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Background Cognitive decline is a non-hemorrhagic, major complication of intracranial dural arteriovenous fistula (DAVF), thought to be primarily related to venous hypertension. However, imaging features to predict cognitive decline are scanty in the literature. Purpose To evaluate functional connectivity (FC) changes of resting-state networks (RSNs) in DAVF before and after treatment and its relation to cognitive impairment. Study type Prospective. Subjects DAVF subjects were screened for inclusion. Pre-embolization (N = 33, mean age 45.9 years, 29 males), 1 month post-embolization (N = 20, mean age 42.7 years, 19 males), and healthy controls (HC, N = 33, mean age 45.09 years, 27 males). Field strength/sequence 3.0 T, resting-state functional magnetic resonance imaging (MRI), three-dimensional (3D) T1, T2 fast spin echo (FSE), diffusion weighted imaging (DWI), susceptibility weighted imaging (SWI), fluid-attenuated inversion recovery, and time of flight. Assessment Data quality assessment was performed. FC analysis was done using group independent component analysis (ICA) and seed to voxel analysis. Neuropsychology (NP) scores of patients were compared with HC and correlated with FC changes. Statistical tests Voxel-wise parametric T-statistics for F-test was executed in FC analysis (p-FDR corrected Results Both RSNs analysis methods showed reduced FC at the precuneus-posterior cingulate cortex (PC-PCC) of default mode network (DMN), anterior cingulate cortex (ACC) of the salience network (SN), and possible compensatory increased connectivity at the frontoparietal (FPN) and dorsal attention (DAN) networks. DAVF with low NP scores showed reduced FC at DMN and SN and minimal to absent connectivity at FPN and DAN. At post-embolization 1-month follow-up, improvement in FC at PC-PCC of DMN and ACC of SN were noted. Data conclusion RS-fMRI in DAVF displayed FC changes that may be related to cognitive decline and its subsequent reversibility after treatment. FC changes at DMN, SN, FPN, and DAN were linked to cognitive decline and the corresponding NP scores. Level of evidence 2 TECHNICAL EFFICACY: Stage 2.

Background The symptoms of dural arteriovenous fistula (dAVF) vary according to the location of the fistula and the pattern of venous drainage. Here, we report the case of a patient with a dAVF-induced glossopharyngeal neuralgia. Case description We report a case of a patient with right glossopharyngeal neuralgia caused by a posterior condylar canal (PCC) dAVF. The glossopharyngeal neuralgia was accompanied by persistent tinnitus and repetitive right side otalgia, as well as ipsilateral shoulder and throat pain, lasting for about 30 s. However, there were no specific findings on otoscopic examination. Cranial magnetic resonance imaging was performed to determine the cause of the symptoms, and a right PCC dAVF was observed. The dAVF was successfully obliterated using transvenous coil embolization. After embolization, the patient's symptoms were completely resolved. Conclusion Although glossopharyngeal neuralgia caused by dAVF is rare, it can present due to intracranial lesions located adjacent to the glossopharyngeal nerve or vagus nerve. Brain MRI is therefore required to identify secondary causes in all patients with glossopharyngeal neuralgia.

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.

Paediatric intracranial vascular malformations are rare and different from adult ones in vascular anatomy, pathophysiology, and symptoms. Their impact on the brain and their symptoms will differ in the antenatal period, in neonates, infants, and children. Clinical presentation includes seizures, focal neurological deficit, haemorrhage, congestive heart failure, hydrovenous disorder, and developmental delays. These malformations are thus associated with a poor prognosis if left untreated. Therefore, aggressive management is generally recommended and must be performed by a multidisciplinary team with extensive experience. Endovascular treatment is the first-choice treatment for most paediatric intracranial vascular malformations. Indication and timing for treatment should be decided on the basis of a careful assessment of neurological symptoms, growth and development, cardiac and other systemic manifestations, and imaging of the malformation and the brain tissue. WHAT THIS PAPER ADDS: Paediatric intracranial vascular malformations are rare, but their prognosis is poor if left untreated. Improved clinical, anatomical, and pathophysiological understanding of these complex lesions has improved prognosis.

BACKGROUND AND PURPOSE There is a relative paucity of radiology literature on spinal cord cavernous malformations (SCMs). We hypothesized that the classic MRI features of cavernous malformations are not highly prevalent. The purpose was to review MR imaging findings of SCMs with a focus on prevalence of hemorrhagic features, including adjacent intramedullary hemorrhage at initial presentation. METHODS In this single-institution study, 78 SCMs in 76 patients diagnosed on imaging/pathology were retrospectively evaluated for size, location, cord expansion, exophytic component, popcorn morphology, signal characteristics, including T1 weighted (T1w) and T2w rims, blood-fluid levels, and adjacent intramedullary hemorrhage. Intramedullary hemorrhage was defined as centrally and/or eccentrically located linear or flame shaped non-edematous signal abnormality extending longitudinally away from SCMs, distinct from lesional rim. RESULTS Sixty-three percent (49/78) of SCMs measured

Variations in the venous drainage of the central nervous system can have imaging and clinical findings that mimic pathology, presenting a challenge for neuroimagers and clinicians. Patients with these variants may undergo unnecessary testing, and patients with pathology may receive delayed diagnoses because of overlap with benign findings. Consequently, the accurate identification of venous variations on cross-sectional imaging and angiography and their potential causes are critical for differentiating benign imaging variants from potential pathologic processes requiring further evaluation. For example, in the epidural space, benign dilation of the epidural venous plexus may be mistaken for evidence of a fistula, abscess, or metastasis. Hypoplasia of a dural venous sinus or an arachnoid granulation may mimic venous sinus thrombosis. The superior ophthalmic vein may demonstrate benign dilation in intubated patients, mimicking thrombosis, increased intracranial pressure, orbital varix, inflammatory pseudotumor, or other conditions. Furthermore, certain venous variations, such as the occipital sinus or emissary veins, may complicate surgery or herald pathology and should be reported. In addition, some supposedly benign variations, such as the developmental venous anomaly, can be complicated by pathology. The objective of this review article is to provide a descriptive and pictorial review of common anatomic and physiologic variations in the venous drainage system of the brain, spine, and orbits that can mimic pathology. Neuroimaging findings of related pathologies and differences in clinical presentations will also be discussed to assist in the approach to differential diagnosis.

Vascular anomalies are separated into vascular tumours and vascular malformations. Vascu- lar malformations are named according to the affected type of vessels, that is venous, capillary, arteriovenous or lymphatic malformations. Up to now, sclerotherapy, embolisation and/or surgery are the treatments of choice, yet they do not often offer a curative treatment. Thus, there is an important need to develop novel disease-specific therapeutic approaches. Inherited forms of vascular malformations led to the identification of several genes that are mutated encoding dysfunctional proteins. Demonstration that tissular second hits are com- monly involved in inherited forms to explain development of lesions led to study somatic muta- tions in sporadically occurring forms. Since the primary discovery demonstrating that venous malformations are due to somatic mutations in TIE2/TEK, most types of vascular anomalies now have a known genetic cause. Thereby, the signalling pathways involved have been unrav- elled, leading to a better understanding of the aetiopathogenesis of vascular anomalies. As – like in cancers – the RAS/MAPK/ERK and the PI3K/AKT/mTOR signalling are enhanced in most vascular anomalies, treatment with cancer drugs interfering with these pathways could represent novel treatment options.

BACKGROUND AND PURPOSE To determine the interobserver reliability of a newly proposed classification scheme for angiographic classification of spinal vascular malformations including arteriovenous fistulas (AVFs) and arteriovenous malformations (AVMs). METHOD A study was performed done in which 1–2 representative angiographic images of 26 spinal AVFs and/or AVMs were independently classified by five fellows in the ACGME accredited Endovascular Surgical Neuroradiology (ESN) program and two external interventionalists in the absence of any other clinical or imaging data. From these observations the interobserver reliability for each category and the overall scheme were determined in terms of the median weighted kappa statistic. RESULTS The overall interobserver reliability for the new classification scheme was a Kappa of 0.53 (Z= 21.3, P=

Background: Freezing of gait (FOG) is defined as anepisodic inability to generate effective stepping in theabsence of any known cause other than parkinsonismor high-level gait disorders.Methods: We present a 59-year-old male with acute,progressive episodes of FOG. Imaging studies revealeda dural arteriovenous fistula (DAVF) associated withedema of the globus pallidus interna (GPi). Cerebralangiography confirmed the diagnosis of DAVF anddemonstrated an occluded straight sinus and a retro-grade blood flow of deep cerebral veins.Results: After endovascular closure of the DAVF, amajor improvement of FOG was observed concomitantwith striking near resolution of GPi congestion.Conclusions: This reversal of the clinical course, cor-related with changes in imaging studies, suggests amajor role of the GPi in the pathology of FOG. V C 2012Movement Disorder Society Key Words: dural arteriovenous fistula; freezing ofgait; globus pallidus Freezing of gait (FOG), defined as an episodic inabilityto generate effective stepping in the absence of anyknown cause other than parkinsonism or high-level gaitdisorders

Superficial temporal vein catheterization was used to embolize a dural arteriovenous malformation of the cavernous sinus in a 44-year-old woman. The pertinent venous anatomy is discussed. This route may provide crucial access to the cavernous sinus when conventional approaches such as the Inferior petrosal sinus catheterization are difficult.

Background Familial cerebral cavernous malformations (FCCM) are vascular malformations inherited as an autosomal-dominant condition. Three genes (KRIT1/CCM1, MGC4607/CCM2, PDCD10/CCM3) have been identified so far. Extra-neurological manifestations include retinal and cutaneous vascular malformations. The cutaneous vascular malformation, which had been more specifically associated with FCCM, is hyperkeratotic cutaneous capillary venous malformation (HCCVM). Objectives To define the frequency of cutaneous vascular malformations in patients with FCCM, to precise their different phenotypes, and to study the association of each cutaneous vascular malformation subtype with the different three mutated CCM genes. Methods Dermatological inquiry was systematically performed in a large series of consecutive FCCM patients. Cutaneous biopsies were reviewed when available. Cutaneous vascular malformations classification was based on predominant anomalous channels, using the current International Society for the Study of Vascular Anomalies classification. Molecular screening of CCM genes was performed. Results Four hundred seventeen consecutive FCCM patients from 182 unrelated families were included. 38 patients (9%) from 25 different families had cutaneous vascular malformations. In these 38 patients, cutaneous vascular malformations were classified as follows: 13 capillary malformations (CM), 15 HCCVM, 8 venous malformations (VM) and 2 unclassified lesions. All patients (92%), but one with CM had a KRIT1/CCM1 mutation. The last patient had no detectable mutation. All of the 15 patients with HCCVM had a KRIT1/CCM1 mutation; 86.7% of cutaneous vascular malformation patients (33 of 38) had a KRIT1/CCM1 mutation. Conclusion Cutaneous vascular malformations are seen in 9% of FCCM patients. Three distinct major cutaneous vascular malformations phenotypes were identified: HCCVM (39%), CM (34%) and VM (21%). CCM1 is the most frequently mutated gene in cutaneous vascular malformations-FCCM patients.

BACKGROUND AND PURPOSE The treatment of a dural arteriovenous fistula (DAVF) can involve surgery, radiosurgery, or endovascular embolization. New embolization techniques and agents have expanded the role of endovascular treatment. METHODS We report the endovascular embolization with Onyx-18 of a DAVF fed by the posterior auricular artery. RESULTS The DAVF was successfully embolized; however, the patient's ear developed ischemic necrosis of the superolateral pinna. CONCLUSIONS Onyx-18 can provide high rates of success in the treatment of DAVFs in well-selected patients. Risks of end organ ischemia must be considered during endovascular embolization and when counseling patients regarding procedural risk.

Summary The cavernous sinus dural arteriovenous fistulas of three patients were successfully embolised by using Onyx (Onyx Liquid Embolic System, MTI, Irvine, CA, USA) as the sole embolic agent, through direct percutaneous transorbital punctures of the cavernous sinuses. Our early experience suggests that this direct approach, coupled with the unique physical properties of Onyx, is a safe and effective alternative to treat cavernous sinus dural arteriovenous fistulas when the conventional transvenous routes are inaccessible.

In this article, the neuroradiological evaluation of traumatic brain injury is reviewed. Different imaging strategies in the assessment of traumatic brain injury are initially discussed, and this is followed by a review of the imaging characteristics of both primary and secondary brain injuries. Computed tomography remains the modality of choice for the initial assessment of acute head injury because it is fast, widely available, and highly accurate in the detection of skull fractures and acute intracranial hemorrhage. Magnetic resonance imaging is recommended for patients with acute traumatic brain injury when the neurological findings are unexplained by computed tomography. Magnetic resonance imaging is also the modality of choice for the evaluation of subacute or chronic traumatic brain injury. Mild traumatic brain injury continues to be difficult to diagnose with current imaging technology. Advanced magnetic resonance techniques, such as diffusion-weighted imaging, magnetic resonance spectroscopy, and magnetization transfer imaging, can improve the identification of traumatic brain injury, especially in the case of mild traumatic brain injury. Further research is needed for other advanced imaging methods such as magnetic source imaging, single photon emission tomography, and positron emission tomography.

Objectives: The objective of this study is to determine the sensitivity of computed tomography angiography (CTA) and magnetic resonance angiography (MRA) compared to the gold standard, conventional carotid angiography, in the diagnosis of a dural arteriovenous fistula (DAVF). Further objectives include identifying outcomes of treatment and complications as a function of pretreatment radiologic classification. Study Design: This is a retrospective review of adults diagnosed with a DAVF between 1990 and 2006. Methods: Data collected included age, gender, presenting clinical symptoms, diagnostic imaging modalities (conventional angiography, CTA, and/or MRA), interventions (self-compression of artery, embolization, craniotomy, or a combination of these), results (symptoms resolved, improved, or same), and complications (embolic or surgical). All DAVFs were classified according to the Cognard classification. Results: There were a total of 46 patients with 51 fistulas, of whom 42 patients (46 fistulas) had complete follow-up data. Using conventional angiography as the gold standard, CTA had a sensitivity of 15.4%, whereas MRA had a sensitivity of 50%. Embolization (either single or multiple treatments) was the most common treatment, being done in 36/46 (78%) fistulas. Presenting symptoms were resolved or improved in 45/46 fistulas (97.8%) regardless of treatment or Cognard classification. There were complications in 12/46 (26%) patients, most of which were transient and resolved without permanent sequelae. Conclusions: A DAVF is a vascular lesion that is best diagnosed with conventional angiography, but can often be found with MRA. Treatment with endovascular embolization is effective and has few significant complications. Laryngoscope, 119:293–297, 2009

We describe three patients with progressive myelopathy, in whom autopsy revealed spinal cord pathology compatible with that of venous congestive myelopathy (VCM) associated with dural arteriovenous fistula (AVF), formerly known as angiodysgenetic necrotizing myelopathy (Foix-Alajournine syndrome). In these three patients, common symptoms were gait disturbance and sensory disturbance of the extremities, and these symptoms slowly worsened. The clinical diagnoses varied and included spinal cord intramedullary tumor, cervical spondylosis and multiple sclerosis. At autopsy, all the patients showed enlarged, tortuous venous vessels on the dorsal surfaces of the spinal cord at the affected levels. In the affected spinal cord parenchyma, necrotic lesions manifested by various degrees of neuronal loss and gliosis, with increased numbers of hyalinized vessels, were evident. The presence or absence of associated spinal dural AVF could not be identified histopathologically. Even with the help of modern neurological examination methods, early and accurate clinical diagnosis of VCM is sometimes difficult. When encountering patients with progressive myelopathy, VCM, although recognized as rare, should be considered as an important differential diagnosis.

We report a rapidly progressive myelopathy in a 74-year-old Japanese man who was admitted to our hospital with a 4-month history of progressive gait disturbance and died of pneumonia followed by respiratory failure on the 22nd day of admission. During the course of his illness, magnetic resonance imaging (MRI) revealed intramedullary lesions with edematous swelling from the medulla oblongata to the spinal cord at the level of the fourth vertebra. After administration of contrast medium, the ventral portion of the lesion was mildly and irregularly enhanced and a dilated vessel was recognized along the ventral surface of the upper cervical cord. At autopsy, ischemic changes were observed in the upper-to-middle cervical cord segments, with so-called arterialized veins in the subarachnoid space. No neoplastic lesions were found within or outside the brain and spinal cord. These pathological findings were essentially those of venous congestive myelopathy (VCM) associated with dural arteriovenous fistulae (AVF), formerly known as Foix-Alajouanine syndrome. VCM associated with dural AVF, which is now considered to be treatable in the early stages, is rare found in the cervical spinal cord. The present autopsy case, with MRI findings, provides further information that might be useful for recognition and diagnosis.

Objectives To elucidate a part of the prenatal natural history of dural sinus malformation of the posterior fossa. Methods Ultrasound and magnetic resonance imaging were performed from 31 to 32 weeks' gestation. Results We observed the progressive development of a thrombus that was visible as an expanding hyperechoic round area within a cystic mass of the posterior fossa. It was characterized, as expected for a vascular malformation, by the presence of blood flow into the aneurismal cavity. Color doppler identified superior sagittal and straight sinuses, and distinguished that their flow continued into the dilated torcular. Prenatal magnetic resonance imaging confirmed an arteriovenous malformation involving the dural sinus. Conclusion The vascular malformation had a fixed volume and preceded the thrombosis, which formed within several days. The present case is the first report with all the prenatal sonographic features of this condition. Copyright © 2007 John Wiley & Sons, Ltd.

The association consisting of posterior fossal malformations, cervicofacial, hemangiomas, arterial anomalies, cardiac defects, eye anomalies, and sternal clefting, or supraumbilical raphe, refers to the occurrence of congenital structural and vascular anomalies in the presence of a facial hemangioma. We report a patient with this association, growth retardation, and developmental delay who was found to have a partially empty sella turcica, central hypothyroidism, and growth hormone deficiency. Endocrinologic evaluation should be considered in any infant with this association.

Parkinsonism associated with dural arteriovenous fistula (DAVF) has been described rarely; however, isolated parkinsonism as the presenting symptom of DAVF has not been reported. Here, we describe a patient with DAVF showing reversible isolated parkinsonism after embolization, which was well correlated with perfusion status of basal ganglia, suggesting that a perfusion defect was responsible for the pathogenesis of the parkinsonism in our DAVF patient.

Congenital hemihypertrophy is an uncommon condition of unknown etiology characterized by unilateral overgrowth of part or all of one side of the body. Hemihypertrophy is known to be associated with certain childhood tumors, most notably Wilms tumor (or nephroblastoma), and for this reason infants with hemihypertrophy are often followed with serial abdominal ultrasounds. Klippel-Trénaunay syndrome (KTS) is the triad of port-wine stain, venous varicosities, and soft tissue and/or bony hypertrophy. Children with KTS typically have localized rather than generalized hemihypertrophy, but occasionally the hypertrophy is more extensive than the vascular anomaly itself. Information is lacking about whether hemihypertrophy in this setting can also be a risk factor for Wilms tumor. We systematically reviewed the medical literature to determine whether well-documented cases of Wilms tumor in the setting of both hemihypertrophy and vascular anomalies have been described, and if found, whether the association was sufficiently frequent that routine screening for Wilms tumor in this setting should be recommended. A review of case reports and case series in the pediatric population was undertaken using specific inclusion and exclusion criteria. We found 4 of 58 subjects with hemihypertrophy and Wilms tumor had a reported vascular anomaly, but in only one case was a clear-cut diagnosis of KTS confirmed. The relationship of the other three vascular anomalies reported was of uncertain significance. In conclusion, our review suggests that the risk of Wilms tumor in the setting of localized soft-tissue hypertrophy in conjunction with a vascular malformation is quite low. More extensive hemihypertrophy extending to body sites remote from the vascular malformation itself could have a higher risk of Wilms tumor, although the magnitude of this risk is uncertain. Our findings suggest that routine serial abdominal ultrasounds in patients with vascular malformations in association with localized soft-tissue hypertrophy are unwarranted.

Dural arteriovenous fistulas (DAVFs) at the cervicomedullary junction are rare and have a wide variation in presentation. We report a case of occipital neuralgia (ON) as a rare presenting symptom of cervicomedullary DAVF causing intramedullary hemorrhage at the C1 level. It is important to consider the underlying causes of ON, and precise neurological examinations and radiological evaluations are needed.

BACKGROUND AND PURPOSE The most common causes of parkinsonism are degenerative and irreversible. Structural causes of reversible parkinsonism are exceptionally rare, but have been reported in association with deep cerebral venous thrombosis and dural arteriovenous fistula (DAVF). We now report a case of parkinsonian syndrome reversed by treatment of a dural arteriovenous malformation. METHODS Case report with retrospective review of relevant clinical, imaging, and treatment data. RESULTS A 79-year-old man presented with complaints of a slowly progressive parkinsonian syndrome and tinnitus. Brain magnetic resonance imaging (MRI) showed numerous serpiginous flow voids highly suggestive of a vascular malformation. Cerebral angiography revealed a Cognard grade IIa + b left transverse sinus DAVF. The patient underwent combined endovascular and surgical treatment in three stages over a week's time, with total obliteration of the DAVF and complete normalization of his symptoms and neurological examination. CONCLUSION Although rare, DAVF should always be considered as a potential cause of progressive parkinsonism. This diagnosis is of great importance because of its potential reversibility.