Benefits of newborn screening and hematopoietic cell transplant in infantile Krabbe disease.

Infantile Krabbe disease (IKD) can be treated with hematopoietic cell transplantation (HCT) if done during the first weeks of life before symptoms develop. To facilitate this, newborn screening (NBS) has been instituted in 8 US states. An application to add IKD to the recommended NBS panel is currently under review.

Novel approaches to quantify CNS involvement in children with Pompe disease.

Objective: To characterize the extent of CNS involvement in children with Pompe disease using brain MRI and developmental assessments.

Methods: The study included 14 children (ages 6-18 years) with infantile Pompe disease (IPD) (n = 12) or late-onset Pompe disease (LOPD) (n = 2) receiving enzyme replacement therapy. White matter (WM) hyperintense foci seen in the brain MRIs were systematically quantified using the Fazekas scale (FS) grading system with a novel approach: the individual FS scores from 10 anatomical areas were summed to yield a total FS score (range absent [0] to severe [30]) for each child.

A Shorter Invitation Period for Manuscript Reviewers: Impact on Time to Completion of Reviews.

The objective of this article was to study the effect of decreasing the time allowed an () reviewer to consider an invitation to review on time for two invitees to accept an invitation and time for both reviewers to return reviews. Neuroradiology manuscripts submitted between September 2015 and June 2017 were randomly assigned in a blinded manner to one of two groups. The groups allowed either a 3-day or a 1-day period for invited reviewers to accept the invitation to review.

Lessons Learned From Neuroradiology Manuscript Reviews: Informative Advice for Prospective Authors in All Fields of Radiology.

Comments from manuscript reviews are helpful for guiding a decision about publication and also afford a source of valuable information about how to improve a manuscript. This article is a compendium of comments from reviews of Neuroradiology/Head and Neck Imaging manuscripts that, collectively, serve as a guide for writing a manuscript in any field of radiology. The comments provide examples of previously published guidelines for a manuscript that will have a high likelihood of being published.

Peering Into Peer Review: Neuroradiology Reviewers Discuss Their Approaches to Assessing a Manuscript.

This article provides comments from a small group of highly qualified reviewers of the () regarding their approach to assessing manuscripts. The objective is to educate authors about the issues to which reviewers particularly attend and about errors that will decrease the likelihood of publication. By following the advice provided in this article, authors should be able to compose better manuscripts and reviewers should be able to generate better reviews.

Imaging Evaluation of the Adult Presenting With New-Onset Seizure.

Objective: The purpose of this study is to discuss the evidence supporting the use of neuroimaging in adult patients presenting with new-onset seizure.

Conclusion: Unenhanced CT should be the initial imaging examination performed for adults presenting with first unprovoked seizure in the acute setting to exclude conditions requiring urgent or emergent intervention. MRI has added benefits and should be considered for adults presenting acutely for whom the initial CT is negative and for those presenting with new-onset seizure in the nonacute setting.

Assessment of quantitative magnetic resonance imaging metrics in the brain through the use of a novel phantom.

Objective: Multisite and longitudinal neuroimaging studies are important in uncovering trajectories of recovery and neurodegeneration following traumatic brain injury (TBI) and concussion through the use of diffusion tensor imaging (DTI) and other imaging modalities. This study assessed differences in anisotropic diffusion measurement across four scanners using a human and a novel phantom developed in conjunction with the Chronic Effects of Neurotrauma Consortium.

Method: Human scans provided measurement within biological tissue, and the novel physical phantom provided measures of anisotropic intra-tubular diffusion to serve as a model for intra-axonal water diffusion.

Analysis of variability of fractional anisotropy values at 3T using a novel diffusion tensor imaging phantom.

We employed a novel diffusion tensor imaging phantom to study intra- and interscanner reproducibility on two 3T magnetic resonance (MR) scanners. Using a phantom containing thousands of hollow micron-size tubes in complex arrays, we performed two experiments using a b value of 1000 s/ms on two Siemens 3T Trio scanners. First, we performed 12-direction scans.

A Programme for Risk Assessment and Minimisation of Progressive Multifocal Leukoencephalopathy Developed for Vedolizumab Clinical Trials.

Introduction: Over the past decade, the potential for drug-associated progressive multifocal leukoencephalopathy (PML) has become an increasingly important consideration in certain drug development programmes, particularly those of immunomodulatory biologics. Whether the risk of PML with an investigational agent is proven (e.g.

Magnetic resonance perfusion for differentiating low-grade from high-grade gliomas at first presentation.

Background: Gliomas are the most common primary brain tumour. They are graded using the WHO classification system, with Grade II-IV astrocytomas, oligodendrogliomas and oligoastrocytomas. Low-grade gliomas (LGGs) are WHO Grade II infiltrative brain tumours that typically appear solid and non-enhancing on magnetic resonance imaging (MRI) scans.

Quantitative DTI metrics in a canine model of Krabbe disease: comparisons versus age-matched controls across multiple ages.

Purpose The purpose of this study was to compare quantitative diffusion tensor imaging metrics in dogs affected with a model of Krabbe disease to age-matched normal controls. We hypothesized that fractional anisotropy would be decreased and radial diffusivity would be increased in the Krabbe dogs. Methods We used a highly reproducible region-of-interest interrogation technique to measure fractional anisotropy and radial diffusivity in three different white matter regions within the internal capsule and centrum semiovale in four Krabbe affected brains and three age-matched normal control brains.

Quantitative diffusion tensor imaging analysis does not distinguish pediatric canines with mucopolysaccharidosis I from control canines.

Purpose We compared fractional anisotropy and radial diffusivity measurements between pediatric canines affected with mucopolysaccharidosis I and pediatric control canines. We hypothesized that lower fractional anisotropy and higher radial diffusivity values, consistent with dysmyelination, would be present in the mucopolysaccharidosis I cohort. Methods Six canine brains, three affected with mucopolysaccharidosis I and three unaffected, were euthanized at 7 weeks and imaged using a 7T small-animal magnetic resonance imaging system.

Diffusion tensor imaging findings suggestive of white matter alterations in a canine model of mucopolysaccharidosis type I.

Purpose We investigated fractional anisotropy (FA) and radial diffusivity (RD) in a canine model of mucopolysaccharidosis (MPS). We hypothesized that canines affected with MPS would exhibit decreased FA and increased RD values when compared to unaffected canines, a trend that has been previously described in humans with white matter diseases. Methods Four unaffected canines and two canines with MPS were euthanized at 18 weeks of age.

Diffusion tensor imaging tensor shape analysis for assessment of regional white matter differences.

Purpose The purpose of this study was to investigate a novel tensor shape plot analysis technique of diffusion tensor imaging data as a means to assess microstructural differences in brain tissue. We hypothesized that this technique could distinguish white matter regions with different microstructural compositions. Methods Three normal canines were euthanized at seven weeks old.

Novel region of interest interrogation technique for diffusion tensor imaging analysis in the canine brain.

Purpose We describe a novel technique for measuring diffusion tensor imaging metrics in the canine brain. We hypothesized that a standard method for region of interest placement could be developed that is highly reproducible, with less than 10% difference in measurements between raters. Methods Two sets of canine brains (three seven-week-old full-brains and two 17-week-old single hemispheres) were scanned ex-vivo on a 7T small-animal magnetic resonance imaging system.

Contemporary Imaging of Cerebral Arteriovenous Malformations.

Objective: Brain arteriovenous malformation (AVM) rupture results in substantial morbidity and mortality. The goal of AVM treatment is eradication of the AVM, but the risk of treatment must be weighed against the risk of future hemorrhage.

Conclusion: Imaging plays a vital role by providing the information necessary for AVM management.

Improving sensitivity and specificity of capturing and detecting targeted cancer cells with anti-biofouling polymer coated magnetic iron oxide nanoparticles.

Detecting circulating tumor cells (CTCs) with high sensitivity and specificity is critical to management of metastatic cancers. Although immuno-magnetic technology for in vitro detection of CTCs has shown promising potential for clinical applications, the biofouling effect, i.e.

Diffusion tensor imaging analysis of the brain in the canine model of Krabbe disease.

Purpose: The goal of this study was to compare the diffusion tensor imaging (DTI) metrics from an end-stage canine Krabbe brain evaluated by MR imaging ex vivo to those of a normal dog brain. We hypothesized that the white matter of the canine Krabbe brain would show decreased fractional anisotropy (FA) values and increased apparent diffusion coefficient (ADC) and radial diffusivity (RD) values.

Methods: An 11-week-old Krabbe dog was euthanized after disease progression.

Imaging of Herpesvirus Infections of the CNS.

Objective: Herpesviridae are a family of DNA viruses remarkable for their ability to both promote acute infection and enter a latent phase with potential of reactivation. Herpes infections are ubiquitous throughout the human life span, regardless of the degree of immunocompetence.

Conclusion: We review the virology and clinical manifestations of each herpesvirus, with emphasis on recent advances in knowledge and characteristic neuroimaging findings important for diagnosis and appropriate clinical management.

Automated segmentation of the canine corpus callosum for the measurement of diffusion tensor imaging.

The goal of this study was to apply image registration-based automated segmentation methods to measure diffusion tensor imaging (DTI) metrics within the canine brain. Specifically, we hypothesized that this method could measure DTI metrics within the canine brain with greater reproducibility than with hand-drawn region of interest (ROI) methods. We performed high-resolution post-mortem DTI imaging on two canine brains on a 7 T MR scanner.

Posterior structural brain volumes differ in maltreated youth with and without chronic posttraumatic stress disorder.

Magnetic resonance imaging studies of maltreated children with posttraumatic stress disorder (PTSD) suggest that maltreatment-related PTSD is associated with adverse brain development. Maltreated youth resilient to chronic PTSD were not previously investigated and may elucidate neuromechanisms of the stress diathesis that leads to resilience to chronic PTSD. In this cross-sectional study, anatomical volumetric and corpus callosum diffusion tensor imaging measures were examined using magnetic resonance imaging in maltreated youth with chronic PTSD (N = 38), without PTSD (N = 35), and nonmaltreated participants (n = 59).