Cerebral developmental venous anomalies: Current concepts

Cerebral developmental venous anomalies are the most frequently encountered cerebral vascular malformation, and as such, are frequently reported as fortuitous findings in computed tomography (CT) and magnetic resonance imaging (MRI) studies. Developmental venous anomalies (DVAs) are generally considered extreme anatomical variations of the cerebral vasculature, and follow a benign clinical course in the vast majority of cases. Here we review current concepts on DVAs with the aim of helping clinicians understand this complex entity. Morphological characteristics that are necessary to conceptualize DVAs are discussed in depth. Images modalities used in diagnosing DVAs are reviewed, including new MRI or CT techniques. Clinical presentation, association with other vascular malformations and cerebral parenchymal abnormalities, and possible physiopathological processes leading to associated imaging or clinical findings are discussed. Atypical forms of DVAs are also reviewed and their clinical significance discussed. Finally, recommendations as to how to manage asymptomatic or symptomatic patients with a DVA are advanced. Ann Neurol 2009;66:271–283     

Thrombosis of a Drainage Vein in Developmental Venous Anomaly (DVA) Leading Venous Infarction: A Case Report and Review of the Literature

Developmental venous anomalies (DVAs) are common congenital venous drainage anomalies. Although they typically have a benign clinical course and a low symptomatic rate, thrombosis of a drainage vein may occur, leading to potentially debilitating complications. We report imaging findings of posterior fossa DVA with a thrombosed drainage vein in a patient with nonhemorrhagic cerebellar infarct. We also review the relevant literature on the subject.     

Ataxia and migraine-like headache in a girl with a cerebellar developmental venous anomaly

Cerebral developmental venous anomalies (DVAs) are generally considered as anatomical variants of the venous system without clinical importance. We report the case of a 15-year-old girl with recurrent episodes of migraine-like headache who presented with subacute vertigo and ataxia associated with intense occipital pain. Magnetic resonance imaging (MRI) showed a DVA with signal modifications of the surrounding brain parenchyma in the left cerebellar hemisphere. The patient's ataxia regressed completely within about 2 months. On a follow-up MRI 4 years later the venous malformation and the parenchymal abnormalities were unchanged. We attribute the patient's focal neurological dysfunction to regional changes in the brain parenchyma, possibly secondary to venous hypertension. Our report provides evidence that also uncomplicated DVAs can become symptomatic and supports the role of the venous congestion within the DVA territory in pathogenesis of some brain parenchymal abnormalities associated with DVAs.     

Functional Caudate Imaging in Symptomatic Huntington's Disease: Positron Emission Tomography Versus SinglePhoton Emission Computed Tomography

Functional neuroimaging with positron emission tomography previously demonstrated reduced caudate glucose metabolism in virtually all symptomatic patients with Huntington's disease (HD) Single-photon emission computed tomography studies of brain blood flow also have shown caudate abnormalities in patients with HD. The present study compared these two functional imaging modalities in 6 patients with HD who had been symptomatic for fewer than 5 years. All patients had significantly impaired caudate-thalamus and caudate-whole-slice glucose metabolism ratios as measured by positron emission tomography. However, only 3 had clearly abnormal caudate-thalamus activity ratios and 2 had clearly abnormal caudate-whole-slice ratios on single-photon emission computed tomography. These findings indicate that single-photon emission computed tomography imaging of caudate blood flow is a less sensitive indicator of caudate dysfunction in early HD than is positron emission tomography imaging of caudate glucose metabol1sm.     

Repeated Intracerebral Hemorrhage from Developmental Venous Anomaly Alone

Developmental venous anomalies (DVAs) are hemodynamically low flow, low resistance vascular malformations without clinical significance. Although most DVAs are asymptomatic and are found incidentally, sometimes they can be symptomatic with intracerebral hemorrhage, many of which are usually caused by associated cavernous malformations (CMs) rather than the DVAs themselves. Only a few cases have been reported in the literature where an intracerebral hemorrhage has been caused by a DVA alone. This report describes a case of an intracerebral hemorrhage due to DVA alone with review of the literature.     

The role of imaging in the pathophysiology and diagnosis of headache

PURPOSE OF REVIEW: Functional neuroimaging in headache patients has revolutionized our understanding of these syndromes and provided unique insights into some of the most common maladies in humans, suggesting that at least migraine and cluster headache are driven primarily from the brain. This review highlights new studies and recent advances in studying headache using neuroimaging. RECENT FINDINGS: Concerning the diagnostics of headache, an EFNS Task Force evaluated recently the usefulness of imaging procedures in non-acute headache patients on the basis of evidence from the literature and defined guidelines on when to use magnetic resonance imaging or computed tomography. Regarding the pathophysiology of primary headache syndromes, repeated and independent findings reinforce the crucial role for the brainstem in acute and probably also in chronic migraine, and the hypothalamic grey in several trigemino-autonomic headaches. If further studies confirm these findings, a better understanding will be gained of where and how acute and preventive therapy can be targeted. SUMMARY: Given the rapid advances in functional neuroimaging, in particular newer techniques such as voxel-based morphometry and magnetic resonance spectrometry, functional imaging continues to play a significant role and opens new avenues in targeting the neural substrates in individual primary headache syndromes.     

Neuroimaging studies of the early stages of psychosis: a critical review.

Psychiatric imaging, in particular functional imaging techniques such as functional magnetic resonance imaging (fMRI) are potentially powerful tools to explore the neurophysiological basis of the early stages of psychosis. Despite this impressive growth, neuroimaging has yet to become an established as diagnostic instrument this area, partly as a result of significant heterogeneity across the findings from research studies. The present review aims to: (i) assess the determinants of inconsistencies in the results from neuroimaging studies of the early stages of psychosis; and (ii) suggest approaches for future imaging research in this field that may reduce methodological differences between studies.     

Neuroimaging of Parkinson's disease: Expanding views

Advances in molecular and structural and functional neuroimaging are rapidly expanding the complexity of neurobiological understanding of Parkinson's disease (PD). This review article begins with an introduction to PD neurobiology as a foundation for interpreting neuroimaging findings that may further lead to more integrated and comprehensive understanding of PD. Diverse areas of PD neuroimaging are then reviewed and summarized, including positron emission tomography, single photon emission computed tomography, magnetic resonance spectroscopy and imaging, transcranial sonography, magnetoencephalography, and multimodal imaging, with focus on human studies published over the last five years. These included studies on differential diagnosis, co-morbidity, genetic and prodromal PD, and treatments from l-DOPA to brain stimulation approaches, transplantation and gene therapies. Overall, neuroimaging has shown that PD is a neurodegenerative disorder involving many neurotransmitters, brain regions, structural and functional connections, and neurocognitive systems. A broad neurobiological understanding of PD will be essential for translational efforts to develop better treatments and preventive strategies. Many questions remain and we conclude with some suggestions for future directions of neuroimaging of PD.     

Neurobiological model of obsessive–compulsive disorder: Evidence from recent neuropsychological and neuroimaging findings

Obsessive–compulsive disorder (OCD) was previously considered refractory to most types of therapeutic intervention. There is now, however, ample evidence that selective serotonin reuptake inhibitors and behavior therapy are highly effective methods for treatment of OCD. Furthermore, recent neurobiological studies of OCD have found a close correlation between clinical symptoms, cognitive function, and brain function. A large number of previous neuroimaging studies using positron emission tomography, single‐photon emission computed tomography or functional magnetic resonance imaging (fMRI) have identified abnormally high activities throughout the frontal cortex and subcortical structures in patients with OCD. Most studies reported excessive activation of these areas during symptom provocation. Furthermore, these hyperactivities were decreased after successful treatment using either selective serotonin reuptake inhibitors or behavioral therapy. Based on these findings, an orbitofronto‐striatal model has been postulated as an abnormal neural circuit that mediates symptomatic expression of OCD. On the other hand, previous neuropsychological studies of OCD have reported cognitive dysfunction in executive function, attention, nonverbal memory, and visuospatial skills. Moreover, recent fMRI studies have revealed a correlation between neuropsychological dysfunction and clinical symptoms in OCD by using neuropsychological tasks during fMRI. The evidence from fMRI studies suggests that broader regions, including dorsolateral prefrontal and posterior regions, might be involved in the pathophysiology of OCD. Further, we should consider that OCD is heterogeneous and might have several different neural systems related to clinical factors, such as symptom dimensions. This review outlines recent neuropsychological and neuroimaging studies of OCD. We will also describe several neurobiological models that have been developed recently. Advanced findings in these fields will update the conventional biological model of OCD.     

Infantile spasms: a proposal for a staged evaluation

The purpose of this study was to determine whether a staged, algorithmic evaluation of infantile spasms could be developed to minimize patient discomfort, treatment delay, and overall costs. A retrospective chart review of patients diagnosed with infantile spasms at the authors’ institution during a 10-year period was performed, with 29 patients identified; 28 charts were reviewed. By history and physical examination, 21 were classified as symptomatic and seven as cryptogenic. Of the 21 symptomatic patients, 13 had a known etiology at presentation; with further testing the specific etiology was determined in two more. Two in the cryptogenic group were reclassified on the basis of neuroimaging findings. Evaluations included neuroimaging (27, 15 abnormal), cerebrospinal fluid studies (nine, all normal), comprehensive metabolic studies (17, all normal), chromosomal analysis (11, two abnormal), and ophthalmologic evaluation (27, six abnormal). The average cost of the studies per patient was $5,076 at the authors’ institution. Etiologic yield was increased by 20% with neuroimaging. The other investigations either confirmed a known etiology or were noncontributory. On the basis of these findings, the authors propose an algorithm for a more focused evaluation of infantile spasms. Using the algorithm, the authors suggest directly proceeding to therapy in patients with specific etiologies determined by history and examination. Further evaluation should start with neuroimaging. Subsequent evaluations should be on the basis of those results. The authors estimate a potential 60-90% reduction in total costs if this algorithm is applied.     

Neuroimaging studies of violence and antisocial behavior

The neurobiological basis of violence and antisocial behavior is poorly understood. Lesion studies have implicated the frontal and temporal lobes in such phenomena. Recent neuroimaging studies have provided more detailed information on the neurobiological correlates of violence and antisocial behavior. Moreover, the results of such imaging studies extend findings from prior lesion studies. These results suggest that violent and antisocial behavior is associated with disruptions in frontotemporal neural systems. This article reviews the neuroimaging literature on violence and antisocial behavior and discusses the strengths and weaknesses of the different methods that have been used in such studies. The author reviews findings from cerebral psychophysiology studies (electroencephalographic studies and evoked potentials), as well as from studies using positron emission tomography (PET), single photon emission computed tomography (SPECT), and magnetic resonance imaging (MRI). The article concludes with a discussion of useful directions for future research. It is clear that the use of neuroimaging methods in combination offers the greatest promise for progress in the understanding of the neural basis of violence and antisocial behavior.     

Electrophysiological measures as potential biomarkers in Huntington's disease: Review and future directions

Neuroimaging is fundamental to identifying quantifiable and objective biomarkers in symptomatic and pre-diagnostic Huntington's disease (HD). However, the challenge remains to find reliable biomarkers with high sensitivity and specificity that can be used to track the functional decline over time and test efficacy of therapeutic intervention. While many recent studies have focused on neuroimaging techniques based on brain hemodynamic activity, comparatively fewer have utilized electroencephalography (EEG) and event-related potentials (ERPs). This review aims to summarise and integrate key electroencephalographical findings from the last two decades in symptomatic and pre-diagnostic HD, in context with recent neuroimaging data, and to use this information to identify promising candidate markers for future research and clinical consideration.     

Large deep venous anomaly presenting as a left frontal lesion

Venous angiomas` also known as deep vein anomalies (DVA), are one of the well-described brain vascular malformations. Frequently they are diagnosed as an incidental finding on neuroimaging (CT or MRI). A DVA may present as a single enhancing venous channel or as a large vascular abnormality illustrated on cerebral angiogram. Such a large DVA may mimic other intracranial lesions that mandate surgical intervention. We describe the radiological findings on CT, MRI, MRA and cerebral angiography of a 26-year-old male who presented with a few months` history of recurrent attacks of light-headiness, dizziness and slurring of speech that usually lasted for 2 minutes and resolved spontaneously. Cerebral angiography illustrated enlarged medullary veins draining into a central venous trunk then into the superior sagittal sinus resembling a caput medusa sign. Large DVAs may present radiologically as a brain lesion. Early recognition of these anomalies would avoid unnecessary or harmful intervention of this, otherwise, benign pathology.     

Sex/gender differences in brain function and structure in alcohol use: A narrative review of neuroimaging findings over the last 10 years

Over the last 10 years, rates of alcohol use disorder (AUD) have increased in women by 84% relative to a 35% increase in men. Rates of alcohol use and high‐risk drinking have also increased in women by 16% and 58% relative to a 7% and 16% increase in men, respectively, over the last decade. This robust increase in drinking among women highlights the critical need to identify the underlying neural mechanisms that may contribute to problematic alcohol consumption across sex/gender (SG), especially given that many neuroimaging studies are underpowered to detect main or interactive effects of SG on imaging outcomes. This narrative review aims to explore the recent neuroimaging literature on SG differences in brain function and structure as it pertains to alcohol across positron emission tomography, magnetic resonance imaging, and functional magnetic resonance imaging modalities in humans. Additional work using magnetic resonance spectroscopy, diffusion tensor imaging, and event‐related potentials to examine SG differences in AUD will be covered. Overall, current research on the neuroimaging of AUD, alcohol consumption, or risk of AUD is limited, and findings are mixed regarding the effect of SG on neurochemical, structural, and functional mechanisms associated with AUD. We address SG disparities in the neuroimaging of AUD and propose a call to action to include women in brain imaging research. Future studies are crucial to our understanding of the neurobiological underpinnings of AUD across neural systems and the vulnerability for AUD among women and men.     

Neuroimaging for patient selection for medial temporal lobe epilepsy surgery: Part 1 Structural neuroimaging

The objective of part one of this review is to present the structural neuroimaging techniques that are currently used to evaluate patients with temporal lobe epilepsy (TLE), and to discuss their potential to define patient eligibility for medial temporal lobe surgery. A PubMed query, using Medline and Embase, and subsequent review, was performed for all English language studies published after 1990, reporting neuroimaging methods for the evaluation of patients with TLE. The extracted data included demographic variables, population and study design, imaging methods, gold standard methods, imaging findings, surgical outcomes and conclusions. Overall, 56 papers were reviewed, including a total of 1517 patients. This review highlights the following structural neuroimaging techniques: MRI, diffusion-weighted imaging, tractography, electroencephalography and magnetoencephalography. The developments in neuroimaging during the last decades have led to remarkable improvements in surgical precision, postsurgical outcome, prognosis, and the rate of seizure control in patients with TLE. The use of multiple imaging methods provides improved outcomes, and further improvements will be possible with future studies of larger patient cohorts.     

Developmental processes and brain imaging studies in Tourette syndrome

OBJECTIVES: It is often difficult to discern how findings of a neuroimaging study relate to the pathophysiology of an illness because imaging correlates may variously represent causes, consequences, or epiphenomena of the condition. The objective of this paper is to exemplify the complexities of interpreting neuroimaging data by reviewing anatomical and functional studies of Tourette syndrome (TS). METHODS: Medline and Psychological Abstracts (PsycInfo) databases were searched for functional and anatomical neuroimaging studies of TS. RESULTS: 9 anatomical and 21 functional cross-sectional imaging studies of TS contributed to this review. Anatomical studies comparing TS patients to age-matched controls have found that lenticular nucleus volumes are reduced in TS adults, while caudate nucleus volumes are reduced in both adults and children with TS. In a study of the cerebral cortex, prefrontal volumes in TS adults were smaller, but in TS children were larger than in those of age-matched controls. Complementing the anatomical findings of reduced volumes of the caudate nucleus in TS, functional studies have suggested that frontal-striatal projections play an important role in the regulation of tic symptoms. The majority of functional studies to date, however, have been limited to the study of adults. These functional studies have yielded variable results that have limited generalizability to the pathophysiology of children with TS. CONCLUSIONS: Although many of the findings in TS imaging may represent pathological causes of the disease, they may also be indicative of compensatory changes in the nervous system of TS subjects. Prospective studies of young children at risk will be necessary to help clarify the relationship between brain abnormalities and the course of the disease.     

Integrating evidence from neuroimaging and neuropsychological studies of obsessive-compulsive disorder: the orbitofronto-striatal model revisited

Obsessive-compulsive disorder (OCD) is a common, heritable and disabling neuropsychiatric disorder. Theoretical models suggest that OCD is underpinned by functional and structural abnormalities in orbitofronto-striatal circuits. Evidence from cognitive and neuroimaging studies (functional and structural magnetic resonance imaging (MRI) and positron emission tomography (PET)) have generally been taken to be supportive of these theoretical models; however, results from these studies have not been entirely congruent with each other. With the advent of whole brain-based structural imaging techniques, such as voxel-based morphometry and multivoxel analyses, we consider it timely to assess neuroimaging findings to date, and to examine their compatibility with cognitive studies and orbitofronto-striatal models. As part of this assessment, we performed a quantitative, voxel-level meta-analysis of functional MRI findings, which revealed consistent abnormalities in orbitofronto-striatal and other additional areas in OCD. This review also considers the evidence for involvement of other brain areas outside orbitofronto-striatal regions in OCD, the limitations of current imaging techniques, and how future developments in imaging may aid our understanding of OCD.     

Brain imaging in posttraumatic stress disorder

This is a review article of neuroimaging studies in post-traumatic stress disorder (PTSD). Findings from structural, biochemical, and functional studies are summarized. Magnetic resonance imaging (MRI) volumetric studies have consistently reported decreased hippocampal volumes in PTSD. Proton magnetic resonance spectroscopy studies report decreased N-acetyl aspartate (NAA) ratios and absolute concentrations in the medial temporal lobe. Although still controversial, these findings from volumetric and spectroscopic studies are thought to represent decreased neuronal density of the hippocampus. Functional imaging studies document different patterns of limbic and paralimbic structure activation in PTSD compared with controls. Of theoretical importance are findings of failure to activate the anterior cingulate as well as amygdala activation during symptom provocation studies. Also, increased amygdala activation was found with a behavioral task targeted to this structure. A neurobiological model is presented that takes into account findings from neuroimaging studies in PTSD as well as animal studies of fear conditioning. This model proposes that central to symptom mediation is a dysfunction of the anterior cingulate, with a failure to inhibit amygdala activation and/or an intrinsic lower threshold of amygdala response to fearful stimuli. The model further proposes that hippocampal atrophy is a result of the chronic hyperarousal symptoms mediated by amygdala activation.     

Evidence-based medicine: neuroimaging of seizures

Evidence-based medicine is useful in epilepsy and neuroimaging (Figs. 1 and 2). An understanding of the pretest probability suggests that focal neurologic deficits are important in predicting the outcome of neuroimaging examinations. In cases of nonacute symptomatic seizures, confusion and postictal deficits should prompt MR evaluation. In remote symptomatic seizures, MR imaging should be performed in a child with unexplained cognitive or motor delays or a child less than 1 year of age. Patients with partial seizures, abnormal EEG, or generalized epilepsy also should be imaged. Acute seizures should be imaged with CT to exclude hemorrhage and because of the availability and speed of the modality. Ictal SPECT is the best neuroimaging examination to localize seizure activity. MR imaging can offer prediction of surgical outcome and may hold promise in the future for dimensional localization of seizure focus. Evidence-based medicine can only work if there is physician communication. The pretest probability is helpful only when an accurate history is provided to the consulting physician. This field will flourish if physicians can develop accurate methods of collating information and reporting it in a timely fashion in the literature.