. In most cases, DAVFs present with pulsatile tinnitus, headache, or orbital symptoms such as chemosis and proptosis. We report a patient with DAVF of Vein of Galen presented with progressive dementia, treated by Onyx embolisation and had good clinical outcome.     

Intracranial dural arteriovenous fistula with spinal venous drainage]

Two cases of intracranial dural arteriovenous fistulae draining into the medullary veins are reported. One patient was a 68-year old man who experienced brief and repeated episodes of paraplegia, followed by a permanent spinal cord syndrome. The other patient was a 74-year-old woman who rapidly developed a syndrome of the medulla and spinal cord. In both cases spinal cord angiography failed to show vascular malformations, but myelography revealed venous imprints and magnetic resonance imaging of the cervical spinal cord disclosed a pre-medullary signal. Cerebral angiography showed an intracranial arteriovenous fistula fed by the external carotid artery and draining into the veins of the posterior fossa and the spinal venous network. Complete occlusion of the fistula was achieved by embolization with microparticles. Intracranial dural arteriovenous fistulae draining into the medullary veins are rare: to our knowledge, only 4 cases have been reported. Such lesions must be considered whenever a spinal cord syndrome has no detectable cause.     

引起脊髓功能障碍的硬膜动静脉瘘的临床特点

目的 回顾性研究引起脊髓功能障碍的硬膜动静脉瘘（DAVF）的临床特点，促进此类疾病的早期诊治。方法 对15例引起脊髓功能障碍的DAVF患者进行诊治。手术相关性DAVF1例，自发性DAVF14例。缓慢起病11例，急性起病4例。磁共振T2加权脊髓周围异常血管流空11例，脊髓被增强7例。血管造影证实硬膜瘘口位于小脑幕1例，颈段4例，胸段7例，腰骶尾段3例。手术靠近瘘口切断引流静脉6例，介入栓塞瘘口6例，介入后手术2例，保守治疗1例。结果 1例手术治疗患者失随访，1例拒绝手术治疗的患者确诊后1周死亡，其余13例患者随访时间为6-58个月。5例手术治疗的患者均治愈；6例介入治疗的患者，3例治愈，2例好转，1例无变化；2例介入后手术治疗的患者，1例好转，1例双下肢顽固性疼痛无变化。结论 掌握引起脊髓功能障碍的DAVF的起病形式、磁共振特点和血管造影检查要点，有利于DAVF的早期诊治、提高治愈率和患者的生命质量。     

Spinal dural arteriovenous fistula

PURPOSE OF REVIEW: To summarize clinical key points, diagnostic features, and results of imaging and therapy of spinal dural arteriovenous fistula (SDAVF). RECENT FINDINGS: SDAVF accounts for 70% of spinal arteriovenous malformation with an annual incidence of 5-10 cases per million. At least 80% of patients are male, and more than 66% of patients are in the sixth and seventh decade of life indicating preponderance of gender and age. Thrombophilia is not a predisposing factor of disease. Clinical course is predominated by symptoms of congestive myelopathy, but subarachnoid hemorrhage may occur. Double SDAVF is a rare problem in the management of disease. Magnetic resonance imaging has replaced myelography as screening procedure. Contrast-enhanced magnetic resonance angiography and multislice computerized tomographic angiography may facilitate diagnostic procedure, however, spinal angiography is still required to confirm diagnosis. Treatment by permanent occlusion of fistula results in clinical improvement in 70% of cases. Microsurgical shunt interruption has proven secure and reliable. Endovascular shunt embolization has been established as a standardized procedure, but occlusion rates are still lower than in surgical treatment. SUMMARY: Advances have been made in diagnosis and treatment of SDAVF, but the disease is still not completely understood.     

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 draining into spinal cord veins: case report

We present an usual case of intracranial dural arteriovenous fistula with perimedullary and spinal cord venous plexus drainage and discuss its etiological, physiopathological, diagnostic and therapeutic aspects.     

Clinical characteristics of dural arteriovenous fistula.

Intracranial dural arteriovenous fistula (DAVF) is an uncommon neurosurgical condition; in particular, it has been infrequently reported in Korea. To understand the general clinical characteristics of DAVFs, the authors reviewed 53 cases and analyzed factors affecting DAVF hemorrhage of and treatment outcome. Since 1980 we have encountered 480 pial and 53 DAVFs, a ratio of 9.1 to 1. The age of these patients ranged from 1 month to 71 years, the most common being in the 6th decade, and females exceeded males by 1.65 to 1. All lesions except three were single, and symptoms were related to location and the venous drainage pattern. The most common location was the cavernous sinus, accounting for about 64% of cases, with the result that the most common clinical symptoms of DAVFs were ocular, namely proptosis and chemosis. The next was tinnitus also found in transverse-sigmoid sinus DAVFs. Intracranial hemorrhage was seen in eight cases,(15%) the primary cause of hemorrhage was retrograde intracranial venous drainage (P=0.017), and one hemorrhage was observed in cases with no intracranial venous drainage. Intracranial hemorrhage was more frequently in transverse-sigmoid than cavernous sinus DAVFs (P=0.049), and this proved to be so even where there was intracranial venous drainage. However, two of 34 patients with cavernous DAVFs became blind in one eye, demonstrating that in such patients, the clinical course could be aggressive. Thirteen patients were treated conservatively. The conservative treatment group was comprised of 13 patients, two of three patients with transverse-sigmoid sinus DAVF expired, and 7 of 10 with cavernous sinus DAVF experienced a clinical improvement or cure. Surgical excision was performed in only two patients. A total of 39 patients underwent embolization; clinical cure was achieved in 13, improvement of symptoms in 12, an unchanged or aggravated result occurred in 9, one died, and four were lost to follow up. During intervention, there was one hemorrhagic complication, owing to obstruction of the venous outflow with embolic materials.In this study, the most common location of DAVFs was the cavernous sinus. The cortical venous drainage remains the primary determinant of intracranial hemorrhage. Common indications for treatment include hemorrhage and neurological deficit. Endovascular treatment is preferred in the majority of cases except tentorial DAVF. The goal of embolization in cavernous DAVF is the alleviation of symptoms, not angiographic cure. But transverse-sigmoid sinus DAVF with venous restriction and leptomeningeal drainage should be treated aggressively.     

脑动静脉畸形、硬脑膜动静脉瘘与软脑膜动静脉瘘的鉴别

正脑动静脉畸形(arteriovenous marformation,AVM)、硬脑膜动静脉瘘(dural arteriovenous fistula,DAVF)及软脑膜动静脉瘘(pial arteriovenous fistula,PAVF)为脑血管畸形的不同类型。脑AVM占脑血管畸形的56.3%～80%,占人口总数的0.001%～0.01%,为颅内动脉瘤的1/7～1/4。     

硬脑膜动静脉瘘介入栓塞治疗

目的 探讨血管内栓塞治疗硬脑膜动静脉瘘(DAVF)的临床技巧与治疗效果.方法 收集珠江医院自2000年至2009年收治的DAVF患者50例,应用微导管技术,以各种不同材料在数字减影血管造影辅助下行血管内栓塞治疗,通过影像学检查及临床症状随访来评价临床疗效.结果术后即刻造影显示43例瘘口完全闭塞,6例瘘口绝大部份不显影,1例瘘口部分消失.随访6个月至7年,临床症状消失34例,症状明显缓解6例,复发7例,症状加重3例;无一例发生并发症.结论 应用微导管技术栓塞是治疗DAVF的有效手段,正确选择栓塞途径、材料和提高术者技术水平是治疗成功的关键.

Abstract：

Objective To explore the clinical skills and curative efficacy of endovascular embolization on dural arteriovenous fistula (DAVF). Methods A total of 50 patients with DAVF,admitted to our hospital from 2000 to 2009, underwent endovascular embolization with different materials through a microcatheter under DSA. After embolization, imaging examination was applied and clinical manifestations were noted during the clinical follow-up to evaluate the clinical efficacies.Results Immediate postoperative angiography indicated that the fistulas were totally occluded in 43patients, occluded evidently in 6 and occluded partially in 1. The 6-84 months follow-up found that clinical symptoms of 34 patients disappeared, 6 alleviated evidently, 7 relapsed, and 3 worsened; no complications were noted. Conclusion The endovascular embolization for DAVF is effective; and choosing the right embolic approach and material and improving the technology are the keys of successful treatment.     

Myelopathy from intracranial dural arteriovenous fistula

Dural arteriovenous fistulas arising intracranially are an uncommon cause of progressive myelopathy. This report is of a patient in whom the diagnosis of the condition was confounded by coexisting small vessel cerebrovascular disease.     

Bilateral ophthalmic-ethmoidal dural arteriovenous fistula presenting with intracranial hemorrhage: a rare entity

Dural arteriovenous fistulas (DAVFs) are rare lesions. The most common locations of DAVFs are cavernous, sigmoid and transverse sinuses. Anterior cranial fossa is one of the less frequent placement for DAVFs and the risk of hemorrhage in this region is increased. Reported hemorrhage risks have been ranged from 62 to 91 %, and an aggressive clinical course is more likely than a benign clinical course. A 47-year-old man was admitted to our emergency room with headache and the computed tomography revealed frontal hemorrhage. Neurological examination was normal. We applied cerebral angiography in our interventional neurology department and an anterior cranial fossa DAVFs, supplied by bilateral ophthalmic-ethmoidal arteries, was determined. DAVFs are a rare cause of intracranial hemorrhages and in the literature anterior cranial fossa DAVFs have been reported scarcely, so that we aimed to present this rare entity.     

硬脊膜动静脉瘘误诊分析

目的 探讨如何避免误诊硬脊膜动静脉瘘。方法 回顾分析3例误诊的硬脊膜动静脉瘘的临床表现，误诊原因及治疗结果。结果 1例误诊为腰椎间盘突出，2例误诊为前列腺肥大，不仅延误了诊断，还因误治加重了神经功能损害或导致正常器官损害。结论 仔细询问病史和查体，适时行脊髓MRI检查是避免误诊的关键。     

Atypical manifestation of dural arteriovenous fistula

A case of secondary dural arteriovenous fistula presenting as infantile stroke, in a fifteen month old boy, is reported. The initial impression on CT scan in this case was misleading, due to the atypical appearance of the pathological periventricular blood vessels, interpreted as periventricular calcification.