Spinal Dural Arteriovenous Fistula in a Young Male Associated with Craniospinal Leptomeningeal Spread of a Treated High-Grade Glioma.

Spinal dural arteriovenous fistulae (SDAVF) are most commonly idiopathic in origin but may occasionally be seen secondary to surgery, trauma, or inflammation. We report a case of 27-year-old male who came with features of a myelopathy. He was found to have an SDAVF associated with leptomeningeal spread (LMS) of a previously treated high-grade cerebral glioma. Hemorrhagic presentation of gliomas, as in this case, is due to upregulation of vascular endothelial growth factor, which has also been postulated to play a role in the development of SDAVFs. This may suggest a possible mechanism of induction of secondary SDAVFs associated with such tumors. While the coexistence of intracranial neoplasms with vascular malformations has been reported previously, this is the first case report of LMS of a high-grade glioma associated with an SDAVF.

Sacral dural arteriovenous fistula mimicking multiple mononeuropathy.

A sacral dural arteriovenous fistula (dAVF) is extremely rare, and the pathophysiological and clinical features have not been established. A 70-year-old man developed gradually progressive right-dominant bilateral sensory disorder of the lower limbs. His clinical course and electrophysiological findings were similar to those of multiple mononeuropathy. However, angiography showed a sacral dAVF at the right intervertebral foramen between the fifth lumbar and first sacral vertebrae. Endovascular embolization of the dAVF improved his clinical symptoms and electrophysiological findings. A sacral dAVF can mimic multiple mononeuropathy in terms of its clinical features and electrophysiological findings. A sacral dAVF is a treatable disease and should be considered as a differential diagnosis of lower extremity disorders.

A Damp-and-Push Technique for the Copolymer (Onyx) Embolization of Dural Arteriovenous Fistula.

BACKGROUND: Copolymer (Onyx) embolization is an effective treatment for dural arteriovenous fistula (dAVF), however, some dAVFs have multiple, high-flow feeding vessels, resulting in insufficient embolization. For the treatment of such patients, we have developed a novel flow-control technique, the 'damp-and-push technique'. The purpose of this study was to evaluate the technical efficiency and safety of this technique. METHODS: Seven patients who had been diagnosed with intracranial dAVF were treated by transarterial Onyx embolization using the damp-and-push technique between 2016 and 2019. This technique was designed to reduce blood flow to the shunt site using a balloon catheter in the major feeding vessel other than the one injected with Onyx, leading to better Onyx penetration and enabling more controlled embolization of complex dAVFs. Retrospectively collected data were reviewed to assess the occlusion rates and clinical outcomes. RESULTS: The dAVF was at a transverse sinus-sigmoid sinus junction in four patients, in the superior sagittal sinus in two, and in the tentorium in one. Five cases were Cognard type Ⅱb and two cases were Cognard type Ⅳ. All the patients were treated by transarterial Onyx injection via the main feeding vessel, combined with flow reduction in the other main feeding vessel using a balloon catheter. Complete occlusion was achieved in six patients and elimination of cerebral venous reflux was achieved in all the patients. There were no immediate or delayed post-interventional complications. CONCLUSIONS: Transarterial Onyx embolization of dAVF using the damp-and-push technique is safe and yields a high complete occlusion rate.

Abnormal development of cerebral arteries and veins in offspring of experimentally preeclamptic rats: Potential role in perinatal stroke.

Preeclampsia, a hypertensive disorder of pregnancy, complicates up to 10 % of all pregnancies and increases the risk for perinatal stroke in offspring. The mechanism of this increase is unknown, but may involve vascular dysfunction. The goal of this study was to evaluate the effect of experimental preeclampsia (ePE) on cerebrovascular function in offspring to eludciate a possible mechanism for this association. Dams were fed a high cholesterol diet beginning on day 7 of gestation to induce experimental preeclampsia. Middle cerebral arteries (MCA) and the Vein of Galen (VoG) were isolated from pups from ePE dams and compared to pups from normal pregnant (NP) dams at postnatal days 16, 23, and 30 and studied pressurized in an arteriograph chamber. Markers of inflammation and oxidative stress were measured in serum. Our results suggest altered structure and function in both MCA and VoG of ePE pups. We also found evidence of systemic inflammation and oxidative stress in ePE pups. These findings provide a potential link between preeclampsia and the occurrence or severity of perinatal stroke.

Central nervous system vascular malformations: A clinical review.

CNS vascular malformation is an umbrella term that encompasses a wide variety of pathologies, with a wide range of therapeutic and diagnostic importance. This range spans lesions with a risk of devastating neurological compromise to lesions with a slow, static or benign course. Advances in neurovascular imaging along with increased utilization of these advances, have resulted in more frequent identification of these lesions. In this article, we provide an overview on definitions and classifications of CNS vascular malformations and outline the etiologic, diagnostic, prognostic, and therapeutic features for each entity. This review covers intracranial and spinal cord vascular malformations and discusses syndromes associated with CNS vascular malformations.

Jugular Venous Reflux Can Mimic Posterior Fossa Dural Arteriovenous Fistulas on MRI-MRA.

Dural arteriovenous fistulas (DAVFs) are high-flow acquired shunts that can carry high risk of intracranial hemorrhage. Because DAVFs can often be managed by endovascular means, early and accurate diagnosis can markedly improve patient morbidity. Time-of-flight and arterial spin-labeling MRA have increased the diagnostic utility of MRI for DAVF by showing hemodynamic rather than anatomic evidence of shunting. The purpose of this article is to describe the cases of seven patients who had co-localization of arterial spin-labeling signal intensity and time-of-flight flow-related enhancement in the left skull base, resulting in a misdiagnosis of DAVF and a recommendation for catheter angiography by the interpreting radiologist. Benign jugular venous reflux is identified as a common mechanism in each case, and the physiology behind this imaging pitfall is described. An algorithmic diagnostic approach to differentiating physiologic venous reflux from true posterior skull base DAVFs is presented.

4D Digital Subtraction Angiography for the Temporal Flow Visualization of Intracranial Aneurysms and Vascular Malformations.

PURPOSE: To assess the benefit and radiation dose of four-dimensional (4D) digital subtraction angiography (DSA) - a time resolved three-dimensional (3D) DSA application - to evaluate the flow and architecture of aneurysms and vascular malformations. METHODS: All patients with cerebrovascular disease were considered who underwent 4D-DSA at our institution between January 2015 and February 2016. For the aneurysm patients, we evaluated the image quality in terms of the visualization of contrast flow in the aneurysm on a 3-point scale (excellent, fair and poor). Interrater agreement between two raters was estimated using Cohen's Kappa statistics. For the shunt disease patients, the additional information obtained from the 4D-DSA was described for each disease. The median radiation dose and volume of contrast medium required for the acquisitions were estimated. RESULTS: A total of 173 patients underwent 4D-DSA; 126 intracranial aneurysms, 10 arteriovenous malformations (AVM), 15 dural arteriovenous fistula (dAVF) and 22 other diseases. For aneurysm patients, excellent and fair visualization of the intra-aneurysmal flow was observed in 27.7%, 72.3%, and excellent (κ = 0.9) agreement between the raters was found. For AVM and dAVF patients, 4D-DSA clarified the complex vasculature by viewing the discrete time phase of contrast filling. Median radiation dose for intracranial lesions was 79.6 mGy for 6s 4D-DSA, and 175 mGy for 12s 4D-DSA. The median amount of contrast medium used was 18.0 ml for 6s 4D-DSA and 21.0 ml for 12s 4D-DSA. CONCLUSIONS: 4D-DSA provided additional information regarding intra-aneurysmal flow and contributed to detect different component of nidus or shunt points.

Endovascular Treatment of Intracranial Dural Arteriovenous Fistulas: A German Single-Center Experience.

BACKGROUND AND PURPOSE: Intracranial dural arteriovenous fistulas (DAVFs) are abnormal shunts between dural arteries and dural venous sinus or cortical veins. We report our experience with endovascular therapy of primary complex DAVFs using modern embolic agents. METHODS: This is a retrospective analysis of patients with DAVFs treated between 2015 and 2019. Patient demographics and technical aspects including the use of embolic agent, access to the fistula, number of treatments, occlusion rates, and complications were addressed. Angiographic treatment success was defined as complete occlusion (CO) of the DAVF. RESULTS: Fifty patients were treated endovascularly. Median age was 61 years and 66% were men. The most common symptom was pulsatile tinnitus in 17 patients (34%). The most frequent location of the DAVF was the transverse-sigmoid sinus (40%). Thirty-six fistulas (72%) had cortical venous reflux. Nonadhesive and adhesive liquid agents were used in 92% as a single material or in combination. CO was achieved in 48 patients (96%). In 28 individuals (56%), only 1 procedure was necessary. Nonadhesive liquid agents were exclusively used in 14 patients (28%) with CO attained in every case. For CO of tentorial DAVFs, multiple sessions were more often required than at the other locations (55 vs. 14%, p = 0.0051). Among 93 procedures, the overall complication rate was 3%. The procedure-related mortality rate was 0%. CONCLUSION: Endovascular treatment of intracranial DAVFs is feasible, safe, and effective with high rates of CO. In more than half of the patients, the DAVF was completely occluded after a single procedure. However, in tentorial DAVFs, multiple sessions were more often required.

Evolution of Developmental Venous Anomalies in the Setting of a Torcular Dural Arteriovenous Fistula and Cerebrofacial Venous Metameric Syndrome.

BACKGROUND: We describe evolution of a developmental venous anomaly (DVA) over time in a patient with a complex intracranial vascular malformation. CASE DESCRIPTION: A 26-year-old male patient initially presented with a scalp vascular malformation and was later diagnosed to have a torcular dural arteriovenous fistula resembling a dural sinus malformation. The dural fistula increased in size over 4 years. The dural fistula also was associated with multiple complex developmental venous anomalies draining the bilateral cerebral hemispheres and cerebellum. The DVA was only faintly demonstrated on the baseline magnetic resonance imaging but appeared to increase in size and extent over time as the dural arteriovenous fistula developed more aggressive angioarchitecture features. In addition to the evolution manifestation of the DVAs, the patient developed multiple de novo cavernous malformations in the venous radicles of the DVA. Increased venous hypertension in the superficial venous system from the dural fistula likely resulted in growth of the DVAs, as they served as the primary means of venous drainage for the bilateral cerebral hemispheres. The patient also had reopening of the persistent falcine sinus, which was not present at baseline. CONCLUSIONS: This would be the first reported case of growth or evolution of a DVA in association with a dural arteriovenous fistula in an adult patient and highlights the dynamic nature of both the medullary venous and dural venous sinuses of the cerebral venous system, even into adulthood.

Differentiation of dural arteriovenous fistula from reflux venous flow on 3D TOF-MR angiography: identifying asymmetric enlargement of external carotid artery branches.

AIM: To differentiate dural arteriovenous fistula (DAVF) from reflux venous flow (RVF) by assessment of asymmetric enlargement of external carotid artery (ECA) branches on three-dimensional (3D) time-of-flight magnetic resonance angiography (TOF-MRA). MATERIALS AND METHODS: Nineteen patients with DAVF and 27 patients with RVF were included from September 2007 to April 2019. The locations of DAVF were the cavernous (n=9) and sigmoid-transverse (n=6) sinuses, and the jugular (n=3) and sphenoparietal (n=1) veins. Two radiologists visually assessed asymmetric enlargement of the ascending pharyngeal artery (ASP), middle meningeal artery (MMA), accessory meningeal artery (AMA), artery of the foramen rotundum, and occipital artery. MMA size was also measured quantitatively. RESULTS: More frequent asymmetric enlargement of the ASP and MMA were seen in DAVF than in RVF (17 of 19 versus 3 of 27 for ASP; 16 of 19 versus 1 of 27 for MMA, p<0.01). The AMA, artery of the foramen rotundum, and occipital arteries were not visualised in RVF patients. The ipsilateral MMA size was significantly larger in DAVF than in RVF (2.14 versus 0.64 mm2, p< 0.01). The diagnostic performance of ipsilateral MMA size for DAVF was good, with an area under the receiver operating characteristic curve of 0.89, with a sensitivity of 84.2% and a specificity of 81.5% at a cut-off of 1.08 mm2. CONCLUSIONS: DAVF showed significantly larger ECA branches than RVF on 3D TOF-MRA. Therefore, thorough evaluation of ECA branches could help to differentiate DAVF from RVF.

Unusual cortical symptoms of dural arteriovenous fistula mimicking transient ischemic attack.

The clinical presentation of dural arteriovenous fistula (DAVF) can vary. A 47-year-old man complained of transient difficulty playing badminton and speech disturbance for 10 minutes. His symptoms were suspected to be visuomotor coordination deficit similar to optic ataxia and anomic aphasia. Magnetic resonance imaging and angiography revealed vasogenic edema and perfusion delay in the left temporo-occipital area and an abnormal connection between the left occipital artery and transverse sinus. Transverse sinus DAVF was diagnosed by conventional cerebral angiography. We believe that this is the unique case of DAVF manifested as visuomotor coordination deficit suspected optic ataxia and anomic aphasia.

Updates in the management of cranial dural arteriovenous fistula.

Dural arteriovenous fistula (dAVF) accounts for approximately 10% of all intracranial vascular malformations. While they can be benign lesions, the presence of retrograde venous drainage and cortical venous reflux makes the natural course of these lesions aggressive high risk of haemorrhage, neurological injury and mortality. Endovascular treatment is often the first line of treatment for dAVF. Both transarterial and transvenous approaches are used to cure dAVF. The selection of treatment approach depends on the angioarchitecture of the dAVF, the location, the direction of venous flow. Surgery and, to a lesser extent, stereotactic radiosurgery are used when endovascular approaches are impossible or unsuccessful.

Basilar artery fenestration and ischemic stroke: An unclear causal relationship.

Basilar artery fenestration is the second most commonly observed fenestration of the cerebral arteries. In addition to our case, we reviewed the clinical, imaging findings, treatment, and prognosis of 9 other reported cases. Patients' mean age was 45.1 years. Half of them had cardiovascular risk factors. Mean time to diagnosis was 9.4 days. The main symptoms were right hemiparesis and dysarthria. Basilar artery fenestration was found in all patients, as well as ours, in addition to a thrombus, found in 2 cases. One patient was treated by IV thrombolysis and thrombectomy. In other cases, antiplatelet drugs or anticoagulants were used. A favorable outcome was observed in most cases with one reported death.

Steroid-Associated Acute Clinical Worsening and Poor Outcome in Patients With Spinal Dural Arteriovenous Fistulas: A Prospective Cohort Study.

STUDY DESIGN: A prospective cohort study. OBJECTIVE: We conducted a prospective cohort study to identify the association between steroids and clinical worsening and compare outcomes between patients with and without preoperative steroid administration. SUMMARY OF BACKGROUND DATA: Patients with spinal dural arteriovenous fistulas (SDAVFs) often were misdiagnosed and treated with steroids which led to acute worsening. METHODS: Patients with angiographically confirmed SDAVFs were recruited consecutively between March 2013 and December 2014 in two referral centers. We reviewed the history of all the patients to identify those patients who were treated with steroids before exclusion of the fistulas. Modified Aminoff & Logue scale (mALS) was used to evaluate the spinal cord function at different time points: before and after steroid administration, before operation, and at 1-year follow-up. Paired t tests were used to assess the mALS of patients with steroid administration at different time points. Unpaired t tests and Pearson chi-square test were used to assess differences between patients with and without steroid administration. RESULTS: Eighteen patients with (18.2%) and 81 patients without (81.8%) steroid administration were included in this study. At baseline, there were no difference between both patient groups, in regards to age, sex, duration, location of fistula, treatment, and preoperative mALS. Patients without steroid administration, however, had statistically significant better outcome according to their mALS at 1-year follow-up (P < 0.05). CONCLUSION: Steroid administration can induce acute clinical worsening in patients with SDAVFs that may persist despite successful obliteration of the fistula and should thus be avoided. LEVEL OF EVIDENCE: 3.

Medullary Compression Due to Ectatic Vertebral Artery-Case Report and Review of Literature.

Medullary compression syndrome due to anomalous course of blood vessels is a rare disease most commonly seen in the adult population. The offending vessels causing this syndrome are mostly posterior inferior cerebellar artery or the vertebral artery. The symptoms of this syndrome vary from most common hypertension to various other neurologic deficits like hemiplegia, dysesthesia, and dysarthria. Intractable dizziness is a rare symptom of this disease. The definite management plan for this disease is microvascular decompression. We present our case of medullary compression syndrome which manifested as intractable dizziness. We describe our experience in the management of this patient as well as present a review of literature of this rare disease.

Repaired coarctation of the aorta, persistent arterial hypertension and the selfish brain.

BACKGROUND: It has been estimated that 20-30% of repaired aortic coarctation (CoA) patients develop hypertension, with significant cardiovascular morbidity and mortality. Vertebral artery hypoplasia (VAH) with an incomplete posterior circle of Willis (ipCoW; VAH + ipCoW) is associated with increased cerebrovascular resistance before the onset of increased sympathetic nerve activity in borderline hypertensive humans, suggesting brainstem hypoperfusion may evoke hypertension to maintain cerebral blood flow: the "selfish brain" hypothesis. We now assess the "selfish brain" in hypertension post-CoA repair. METHODS: Time-of-flight cardiovascular magnetic resonance angiography from 127 repaired CoA patients (34 ± 14 years, 61% male, systolic blood pressure (SBP) 138 ± 19 mmHg, diastolic blood pressure (DBP) 76 ± 11 mmHg) was compared with 33 normotensive controls (42 ± 14 years, 48% male, SBP 124 ± 10 mmHg, DBP 76 ± 8 mmHg). VAH was defined as < 2 mm and ipCoW as hypoplasia of one or both posterior communicating arteries. RESULTS: VAH + ipCoW was more prevalent in repaired CoA than controls (odds ratio: 5.8 [1.6-20.8], p = 0.007), after controlling for age, sex and body mass index (BMI). VAH + ipCoW was an independent predictor of hypertension (odds ratio: 2.5 [1.2-5.2], p = 0.017), after controlling for age, gender and BMI. Repaired CoA subjects with VAH + ipCoW were more likely to have difficult to treat hypertension (odds ratio: 3.3 [1.01-10.7], p = 0.049). Neither age at time of CoA repair nor any specific repair type were significant predictors of VAH + ipCoW in univariate regression analysis. CONCLUSIONS: VAH + ipCoW predicts arterial hypertension and difficult to treat hypertension in repaired CoA. It is unrelated to age at time of repair or repair type. CoA appears to be a marker of wider congenital cerebrovascular problems. Understanding the "selfish brain" in post-CoA repair may help guide management. JOURNAL SUBJECT CODES: High Blood Pressure; Hypertension; Magnetic Resonance Imaging (MRI); Cardiovascular Surgery; Cerebrovascular Malformations.

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.

Imaging Classification of Internal Carotid Artery Hypoplasia Based on Distal Ophthalmic Segment Occlusion.

BACKGROUND: Internal carotid artery (ICA) hypoplasia (ICAH) is rare. The classification of ICAH is largely unclear. The aim of the study is to propose a new imaging classification for ICAH based on the occlusion of the distal ophthalmic segment and discuss the clinical and radiological differences between the different types. MATERIALS AND METHODS: This was a retrospective study of patients with congenital ICAH diagnosed at the Department of Neurology of the China-Japan Friendship Hospital between June 2011 and June 2016. The patients underwent temporal bone computed tomography (CT), brain CT, cranial magnetic resonance imaging, transcranial Doppler, and head and neck CT angiography. RESULTS: A total of 20 ICAH patients were divided into the distal occlusion (12 cases; 60%) and nondistal occlusion (8 cases; 40%) types based on whether the distal ophthalmic segment was occluded. The frequencies of collateral circulation from the circle of Willis (P = .01) and dilated cerebrovascular lesions (P = .001) in the distal occlusion type was higher than in the nondistal occlusion type. Five (25%) patients developed adverse cerebrovascular events during followup: 3 ischemic cases were of the nondistal occlusion type, and 2 cases with subarachnoid hemorrhage were of the distal occlusion type. CONCLUSIONS: A novel classification of ICAH was revealed based on the occlusion of the distal ophthalmic segment. The 2 types may show differences in collateral circulation patterns, coexisting cerebrovascular abnormalities, and potential clinical outcomes.

Clinical and neuroimaging findings of spinal dural arteriovenous fistulas: How to avoid misdiagnosis of this disease.

OBJECTIVE: Spinal dural arteriovenous fistulas (DAVFs) are rare but can cause serious gait and micturition disturbances. Delays in diagnosis and treatment result in poor clinical outcomes; however, the process of misdiagnosis is unknown. METHODS: Forty consecutive patients were retrospectively analyzed. RESULTS: Thirty-one patients (78%) were initially misdiagnosed with lumbar spinal stenosis or other diseases, mostly by orthopedic surgeons, even though most patients (85%) had specific symptoms or characteristic neuroimaging findings of spinal DAVFs: they often presented with spastic gait (thoracic myelopathy), progressive ascending numbness that begins in the distal lower extremities (epicous syndrome), and urinary tract symptoms (conus medullaris syndrome); initial lumbar MRI showed T2 signal change in the conus medullaris and vascular flow voids around the cord. The median time from onset to treatment was longer in patients with a misdiagnosis than in those with the correct diagnosis (11 vs 4 months). In all patients, the fistula was completely obliterated by the direct microsurgical procedure; however, patients with a misdiagnosis had developed additional disabilities by the time a correct diagnosis was made (Aminoff-Logue gait grade of 3.6 ± 1.4 vs 2.1 ± 1.5 p = 0.013), and achieved markedly smaller improvements after the treatment (Aminoff-Logue gait grade of 3.0 ± 1.6 vs 1.1 ± 1.5, p = 0.006) than those with the correct diagnosis of spinal DAVFs. CONCLUSIONS: When common spinal stenosis fail to explain the symptoms such as thoracic myelopathy, epiconus syndrome, and conus medullaris syndrome, the possibility of spinal DAVFs should be considered. If lumbar MRI shows conus medullaris lesions, thoracic MRI should be performed to confirm the diagnosis.