Cerebral Vascular Malformations Research Articles

Krit1 loss-of-function increases TNF-α -induced apoptosis by inhibiting Notch1 in endothelial cells

Background: Cerebral cavernous malformations (CCMs) are common cerebral vascular malformations causing neurological deficits in younger individuals and stroke in middle age. CCM is frequently an inherited disease due to heterozygous mutations in one of three genes: CCM1 (KRIT1), CCM2, and CCM3 (PDCD10). These proteins control endothelial cells junctions, proliferation, migration and, therefore, angiogenesis.

Continuous spinal anesthesia for 1 cases of cesarean section after cerebral vascular malformation

Maintaining hemodynamic stability is the most important to the parturient woman who undergoing cesarean section after cerebral vascular malformation. Avoiding high blood pressure caused by intracranial deformity and vascular rupture again after healing, avoid low blood pressure leading to maternal cerebral blood flow insufficiency and fetal distress caused by decreased placental blood flow. Continuous spinal anesthesia(CSA) by microcatheter injection of drugs into the subarachnoid space, and can provide more stable hemodynamic status with rapid onset and perfect anesthetic effect in obstetric patients. Through this case, the particularity of caesarean section management and the advantages of CSA in patients with cerebral vascular malformation were introduced in this paper. Key words: Intracranial vascular malformation; Cesarean section; Continuous subarachnoid anesthesia; Hemodynamics

Lesiones Proliferativas Vasculares Cerebrales

Introducción: Las malformaciones vasculares cerebrales, tienen una incidencia del 6%, y son clasificadas de múltiples formas según topografía, morfología, y/o demografía. Con respecto a esta ultima división, dichas malformaciones se pueden clasificar en malformaciones de tipo no proliferativas o proliferativas. Las lesiones proliferativas se dividen en: Moya-Moya (M-M), angiopatia proliferativa cerebral (ACP), y hemangiomas.Materiales y Métodos: En primer lugar se presenta un caso clínico de un paciente que fue asistido en el Centro Endovascular Neurológico, donde se estudió y realizó el diagnostico de lesión proliferativa vascular, operándose para tratamiento de hemorragia intraventricular con hidrocefalia. A partir de este caso clínico ilustrativo se realizó una puesta al día sobre esta patología. Discusión: A La APC es una malformación rara, con una frecuencia del 3,4%, y el M-M tiene una incidencia mayor en Japón (3:100,000). Ambos tienen mayor frecuencia en el sexo femenino, pero la APC se presenta habitualmente a los 22 anos, mientras que el M-M tiene dos picos de presentación: uno a los 5 y otro a los 40 anos. Habitualmente este tipo de lesiones proliferativas se presentan clínicamente como accidentes cerebro vasculares (ACV) de causa isquémica. El estudio gold estándar para el diagnostico de estas malformaciones es la angiografía cerebral. Las APC son malformaciones de gran tamaño pudiendo ocupar un lóbulo o hemisferio entero, con innumerables vasos que se extienden entre neuronas y sustancia blanca normal. En la mayoría de los casos el tratamiento está dirigido para mejorar la hipoperfusión circulatoria con el fin de prevenir nuevos eventos isquémicos. Conclusión: Es importante poder diagnosticar este tipo de lesiones a tiempo, así como diferenciar la angiopatía proliferativa o Moya moya, de otro tipos de malformaciones, ya que tiene implicancias en el tratamiento y pronostico.

Etiologies of Non-Genetic Epilepsies of Adults Newly Diagnostic in Ouagadougou in Burkina Faso

Introduction: Epilepsy is a common disease in sub-Saharan Africa. The etiological diagnosis of epilepsies is still based on interrogation and clinical examination, due to a lack of means of complementary diagnostic investigations, electroencephalogram (EEG), computed tomography (CT) or magnetic resonance imaging (MRI). The aim of our study was to determine the etiological varieties of non-genetic epilepsies of adults, newly diagnosed in Ouagadougou, according to diagnostic, clinical, EEG and neuroradiological criteria (encephalic CT and/or MRI). Patients and methods: This was a prospective, cross-sectional, descriptive study that ran from September 1 to August 31, 2017, and included patients diagnosed with non-genetic epilepsy in adults, newly diagnosed in Ouagadougou, Burkina Faso. For each of the patients included in the study, the etiological diagnosis was based on the results of CT and /or brain MRI, in addition to the electroclinical criteria of non-genetic epilepsy. Results: We collected 137 patients; the average age was 41.8 ± 17.6 years, with 51.8% men (71 patients). The average age of onset of seizures was 34.3 years and the average duration of seizures was 7.3 years. Of all patients, 87.5% had focal seizures, 5.2% generalized seizures and 7.3% non-classifiable seizures. All of our patients had EEG and brain scan, only 11.8% had brain MRI. EEG was normal in 13.1%; there were inter-critical epileptic paroxysms in 86.9%. Localized atrophy associated with underlying parenchymal hypodensity with 48 cases (35%), porencephalic cavities with 16 cases (11.8%), circumscribed cortico-subcortical hypodense without contrast enhancement with 14 cases (10.2%), brain tumors with 12 cases (8.8%), were the most representative neuroradiological abnormalities. The structural causes and unknown causes were found respectively in 54% and in 46% of cases. CNS infections (16.8%), sequelae of stroke (11.7%), sequelae of cranioencephalic trauma (10.9%), brain tumors (8.7%), sequelae of Perinatal encephalopathy (4%) and cerebral vascular malformations (cavernoma) (1.5%) were the epileptogenic structural abnormalities found. Conclusion: Our results confirm the predominance of infectious and post-traumatic causes and the emergence of cerebrovascular causes in sub-Saharan Africa. Some epileptogenic lesions, such as certain brain tumors, focal cortical dysplasias, hippocampal sclerosis, have been under diagostized because of the poor availability and accessibility of cerebral MRI.

Spontaneous intracerebral hemorrhage.

Abstract Spontaneous intracranial hemorrhage (ICH) is the second most common case of stroke and has 30-day mortality estimated at 44% to 52%. ICH is most commonly caused by arterial hypertension, followed by cerebral amyloid angiopathy, vascular malformations, ruptured aneurysms, and tumors. The signs and symptoms of ICH depend on the location and size of the hemorrhage and have a gradual onset over a period of minutes to hours. Imaging studies are the primary diagnostic tool for detecting ICH, and CT is being considered the “gold standard” for identifying acute hemorrhage. A contrast-enhanced MRI or CT angiography is useful to detect underlying causative lesions such as tumors or vascular malformations. Management of ICH includes both medical and surgical treatment. Medical therapy aims to stabilize the patient and prevent hemorrhage extension by focusing on blood pressure control, reversal of coagulopathies, and treatment of intracranial hypertension. Surgical management was historically mainly limited to evacuation of the hematoma via open craniotomy, but minimally invasive surgical approaches are increasingly employed. These include but are not limited to catheter-based thrombolytic therapy, endoscopic evacuation, and ultrasonic lysis of the hematoma. This chapter details the etiology, pathophysiology, presentation, diagnosis, and treatment of spontaneous ICH.

Neurosurgical Issues in Pregnancy.

Although rare, intracranial hemorrhage due to rupture of cerebral vascular malformations or intracranial aneurysms during pregnancy is a potentially devastating and life-threatening disorder, posing a diagnostic and therapeutic challenge to a multidisciplinary team of neurosurgeons, neurologists, obstetricians, and anesthesiologists. Despite the significant risk of morbidity and mortality affecting both the mother and the unborn child, knowledge of the natural history, epidemiology, and appropriate management of cerebral vascular malformations and intracranial aneurysms in pregnant women is limited. Although emergent neurosurgical concerns usually outweigh obstetric considerations, and treatment of these disorders is generally similar in pregnant and nonpregnant women, any necessary and feasible modifications to protect the unborn child should be made. This article reviews the management of ruptured intracranial aneurysms and cerebral vascular malformations, including arteriovenous malformations, cavernous malformations, and moyamoya disease, in women during pregnancy, partus, and puerperium.

Prenatal diagnosis of concurrent facial and cerebral vascular malformation which caused congestive heart failure

Abstract Arteriovenous malformations (AVMs) are rarely reported antenatally. Most in utero diagnosis of vascular malformation is related to vein of Galen malformation (VGM). We describe a case of simultaneously diagnosed pial arteriovenous fistula (AVF) and facial vascular malformation in a 20 weeks old fetus. The dilated intracranial venous pouch appeared as a midline anechoic structure which was misdiagnosed as a VGM in her previous ultrasound exam. Another AVM was diagnosed in the same side of fetal face which fed by a branch of external carotid artery and communicated with the mentioned pial AVF. High output cardiac failure and hydrops were evident. To our knowledge this is the first report of prenatally detected combination of facial and cerebral vascular malformations at such as early pregnancy week.

Hemispheric developmental venous anomaly draining into a vein of Galen varix: a case report and literature review

BackgroundDevelopmental venous anomaly (DVA), previously known as “venous angioma”, is the most common cerebral vascular malformation, but hemispheric DVA is extremely rare.Case presentationIn this article we present a 21-year-old female with a large DVA draining the whole left hemisphere, combined with a vein of Galen varix and falcine sinus. To our knowledge, only 4 cases of hemispheric DVA have been reported before, however, none with a vein of Galen varix. This patient’s digital subtraction angiography also revealed double straight sinus with severe stenosis of one of the sinuses, which was considered to be the possible reason of the venous varix. The patient was managed conservatively, and her clinical status was stable for 1 year.ConclusionsBecause brain DVA drains the normal cerebral parenchyma and the hemorrhage risk is relatively low, hemispheric DVA is considered to be a benign anomaly, and conservative treatment is recommended.

Patients with Blunt Traumatic Brain Injury: A Role for Computed Tomography Angiography of the Head to Evaluate Nontraumatic Causes?

In the setting of trauma, the cause of intracranial hemorrhage (ICH) is frequently attributed to the physical, traumatic event. Caution should still be directed toward nontraumatic (or spontaneous) causes responsible for the trauma, such as hypertension, cerebral amyloid angiopathy, aneurysms, vascular malformation, and hemorrhagic infarcts. The role for immediate computed tomography angiography remains controversial to evaluate for nontraumatic causes. A systematic review of the available literature in the Medline PubMed database. In the available literature, only 12 patients with traumatic brain injury underwent computed tomography angiography of the head that either showed a vascular malformation and/or altered clinical management because of concerns of a vascular malformation. The ICH in 11 patients was attributed to rupture of a cerebral aneurysm; the other patient received a diagnostic angiogram that was negative. ICH in patients with traumatic brain injury seems to be vastly associated with the traumatic event. Only rare cases have been attributed to aneurysmal rupture. None has been associated with arteriovenous malformation. Nevertheless, clinical vigilance remains reasonable, especially in younger patients and those with hemorrhage within the subarachnoid cisterns or sylvian fissure.

Coexistence of anaplastic astrocytoma and arteriovenous malformation

Coexistence of astrocytoma with cerebral vascular malformations is unusual, especially if both lesions occur separately. Preoperative angiogram will help us to identify any coexisting arteriovenous malformation (AVM). This will in turn change the treatment strategy for astrocytoma. Preoperative embolization for AVM may be done prior to the glioma excision. There is increased risk of bleeding if AVMs are not preoperatively diagnosed. AVMs coexisting with astrocytomas are rare. We report a case of anaplastic astrocytoma coexisting with an acquired AVM.

Chapter 26 - Developmental venous anomalies

Developmental venous anomalies (DVAs) are relatively common lesions, present in up to 3% of the population. The defining characteristic of these lesions is the confluence of radially oriented veins into a single dilated venous channel. DVAs are also known as cerebral venous angiomas, cerebral venous malformations, and cerebral venous medullary malformations. They are the most common type of cerebral vascular malformation found on autopsy studies, and they are often encountered as incidental findings on neuroimaging studies. DVAs are congenital lesions thought to arise from aberrations that occur during venous development, but continue to provide the normal venous drainage to the cerebral territory in which they reside. Although the natural history of DVAs is benign, they may be associated with cavernous malformations or other vascular abnormalities, which can lead to hemorrhage in the vicinity of the DVA. Surgical or endovascular obliteration of DVAs carries a significant risk of venous infarction; thus, conservative management is the treatment of choice for patients with these lesions.

Role of endovascular embolization for trigeminal neuralgia related to cerebral vascular malformation

The objective of this article is to describe the trigeminal neuralgia related to cerebral vascular malformation that is rarely reported and the experience referring to endovascular treatment. A total of 10 patients who had cerebral vascular malformation (AVM and dAVF) in a single center presented with trigeminal neuralgia. Clinical and angiographic presentations as well as their clinical outcomes after embolization were reviewed. Of the 10 cases, seven dAVFs and three AVMs were detected. In contrast to the dilated feeding arteries, an ectasia of the draining vein that is adjacent to the root entry zone of the trigeminal nerve such as the petrosal vein and lateral mesencephalic vein has the major role in causing the trigeminal neuralgia. All of these patients had relief of facial pain after endovascular embolization during follow-up (mean 57.3 months, range 5 to 100 months). There were no permanent neurological deficits. Endovascular embolization is an effective method in treating trigeminal neuralgia related to cerebral vascular malformation.

The Many Faces of Cerebral Developmental Venous Anomaly and Its Mimicks: Spectrum of Imaging Findings.

Developmental venous anomalies (DVAs) are the most common cerebral vascular malformations and are usually found incidentally on neuroimaging studies. Despite the benign nature of DVAs, occasionally, they can be symptomatic. The objective of this article is to review the spectrum of imaging findings of DVAs on conventional and advanced imaging studies. In addition, neuroimaging findings of symptomatic DVAs as well as imaging mimicks will also be described to assist in the approach to differential diagnosis.

The H-ATOMIC Criteria for the Etiologic Classification of Patients with Intracerebral Hemorrhage.

Background and PurposeThere are no generally accepted criteria for the etiologic classification of intracerebral hemorrhage (ICH). For this reason, we have developed a set of etiologic criteria and have applied them to a large number of patients to determine their utility.MethodsThe H-ATOMIC classification includes 7 etiologic categories: Hypertension, cerebral Amyloid angiopathy, Tumour, Oral anticoagulants, vascular Malformation, Infrequent causes and Cryptogenic. For each category, the etiology is scored with three degrees of certainty: Possible(3), Probable(2) and Definite(1). Our aim was to perform a basic study consisting of neuroimaging, blood tests, and CT-angio when a numerical score (SICH) suggested an underlying structural abnormality. Combinations of >1 etiologic category for an individual patient were acceptable. The criteria were evaluated in a multicenter and prospective study of consecutive patients with spontaneous ICH.ResultsOur study included 439 patients (age 70.8 ± 14.5 years; 61.3% were men). A definite etiology was achieved in 176 (40.1% of the patients: Hypertension 28.2%, cerebral Amyloid angiopathy 0.2%, Tumour 0.2%, Oral anticoagulants 2.2%, vascular Malformation 4.5%, Infrequent causes 4.5%). A total of 7 patients (1.6%) were cryptogenic. In the remaining 58.3% of the patients, ICH was attributable to a single (n = 56, 12.7%) or the combination of ≥2 (n = 200, 45.5%) possible/probable etiologies. The most frequent combinations of etiologies involved possible hypertension with possible CAA (H3A3, n = 38) or with probable CAA (H3A2, n = 29), and probable hypertension with probable OA (H2O2, n = 27). The most frequent category with any degree of certainty was hypertension (H1+2+3 = 80.6%) followed by cerebral amyloid angiopathy (A1+2+3 = 30.9%).ConclusionsAccording to our etiologic criteria, only about 40% patients received a definite diagnosis, while in the remaining patients ICH was attributable to a single possible/probable etiology or to more than one possible/probable etiology. The use of these criteria would likely help in the management of patients with ICH.

Hemichorea and Hemiballismus Associated with Cerebral Vascular Malformation Induced by Hyperglycemia: Case Report

Hemichorea and Hemiballismus Associated with Cerebral Vascular Malformation Induced by Hyperglycemia: Case Report

Signal quality of endovascular electroencephalography

Objective, Approach. A growing number of prototypes for diagnosing and treating neurological and psychiatric diseases are predicated on access to high-quality brain signals, which typically requires surgically opening the skull. Where endovascular navigation previously transformed the treatment of cerebral vascular malformations, we now show that it can provide access to brain signals with substantially higher signal quality than scalp recordings. Main results. While endovascular signals were known to be larger in amplitude than scalp signals, our analysis in rabbits borrows a standard technique from communication theory to show endovascular signals also have up to 100× better signal-to-noise ratio. Significance. With a viable minimally-invasive path to high-quality brain signals, patients with brain diseases could one day receive potent electroceuticals through the bloodstream, in the course of a brief outpatient procedure.

Uncommon Clinical Manifestation of Cerebral Arteriovenous Malformation in Children

Seizures are the second presentation of cerebral vascular malformations, while the first cause is bleeding. However, there are few publications about it, so we describe a case in which the photosensitive epilepsy is the first clinical manifestation of an occipital arteriovenous malformation, for scientific interest and clinical knowledge about it. Our patient was diagnosed by magnetic resonance imaging and arteriography, and he was treated with embolizations with good outcome.

Developmental Venous Anomaly: Benign or Not Benign

Developmental venous anomalies (DVAs), previously called venous angiomas, are the most frequently encountered cerebral vascular malformations. However, DVA is considered to be rather an extreme developmental anatomical variation of medullary veins than true malformation. DVAs are composed of dilated medullary veins converging centripetally into a large collecting venous system that drains into the superficial or deep venous system. Their etiology and mechanism are generally accepted that DVAs result from the focal arrest of the normal parenchymal vein development or occlusion of the medullary veins as a compensatory venous system. DVAs per se are benign and asymptomatic except for under certain unusual conditions. The pathomechanisms of symptomatic DVAs are divided into mechanical, flow-related causes, and idiopathic. However, in cases of DVAs associated with hemorrhage, cavernous malformations (CMs) are most often the cause rather than DVAs themselves. The coexistence of CM and DVA is common. There are some possibilities that DVA affects the formation and clinical course of CM because CM related to DVA is generally located within the drainage territory of DVA and is more aggressive than isolated CM in the literature. Brain parenchymal abnormalities surrounding DVA and cerebral varix have also been reported. These phenomena are considered to be the result of venous hypertension associated with DVAs. With the advance of diagnostic imagings, perfusion study supports this hypothesis demonstrating that some DVAs have venous congestion pattern. Although DVAs should be considered benign and clinically silent, they can have potential venous hypertension and can be vulnerable to hemodynamic changes.

AN UNUSUAL CASE OF CEREBELLAR VENOUS ANGIOMA ASSOCIATED WITH TEMPORAL CAVERNOMA – PATHOPHYSIOLOGICAL, DIAGNOSTIC, AND SURGICAL CONSIDERATIONS

Cerebral vascular malformations are hamartomas, classified into four distinct groups:arteriovenous malformations, cavernous malformations, capillary telangiectasias, anddevelopmental venous anomalies. These abnormal vascular entities have distinct histopathological, radiological, and clinical features, which make them different from one another. We report a case of a 37-year-old man, who presented with headaches, generalized grand mal seizures, and an episode of loss of consciousness, due to a left temporal cavernoma. Gadolinium-enhanced T1-weighted MR images showed a left temporal “popcorn-like” lesion, with heterogeneousenhancement, measuring 15/17/18 mm, suggestive of a cavernoma (angiographically occultmalformation). The T2-weighted MRI showed a right cerebellar venous plexus, draining into alarger central vein and the angiogram revealed the pathognomonic caput medusae aspect of avenous angioma. Microsurgical resection of the left temporal cavernous malformation wasperformed using a left frontal temporal approach. The venous angioma was spared to avoid venousinfarction and cerebral edema with devastating vital consequences. The intra- and postoperativecourses were uneventful with total recovery. The seizures remitted under anticonvulsant therapy,and the postoperative computer tomography investigation were within normal limits. The venous angioma was situated in the right cerebellar hemisphere, rather than near the cavernoma, its location making this the particular aspect of this case.

Fusion Technique Using Three-Dimensional Digital Subtraction Angiography in the Evaluation of Complex Cerebral and Spinal Vascular Malformations

The authors introduce a new fusion technique using 2 three-dimensional digital subtraction angiography images acquired by a flat-panel detector angiographic system to understand the detailed angioarchitecture of complex cerebral and spinal vascular malformations. Eleven consecutive cases of arteriovenous fistula or arteriovenous malformation with lesions that involved 2 major vessels (i.e., internal carotid arteries, external carotid arteries, vertebral arteries, or spinal arteries) were included. Three-dimensional rotational angiography was performed in each affected vessel after conventional cerebral or spinal angiography. Subsequently, the 2 three-dimensional digital subtraction angiography images were fused. The fused images provided the accurate three-dimensional angioarchitecture of complex vascular malformations clearly with high spatial resolution. In particular, the relationship between the nidus/fistulous point, the feeding arteries from each major vessel, and the draining veins could be easily delineated from different directions and multiple angles. Surgical or endovascular treatment was performed in all cases based on the information from these fused three-dimensional images. A fusion technique using two three-dimensional digital subtraction angiography is beneficial for preoperative planning and successful treatment in cases of complex cerebral and spinal vascular malformations.