Radiological and clinical features of vein of Galen malformations.

BackgroundVein of Galen malformations (VOGMs) are rare and complex congenital arteriovenous fistulas. The clinical and radiological features of VOGMs and their relation to clinical outcomes are not fully characterized.ObjectiveTo examine the clinical and radiological features of VOGMs and the predictors of outcome in patients.MethodsWe retrospectively reviewed the available imaging and medical records of all patients with VOGMs treated at the University of California, San Francisco between 1986 and 2013. Radiological and clinical features were identified. We applied the modified Rankin Scale to determine functional outcome by chart review. Predictors of outcome were assessed by χ(2) analyses.ResultsForty-one cases were confirmed as VOGM. Most patients (78%) had been diagnosed with VOGM in the first year of life. Age at treatment was bimodally distributed, with predominantly urgent embolization at <10 days of age and elective embolization after 1 year of age. Patients commonly presented with hydrocephalus (65.9%) and congestive heart failure (61.0%). Mixed-type (31.7%) VOGM was more common in our cohort than purely mural (29.3%) or choroidal (26.8%) types. The most common feeding arteries were the choroidal and posterior cerebral arteries. Transarterial embolization with coils was the most common technique used to treat VOGMs at our institution. Functional outcome was normal or only mildly disabled in 50% of the cases at last follow-up (median=3 years, range=0-23 years). Younger age at first diagnosis, congestive heart failure, and seizures were predictive of adverse clinical outcome. The survival rate in our sample was 78.0% and complete thrombosis of the VOGM was achieved in 62.5% of patients.ConclusionsVOGMs continue to be challenging to treat and manage. Nonetheless, endovascular approaches to treatment are continuing to be refined and improved, with increasing success. The neurodevelopmental outcomes of affected children whose VOGMs are treated may be good in many cases

“Looks familiar, but I do not know who she is”:The role of the anterior right temporal lobe in famous face recognition

Processing a famous face involves a cascade of steps including detecting the presence of a face, recognizing it as familiar, accessing semantic/biographical information about the person, and finally, if required, production of the proper name. Decades of neuropsychological and neuroimaging studies have identified a network of occipital and temporal brain regions ostensibly comprising the 'core' system for face processing. Recent research has also begun to elucidate upon an 'extended' network, including anterior temporal and frontal regions. However, there is disagreement about which brain areas are involved in each step, as many aspects of face processing occur automatically in healthy individuals and rarely dissociate in patients. Moreover, some common phenomena are not easily induced in an experimental setting, such as having a sense of familiarity without being able to recall who the person is. Patients with the semantic variant of Primary Progressive Aphasia (svPPA) often recognize a famous face as familiar, even when they cannot specifically recall the proper name or biographical details. In this study, we analyzed data from a large sample of 105 patients with neurodegenerative disorders, including 43 svPPA, to identify the neuroanatomical substrates of three different steps of famous face processing. Using voxel-based morphometry, we correlated whole-brain grey matter volumes with scores on three experimental tasks that targeted familiarity judgment, semantic/biographical information retrieval, and naming. Performance in naming and semantic association significantly correlates with grey matter volume in the left anterior temporal lobe, whereas familiarity judgment with integrity of the right anterior middle temporal gyrus. These findings shed light on the neuroanatomical substrates of key components of overt face processing, addressing issues of functional lateralization, and deepening our understanding of neural substrates of semantic knowledge

Predictors of neurosurgical career choice among residents and residency applicants

OBJECTIVE: Medical students applying for neurosurgery residency positions almost all aspire to become academic neurosurgeons. However, most graduates of neurosurgery residency programs ultimately follow careers in private practice. We hypothesized that there might be factors in a resident\u27s application, interviews, or performance during residency that might predict this change in career orientation. METHODS: We retrospectively reviewed the applications, interview evaluations, and residency performance reviews of graduates from the Neurological Surgery program at the University of California, San Francisco and examined factors that might correlate with their career choices. RESULTS: Between 1968 and 2003, 69 neurosurgical residents graduated from the residency program. Of the 54 respondents (78%), 25 (46%) held academic positions and 29 (54%) were in private practice. None of the application data correlated with career choice, and no significant differences in interview scores were observed between academic and private practitioners. Board scores, publications, presentations, and awards received during residency were not predictive of practice orientation. Favorable evaluations during the junior (P = 0.04) and chief residency (P = 0.03) years and pursuit of a subspecialty fellowship (P = 0.006) were predictive. CONCLUSION: Honest discussion between residents and faculty about the choice between academic and private practice careers may be as informative as the three identified predictors of residents\u27 career orientation. Academic faculty members can encourage communication by discouraging the perception that a resident\u27s education might be compromised if he or she expresses interest in private practice rather than academic neurosurgery. Open communication on this issue might enable faculty mentors to actively advise residents in their career decisions and improve the residents\u27 educational environment. Copyright © by the Congress of Neurological Surgeons

Intra-arterial chemotherapy for bilateral retinoblastoma via left ophthalmic artery and right anterior deep temporal artery

A 12-month-old boy with a history of bilateral retinoblastoma refractory to systemic chemotherapy, laser therapy and cryotherapy, with excellent response to previous intra-arterial melphalan infusion, presents with active tumour deposits in the right eye. Repeat intra-arterial chemotherapy was recommended. Previous bilateral melphalan infusion was uneventful using flow-guided catheterisation technique. Direct catheterisation of the right ophthalmic artery was unsuccessful despite employment of several flow-guided and over-the-wire catheters. Superselective catheterisation of the ipsilateral middle meningeal artery was unable to identify an anastomotic connection to the ophthalmic artery; however, angiography of the anterior deep temporal artery identified an alternate route for chemotherapy infusion. The anterior deep temporal artery was successfully and safely catheterised to infuse chemotherapy into the ophthalmic artery. The anterior deep temporal artery is an important potential anastomotic connection to the ophthalmic artery that can be used safely and effectively for central retinal artery chemotherapy infusion for retinoblastoma treatment

Intracranial vascular imaging detects arterial wall abnormalities in persons with treated HIV infection

ObjectiveAlthough a substantial proportion of ischemic strokes in persons with HIV infection (PWH) is related to large artery disease, studies evaluating elevated cerebrovascular risk in PWH have focused primarily on microvascular disease. We compared the burden of intracranial large artery disease on vessel wall MRI (VW-MRI) in PWH and HIV-uninfected individuals.DesignCross-sectional study.MethodsWe recruited antiretroviral therapy-treated PWH with undetectable plasma viral load and HIV-uninfected individuals. All participants were at least 40 years of age and at moderate-to-high cardiovascular risk. We used Poisson and mixed effects logistic regression models to compare the number and associated characteristics of enhancing intracranial arteries on VW-MRI by HIV status.ResultsOf 46 participants (mean age 59 years), 33 were PWH. PWH had nearly four-fold as many enhancing intracranial arteries on VW-MRI than HIV-uninfected individuals (rate ratio 3.94, 95% CI 1.57-9.88, P = 0.003). The majority of wall enhancement was eccentric (76%) and short-segment (93%), suggestive of intracranial atherosclerotic disease (ICAD). Sixty-nine percent of enhancing arteries were not associated with luminal narrowing on magnetic resonance angiography (MRA). None of these characteristics differed significantly by HIV status.ConclusionIn persons at moderate-to-high cardiovascular risk, HIV infection, even when well controlled, may be associated with a greater burden of intracranial large artery disease and, specifically, of ICAD. Studies of the mechanisms underlying higher rates of ischemic stroke in PWH should include evaluation for intracranial large artery disease. VW-MRI provides added value as an adjunct to traditional luminal imaging when evaluating cerebrovascular risk in PWH

Between Always and Never: Evaluating Uncertainty in Radiology Reports Using Natural Language Processing.

The ideal radiology report reduces diagnostic uncertainty, while avoiding ambiguity whenever possible. The purpose of this study was to characterize the use of uncertainty terms in radiology reports at a single institution and compare the use of these terms across imaging modalities, anatomic sections, patient characteristics, and radiologist characteristics. We hypothesized that there would be variability among radiologists and between subspecialities within radiology regarding the use of uncertainty terms and that the length of the impression of a report would be a predictor of use of uncertainty terms. Finally, we hypothesized that use of uncertainty terms would often be interpreted by human readers as hedging. To test these hypotheses, we applied a natural language processing (NLP) algorithm to assess and count the number of uncertainty terms within radiology reports. An algorithm was created to detect usage of a published set of uncertainty terms. All 642,569 radiology report impressions from 171 reporting radiologists were collected from 2011 through 2015. For validation, two radiologists without knowledge of the software algorithm reviewed report impressions and were asked to determine whether the report was uncertain or hedging. The relationship between the presence of 1 or more uncertainty terms and the human readers assessment was compared. There were significant differences in the proportion of reports containing uncertainty terms across patient admission status and across anatomic imaging subsections. Reports with uncertainty were significantly longer than those without, although report length was not significantly different between subspecialities or modalities. There were no significant differences in rates of uncertainty when comparing the experience of the attending radiologist. When compared with reader 1 as a gold standard, accuracy was 0.91, sensitivity was 0.92, specificity was 0.9, and precision was 0.88, with an F1-score of 0.9. When compared with reader 2, accuracy was 0.84, sensitivity was 0.88, specificity was 0.82, and precision was 0.68, with an F1-score of 0.77. Substantial variability exists among radiologists and subspecialities regarding the use of uncertainty terms, and this variability cannot be explained by years of radiologist experience or differences in proportions of specific modalities. Furthermore, detection of uncertainty terms demonstrates good test characteristics for predicting human readers assessment of uncertainty

Comparison of Deep Brain Stimulation Lead Targeting Accuracy and Procedure Duration between 1.5- and 3-Tesla Interventional Magnetic Resonance Imaging Systems: An Initial 12-Month Experience.

BackgroundInterventional magnetic resonance imaging (iMRI) allows deep brain stimulator lead placement under general anesthesia. While the accuracy of lead targeting has been described for iMRI systems utilizing 1.5-tesla magnets, a similar assessment of 3-tesla iMRI procedures has not been performed.ObjectiveTo compare targeting accuracy, the number of lead targeting attempts, and surgical duration between procedures performed on 1.5- and 3-tesla iMRI systems.MethodsRadial targeting error, the number of targeting attempts, and procedure duration were compared between surgeries performed on 1.5- and 3-tesla iMRI systems (SmartFrame and ClearPoint systems).ResultsDuring the first year of operation of each system, 26 consecutive leads were implanted using the 1.5-tesla system, and 23 consecutive leads were implanted using the 3-tesla system. There was no significant difference in radial error (Mann-Whitney test, p = 0.26), number of lead placements that required multiple targeting attempts (Fisher's exact test, p = 0.59), or bilateral procedure durations between surgeries performed with the two systems (p = 0.15).ConclusionsAccurate DBS lead targeting can be achieved with iMRI systems utilizing either 1.5- or 3-tesla magnets. The use of a 3-tesla magnet, however, offers improved visualization of the target structures and allows comparable accuracy and efficiency of placement at the selected targets

A feasibility study for quantitative assessment of cerebrovascular malformations using flutriciclamide ([18F]GE-180) PET/MRI.

AimNeuroinflammation plays a key role in both the pathogenesis and the progression of cerebral cavernous malformations (CCM). Flutriciclamide ([18F]GE-180) is a translocator protein (TSPO) targeting positron emission tomography (PET) tracer, developed for imaging neuroinflammation. The objectives of this study were to describe characteristics of flutriciclamide uptake in different brain tissue regions in CCM patients compared to controls, and to evaluate flutriciclamide uptake and iron deposition within CCM lesions.Materials and methodsFive patients with CCM and six controls underwent a 60 or 90 min continuous PET/MRI scan following 315 ± 68.9 MBq flutriciclamide administration. Standardized uptake value (SUV) and standardized uptake value ratio (SUVr) were obtained using the striatum as a pseudo-reference. Quantitative susceptibility maps (QSM) were used to define the location of the vascular malformation and calculate the amount of iron deposition in each lesion.ResultsIncreased flutriciclamide uptake was observed in all CCM lesions. The temporal pole demonstrated the highest radiotracer uptake; the paracentral lobule, cuneus and hippocampus exhibited moderate uptake; while the striatum had the lowest uptake, with average SUVs of 0.66, 0.55, 0.63, 0.55, and 0.33 for patient with CCM and 0.57, 0.50, 0.48, 0.42, and 0.32 for controls, respectively. Regional SUVr showed similar trends. The average SUV and QSM values in CCM lesions were 0.58 ± 0.23 g/ml and 0.30 ± 0.10 ppm. SUVs and QSM were positively correlated in CCM lesions (r = 0.53, p = 0.03).ConclusionThe distribution of flutriciclamide ([18F]GE-180) in the human brain and CCM lesions demonstrated the potential of this TSPO PET tracer as a marker of neuroinflammation that may be relevant for characterizing CCM disease progression along with QSM

The regional pattern of abnormal cerebrovascular reactivity in HIV-infected, virally suppressed women

The purpose of this study was to assess whole brain and regional patterns of cerebrovascular reactivity (CVR) abnormalities in HIV-infected women using quantitative whole brain arterial spin labeling (ASL). We hypothesized that HIV-infected women would demonstrate decreased regional brain CVR despite viral suppression. This cross-sectional study recruited subjects from the Bay Area Women's Interagency Health Study (WIHS)-a cohort study designed to investigate the progression of HIV disease in women. In addition to conventional noncontrast cerebral MRI sequences, perfusion imaging was performed before and after the administration of intravenous acetazolamide. CVR was measured by comparing quantitative ASL brain perfusion before and after administration of intravenous acetazolamide. In order to validate and corroborate ASL-based whole brain and regional perfusion, phase-contrast (PC) imaging was also performed through the major neck vessels. FLAIR and susceptibility weighted sequences were performed to assess for white matter injury and microbleeds, respectively. Ten HIV-infected women and seven uninfected, age-matched controls were evaluated. Significant group differences were present in whole brain and regional CVR between HIV-infected and uninfected women. These regional differences were significant in the frontal lobe and basal ganglia. CVR measurements were not significantly impacted by the degree of white matter signal abnormality or presence of microbleeds. Despite complete viral suppression, dysfunction of the neurovascular unit persists in the HIV population. Given the lack of association between CVR and traditional imaging markers of small vessel disease, CVR quantification may provide an early biomarker of pre-morbid vascular disease