Brain Volumes in Opsoclonus-Myoclonus Ataxia Syndrome: A Longitudinal Study.

INTRODUCTION: Little is known about the longitudinal trajectory of brain growth in children with opsoclonus-myoclonus ataxia syndrome. We performed a longitudinal evaluation of brain volumes in pediatric opsoclonus-myoclonus ataxia syndrome patients compared with age- and sex-matched healthy children. PATIENTS AND METHODS: This longitudinal case-control study included brain magnetic resonance imaging (MRI) scans from consecutive pediatric opsoclonus-myoclonus ataxia syndrome patients (2009-2020) and age- and sex-matched healthy control children. FreeSurfer analysis provided automatic volumetry of the brain. Paired t tests were performed on the curvature of growth trajectories, with Bonferroni correction. RESULTS: A total of 14 opsoclonus-myoclonus ataxia syndrome patients (12 female) and 474 healthy control children (406 female) were included. Curvature of the growth trajectories of the cerebral white and gray matter, cerebellar white and gray matter, and brainstem differed significantly between opsoclonus-myoclonus ataxia syndrome patients and healthy control children (cerebral white matter, P = .01; cerebral gray matter, P = .01; cerebellar white matter, P < .001; cerebellar gray matter, P = .049; brainstem, P < .01). DISCUSSION/CONCLUSION: We found abnormal brain maturation in the supratentorial brain, brainstem, and cerebellum in children with opsoclonus-myoclonus ataxia syndrome.

Increased rate of significant findings on brain MRI during the early stage of the COVID-19 pandemic.

OBJECTIVES: To assess the effect of the COVID-19 pandemic on the proportion of abnormal paediatric neuroimaging findings as a surrogate marker for potential underutilisation. METHODS: Consecutive paediatric brain MRIs performed between March 27th and June 19th 2019 (Tbaseline) and March 23rd and June 1st 2020 (Tpandemic) were reviewed and classified according to presence or absence and type of imaging abnormality, and graded regarding severity on a 5-point Likert scale, where grade 4 was defined as abnormal finding requiring non-urgent intervention and grade 5 was defined as acute illness prompting urgent medical intervention. Non-parametric statistical testing was used to assess for significant differences between Tpandemic vs. Tbaseline. RESULTS: Fewer paediatric MRI brains were performed during Tpandemic compared to Tbaseline (12.2 vs 14.7 examinations/day). No significant difference was found between the two time periods regarding sex and age (Tbaseline: 557 females (44.63%), 7.95 ± 5.49 years, Tpandemic: 385 females (44.61%), 7.64 ± 6.11 years; p = 1 and p = .079, respectively). MRI brain examinations during Tpandemic had a higher likelihood of being abnormal, 41.25% vs. 25.32% (p<.0001). Vascular abnormalities were more frequent during Tpandemic (11.01% vs 8.01%, p = .02), congenital malformations were less common (8.34% vs 12.34%, p = .004). Severity of MRI brain examinations was significantly different when comparing group 4 and group 5 individually and combined between Tbaseline and Tpandemic (p = .0018, p < .0001, and p <.0001, respectively). CONCLUSIONS: The rate of abnormality and severity found on paediatric brain MRI was significantly higher during the early phase of the pandemic, likely due to underutilisation.

Volumetric differences of thalamic nuclei in children with trisomy 21.

OBJECTIVES: Histological studies have shown alterations of thalamic nuclei in patients with Down syndrome (DS). The correlation of these changes on MRI (magnetic resonance imaging) is unclear. Therefore, this study investigates volumetric differences of thalamic nuclei in children with DS compared to controls. METHODS: Patients were retrospectively identified between 01/2000 and 10/2021. Patient inclusion criteria were: (1) 0-18 years of age, (2) diagnosis of DS, and (3) availability of a brain MRI without parenchymal injury and a non-motion-degraded volumetric T1-weighted sequence. Whole thalamus and thalamic nuclei (n = 25) volumes were analyzed bilaterally relative to the total brain volume (TBV). Two-sided t-tests were used to evaluate differences between groups. Differences were considered significant if the adjusted p-value was <0.05 after correction for multiple hypothesis testing using the Holm-Bonferroni method. RESULTS: 21 children with DS (11 females, 52.4%, mean age: 8.6 ± 4.3 years) and 63 age- and sex-matched controls (32 females, 50.8%, 8.6 ± 4.3 years) were studied using automated volumetric segmentation. Significantly smaller ratios were found for nine thalamic nuclei and the whole thalamus on the right and five thalamic nuclei on the left. TBV was significantly smaller in patients with DS (p < 0.001). No significant differences were found between the groups for age and sex. CONCLUSIONS: In this exploratory volumetric analysis of the thalamus and thalamic nuclei, we observed statistically significant volumetric changes in children with DS. Our findings confirm prior neuroimaging and histological studies and extend the range of involved thalamic nuclei in pediatric DS.

MRI Volumetric Analysis of the Hypothalamus and Limbic System across the Pediatric Age Span.

PURPOSE: Literature is scarce regarding volumetric measures of limbic system components across the pediatric age range. The purpose of this study is to remedy this scarcity by reporting continuous volumetric measurements of limbic system components, and to provide consistent stratification data including age-related trajectories and sex-related differences in the pediatric age range in order to improve the recognition of structural variations that might reflect pathology. METHODS: In this retrospective study, MRI sequences of children with normal clinical MRI examinations of the brain acquired between January 2010 and December 2019 were included. Isotropic 3D T1-weighted were processed using FreeSurfer version 7.3. Total brain volume and volumes of the limbic system including the hippocampus, parahippocampal gyrus, amygdala, hypothalamus, cingulate gyrus, entorhinal cortex, anteroventral thalamic nucleus, and whole thalamus were assessed. Parcellated output was displayed with the respective label map overlay and images were visually inspected for accuracy of regional segmentation results. Continuous data are provided as mean and standard deviation with quadratic trendlines and as mean and 95% confidence intervals. Categorical data are presented as integers and percentages (%). RESULTS: A total of 724 children (401 female, 55.4%), with a mean age at time of MRI of 10.9 ± 4.2 years (range: 1.9-18.2 years), were included in the study. For females, the total brain volume increased from 955 ± 70 mL at the age of 2-3 years to 1140 ± 110 mL at the age of 17-18 years. Similarly, the total brain volume increased for males from 1004 ± 83 mL to 1263 ± 96 mL. The maximum volume was noted at 11-12 years for females (1188 ± 90 mL) and at 14-15 years for males (1310 ± 159 mL). Limbic system structures reached their peak volume more commonly between the 13-14 years to 17-18 years age groups. The male cingulate gyrus, entorhinal cortex, and anteroventral thalamic nucleus reached peak volume before or at 9-10 years. CONCLUSION: This study provides unique age- and sex-specific volumes of the components of the limbic system throughout the pediatric age range to serve as normal values in comparative studies. Quantification of volumetric abnormalities of the limbic system on brain MRI may offer insights into phenotypical variations of diseases and may help elucidate new pathological phenotypes.

Fet-Net Algorithm for Automatic Detection of Fetal Orientation in Fetal MRI.

Identifying fetal orientation is essential for determining the mode of delivery and for sequence planning in fetal magnetic resonance imaging (MRI). This manuscript describes a deep learning algorithm named Fet-Net, composed of convolutional neural networks (CNNs), which allows for the automatic detection of fetal orientation from a two-dimensional (2D) MRI slice. The architecture consists of four convolutional layers, which feed into a simple artificial neural network. Compared with eleven other prominent CNNs (different versions of ResNet, VGG, Xception, and Inception), Fet-Net has fewer architectural layers and parameters. From 144 3D MRI datasets indicative of vertex, breech, oblique and transverse fetal orientations, 6120 2D MRI slices were extracted to train, validate and test Fet-Net. Despite its simpler architecture, Fet-Net demonstrated an average accuracy and F1 score of 97.68% and a loss of 0.06828 on the 6120 2D MRI slices during a 5-fold cross-validation experiment. This architecture outperformed all eleven prominent architectures (p < 0.05). An ablation study proved each component's statistical significance and contribution to Fet-Net's performance. Fet-Net demonstrated robustness in classification accuracy even when noise was introduced to the images, outperforming eight of the 11 prominent architectures. Fet-Net's ability to automatically detect fetal orientation can profoundly decrease the time required for fetal MRI acquisition.

Data governance functions to support responsible data stewardship in pediatric radiology research studies using artificial intelligence.

The integration of human and machine intelligence promises to profoundly change the practice of medicine. The rapidly increasing adoption of artificial intelligence (AI) solutions highlights its potential to streamline physician work and optimize clinical decision-making, also in the field of pediatric radiology. Large imaging databases are necessary for training, validating and testing these algorithms. To better promote data accessibility in multi-institutional AI-enabled radiologic research, these databases centralize the large volumes of data required to effect accurate models and outcome predictions. However, such undertakings must consider the sensitivity of patient information and therefore utilize requisite data governance measures to safeguard data privacy and security, to recognize and mitigate the effects of bias and to promote ethical use. In this article we define data stewardship and data governance, review their key considerations and applicability to radiologic research in the pediatric context, and consider the associated best practices along with the ramifications of poorly executed data governance. We summarize several adaptable data governance frameworks and describe strategies for their implementation in the form of distributed and centralized approaches to data management.

Cerebral White Matter Tract Anatomy.

Advances in MR imaging techniques have allowed for detailed in vivo depiction of white matter tracts. The study of white matter structure and connectivity is of paramount importance in leukodystrophies, demyelinating disorders, neoplasms, and various cognitive, neuropsychiatric, and developmental disorders. The advent of advanced "function-preserving" surgical techniques also makes it imperative to understand white matter anatomy and connectivity, to provide accurate road maps for tumor and epilepsy surgery. In this review, we will describe cerebral white matter anatomy with the help of conventional MRI and diffusion tensor imaging.

Volumetric Analysis of Hearing-Related Structures of Brain in Children with GJB2-Related Congenital Deafness.

Background: Children with non-syndromic hereditary sensorineural hearing loss (SNHL) provide an opportunity to explore the impact of hearing on brain development. Objective: This study investigates volumetric differences of key hearing-related structures in children with gap junction protein beta 2 GJB2-related SNHL compared to controls. Materials and methods: Ninety-four children with SNHL (n = 15) or normal hearing (n = 79) were studied using automated volumetric segmentation. Heschl's gyrus (HG), anterior HG (aHG), planum temporale (PT), medial geniculate nucleus (MGN), and nucleus accumbens (NA) were analyzed relative to total brain volume (TBV) at two different age groups: (1) 7−12 months and (2) 13 months−18 years. Two-sided t-tests were used to evaluate differences between groups. Differences were considered significant if p < 0.007. Results: Significantly smaller aHG-to-TBV ratios were found in 13-month-to-18-year-old patients (p < 0.0055). HG-, PT-, MGN-, and NA-to-TBV ratios were smaller in the same age group, without reaching a significant level. Conversely, HG- and NA-to-TBV were larger in the younger age group. No significant differences were found between the groups for age and TBV. Conclusions: In this exploratory volumetric analysis of key hearing-related structures, we observed age-related changes in volume in children with GJB2-related SNHL.

Rotational Vertebrobasilar Insufficiency: Is There a Physiological Spectrum? Phase-Contrast Magnetic Resonance Imaging Quantification in Healthy Volunteers.

BACKGROUND: Some cases of cerebral ischemia have been attributed to dynamic flow limitation in neck vessels. It however remains unknown whether this represents the extreme end of a physiological response. METHODS: Eighteen healthy volunteers were recruited to this prospective study. Cervical blood flow (ml/min/m2) was assessed using phase-contrast MRI, and cerebral perfusion ratios were assessed using arterial spin labeling perfusion at neutral position, predefined head rotations, as well as flexion and extension. Inter-reader agreements were assessed using intraclass correlation coefficient. RESULTS: The mean age was 38.6 ± 10.8 (range = 22-56) years, for five male participants and 13 females. The means for height and weight were 168 cm and 73.2 kg, respectively. There were no significant differences in individual arterial blood flow with change in head position (P > 0.05). Similarly, the repeated-measures analysis of variance test demonstrated no significant difference in perfusion ratios in relation to head position movement (P > 0.05). Inter-reader agreement was excellent (intraclass correlation coefficient = 0.97). CONCLUSIONS: There is neither significant change in either individual cervical arterial blood flow nor cerebral perfusion within the normal physiological/anatomical range of motion in healthy individuals. It is therefore reasonable to conclude that any such hemodynamic change identified in a patient with ischemic stroke be considered causative.

Musculoskeletal ultrasound in hemophilia: Results and recommendations from a global survey and consensus meeting.

INTRODUCTION: For persons with hemophilia, optimization of joint outcomes is an important unmet need. The aim of this initiative was to determine use of ultrasound in evaluating arthropathy in persons with hemophilia, and to move toward consensus among hemophilia care providers regarding the preferred ultrasound protocols for global adaptation. METHODS: A global survey of hemophilia treatment centers was conducted that focused on understanding how and why ultrasound was being used and endeavored to move toward consensus definitions of both point-of-care musculoskeletal ultrasound (POC-MSKUS) and full diagnostic ultrasound, terminology to describe structures being assessed by ultrasound, and how these assessments should be interpreted. Next, an in-person meeting of an international group of hemophilia health care professionals and patient representatives was held, with the objective of achieving consensus regarding the acquisition and interpretation of POC-MSKUS and full diagnostic ultrasound for use in the assessment of musculoskeletal (MSK) pathologies in persons with hemophilia. RESULTS: The recommendations were that clear definitions of the types of ultrasound examinations should be adopted and that a standardized ultrasound scoring/measurement system should be developed, tested, and implemented. The scoring/measurement system should be tiered to allow for a range of complexity yet maintain the ability for comparison across levels. CONCLUSION: Ultrasound is an evolving technology increasingly used for the assessment of MSK outcomes in persons with hemophilia. As adoption increases globally for clinical care and research, it will become increasingly important to establish clear guidelines for image acquisition, interpretation, and reporting to ensure accuracy, consistency, and comparability across groups.

Current status of MR imaging of juvenile idiopathic arthritis.

Juvenile idiopathic arthritis (JIA) is the most common chronic arthropathy in the pediatric population. Although the diagnosis is essentially clinical for many affected joints, MR imaging has become an important tool for the assessment of joints that are difficult to evaluate clinically, such as temporomandibular and sacroiliac joints, and for screening of inflammatory changes in the entire body by whole body MRI (WBMRI) assessment. The utilization of MR imaging is challenging in the pediatric population given the need for discrimination between pathological and physiological changes in the growing skeleton. Several multicentric multidisciplinary organizations have made major efforts over the past decades to standardize, quantify, and validate scoring systems to measure joint changes both cross-sectionally and longitudinally according to rigorous methodological standards. In this paper, we (1) discuss current trends for the diagnosis and management of JIA, (2) review challenges for detecting real pathological changes in growing joints, (3) summarize the current status of standardization of MRI protocols for data acquisition and the quantification of joint pathology in JIA by means of scoring systems, and (4) outline novel MR imaging techniques for the evaluation of anatomy and function of joints in JIA. Optimizing the role of MRI as a robust biomarker and outcome measure remains a priority of future research in this field.

Dual phase infusion with bolus tracking: technical innovation for cardiac and respiratory navigated magnetic resonance angiography using extracellular contrast.

This technical innovation paper describes a technique for performing cardiac-gated, respiratory-navigated cardiovascular magnetic resonance angiography using an extracellular gadolinium-based contrast agent at 1.5 Tesla (T) with a dual phase bolus injection and slow infusion technique.

Magnetic resonance imaging with RF encoding on curved natural slices.

While the idea of using spatial encoding fields (SEM) for image formation has been proven, conventional wisdom still holds that a magnetic resonance imaging (MRI) system begins with a highly uniform magnetic field. In particular, radio frequency (RF) encoding MRIs designed and tested to date have largely used uniform magnetic fields. Here we demonstrate magnetic resonance imaging in a magnetic field with a built-in gradient that gives non-planar slices - curved surfaces - when the nuclear spins are excited with narrow band RF pulses. Image encoding on these naturally occurring non-planar slices was accomplished with RF encoding using a non-linear spatially varying B1 phase gradient. The imaging methods were demonstrated on a small prototype MRI instrument. The MRI has no switched magnetic field gradients - it is "gradient-free". A low field gradient-free MRI with low mass permanent magnets and simple, low power, RF encoding hardware is ideal for deployment on the International Space Station for the study of astronaut muscle and bone mass loss. Gradient-free natural slice encoding MRI designs would also be portable enough for application in remote terrestrial locations, in emergency rooms and in operating rooms where they can be used with minimally invasive and robotic surgery.

A technique for rapid single-echo spin-echo T2 mapping.

A rapid technique for mapping of T(2) relaxation times is presented. The method is based on the conventional single-echo spin echo approach but uses a much shorter pulse repetition time to accelerate data acquisition. The premise of the new method is the use of a constant difference between the echo time and pulse repetition time, which removes the conventional and restrictive requirement of pulse repetition time >> T(1). Theoretical and simulation investigations were performed to evaluate the criteria for accurate T(2) measurements. Measured T(2)s were shown to be within 1% error as long as the key criterion of pulse repetition time/T(2) > or =3 is met. Strictly, a second condition of echo time/T(1) << 1 is also required. However, violations of this condition were found to have minimal impact in most clinical scenarios. Validation was conducted in phantoms and in vivo T(2) mapping of healthy cartilage and brain. The proposed method offers all the advantages of single-echo spin echo imaging (e.g., immunity to stimulated echo effects, robustness to static field inhomogeneity, flexibility in the number and choice of echo times) in a considerably reduced amount of time and is readily implemented on any clinical scanner.

Sex differences in the human corpus callosum microstructure: a combined T2 myelin-water and diffusion tensor magnetic resonance imaging study.

Sex differences in structure and organization of the corpus callosum (CC) have been identified in healthy adults and may be linked to distinct functional lateralization and processing in men and women. Magnetic resonance imaging (MRI) has facilitated noninvasive assessment of CC sex differences in morphology by volumetric imaging and microstructural organization by diffusion tensor imaging (DTI). Incorporation of recently developed myelin-water fraction (MWF) imaging may improve our understanding of CC sex differences. The aim of the current study was to combine DTI and diffusion tractography with MWF imaging to investigate CC sex differences in 22 healthy adults (11 male, 11 female). We performed MWF imaging using a 5-echo linear combination of spin echo images, and quantified mean diffusivity, axial diffusivity, radial diffusivity and fractional anisotropy (FA) by DTI. Fiber density index (FDi) was quantified using diffusion tractography. The MWF in males was significantly greater than females for the rostral body (p<0.05) and posterior midbody (p<0.005); whereas, the splenium MWF in males was significantly less than females (p<0.05). The DTI analysis revealed significantly increased FA in males compared with females within the genu of the CC (p<0.05). No significant sex-differences existed for mean diffusivity, axial diffusivity, radial diffusivity or FDi. Correlations between DTI parameters and MWF were significant but weak. Results of this study demonstrate regionally dependent sex differences in microstructural composition and organization of the CC and the lack of correlation between DTI and MWF suggest both measures provide unique information within the CC.

MR imaging at 3.0 T in children: technical differences, safety issues, and initial experience.

The high signal-to-noise ratio and contrast-to-noise ratio of 3.0-T magnetic resonance (MR) imaging can be used to obtain high-resolution thin-section images in a short acquisition time. These advantages are associated with an increased specific absorption rate (SAR) and more artifacts owing to B(1) inhomogeneity and increased susceptibility and chemical shift. Potential advantages of 3-T imaging in children include acquisition of good-quality images even with a small field of view (FOV). The shorter overall acquisition time of 3-T imaging is useful in children, who may not be able to cooperate for long. Shorter acquisition times also improve safety by reducing patient monitoring time within the enclosed bore of an MR imaging unit. SAR-related issues and dielectric artifacts are less problematic with a small FOV. Parallel imaging helps reduce SAR, susceptibility artifacts, and blurring of T2-weighted fast spin-echo (FSE) and single-shot FSE images by reducing the echo train length.

Quantitative permeability magnetic resonance imaging in acute ischemic stroke: how long do we need to scan?

Blood-brain barrier (BBB) permeability estimation with dynamic contrast-enhanced MRI (DCE-MRI) has shown significant potential for predicting hemorrhagic transformation (HT) in patients presenting with acute ischemic stroke (AIS). In this work, the effects of scan duration on quantitative BBB permeability estimates (KPS) were investigated. Data from eight patients (three with HT) aged 37-93 years old were retrospectively studied by directly calculating the standard deviation of KPS as a function of scan time. The uncertainty in KPS was reduced only slightly for a scan time of 3 min and 30 s (4% reduction in P value from .047 to .045). When more than 3 min and 30 s of data were used, quantitative permeability MRI was able to separate those patients who proceeded to HT from those who did not (P value <.05). Our findings indicate that reducing permeability acquisition times is feasible in keeping with the need to maintain time-efficient MR protocols in the setting of AIS.

Accurate liver T2 measurement of iron overload: a simulations investigation and in vivo study.

PURPOSE: To investigate the accuracy of T 2 liver iron quantification using different curve-fitting models under varying acquisition conditions, and to compare in iron-overloaded patients the reliability of rapid T 2 measurements against approved and slower T(2) protocols. MATERIALS AND METHODS: Simulations were conducted to assess the influence of various factors on the accuracy of T 2 measurement: curve-fitting model, signal-to-noise ratio (SNR), and echo time (TE) spacing. Fifty-four iron-overloaded pediatric patients were assessed using a standard T(2) and two variations of T 2 acquisitions. In both simulations and in vivo data, three analysis models were evaluated: monoexponential, constant offset, and truncated. RESULTS: Simulations show the truncated model provides the best accuracy but is susceptible to underestimating high iron species under low SNR or high minimum TE. In contrast, the offset model tends to overestimate but maintains the most reliable measurements across the relevant range of iron levels. Furthermore, a much lower SNR can be tolerated if the acquisition uses a low minimum TE. In vivo results confirm theoretical findings and show that T 2 measurements can be as reliable as those from approved and slower T(2) protocols. CONCLUSION: Guidelines are provided on choosing an appropriate model under specific noise conditions and acquisition schemes to ensure accurate and rapid T2 liver iron quantification.

Low detection rate of craniocervical arterial dissection in children using time-of-flight magnetic resonance angiography: causes and strategies to improve diagnosis.

Craniocervical arterial dissection is a frequent cause of childhood arterial ischemic stroke requiring prompt diagnosis and treatment. However, there is no universal guideline for diagnostic cerebrovascular imaging in children. We assessed the role of time-of-flight magnetic resonance angiography in diagnosing craniocervical arterial dissection. We included children (1 month to 18 years) with craniocervical arterial dissection and ischemic stroke from January 1998 to June 2007. Institutional protocol required magnetic resonance imaging (MRI)/ magnetic resonance angiography for all ischemic stroke cases and conventional cerebral angiography if magnetic resonance angiography showed any arteriopathy. Among 233 ischemic stroke cases, craniocervical arterial dissection was diagnosed in 13 patients (5.6%; 10 males), with a mean age of 7.5 years. Initial time-of-flight magnetic resonance angiography missed dissection in 8 patients (61.5%) because the neck vessels were not scanned (n = 2), of suboptimal technique (n = 3), and of diagnostic error (n = 3). T1 fat-saturated MRI and contrast-enhanced magnetic resonance angiography were not performed in 12 patients. If suspicion for arterial dissection is high, T1 fat-saturated neck imaging and contrast-enhanced magnetic resonance angiography should be considered to maximize detection.

Regional pulmonary blood flow: Comparison of dynamic contrast-enhanced MR perfusion and phase-contrast MR.

Regional pulmonary blood flow can be assessed using both dynamic contrast-enhanced (DCE) MR and phase-contrast (PC) MR. These methods provide somewhat complementary information: DCE MR can assess flow over the entire lung while PC MR can detect rapid changes in flow to a targeted region. Although both methods are considered accurate, one may be more feasible than the other depending on pathology, patient condition, and availability of an intravenous route. The objective of this study was to establish a consensus between the two methods by comparing paired DCE MR and PC MR measurements of relative blood flow in Yorkshire piglets (N = 5, age = 7 days, weight = 3.3 +/- 0.6 kg) under various physiological states including regional lung collapse. A strong correlation (R(2) = 0.71, P < 0.01) was observed between the methods. In conclusion, DCE MR and PC MR provide a consistent measure of changes in regional pulmonary blood flow.