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Purpose: To assess image quality and diagnostic confidence of 3D T1-weighted spoiled gradient echo (SPGR) MRI using artificial intelligence (AI) reconstruction. Materials and methods: This prospective, IRB-approved study enrolled 50 pediatric patients (mean age = 11.8 ± 3.1 years) undergoing clinical brain MRI. In addition to standard of care (SOC) compressed SENSE (CS = 2.5), 3D T1-weighted SPGR images were obtained with higher CS acceleration factors (5 and 8) to evaluate the ability of AI reconstruction to improve image quality and reduce scan time. Images were reviewed independently on dedicated research PACS workstations by two neuroradiologists. Quantitative analysis of signal intensities to calculate apparent grey and white matter signal to noise (aSNR) and grey-white matter apparent contrast to noise ratios (aCNR) was performed. Results: AI improved overall image quality compared to standard CS reconstruction in 35% (35/100) of evaluations in CS = 2.5 (average scan time = 221 ± 6.9 s), 100% (46/46) of CS = 5 (average scan time = 113.3 ± 4.6 s) and 94% (47/50) of CS = 8 (average scan time = 74.1 ± 0.01 s). Quantitative analysis revealed significantly higher grey matter aSNR, white matter aSNR and grey-white matter aCNR with AI reconstruction compared to standard reconstruction for CS 5 and 8 (all p-values < 0.001), however not for CS 2.5. Conclusions: AI reconstruction improved overall image quality and gray-white matter qualitative and quantitative aSNR and aCNR in highly accelerated (CS = 5 and 8) 3D T1W SPGR images in the majority of pediatric patients. [ABSTRACT FROM AUTHOR]

Background: Artificial intelligence (AI) reconstruction techniques have the potential to improve image quality and decrease imaging time. However, these techniques must be assessed for safe and effective use in clinical practice. Objective: To assess image quality and diagnostic confidence of AI reconstruction in the pediatric brain on fluid-attenuated inversion recovery (FLAIR) imaging. Materials and methods: This prospective, institutional review board (IRB)-approved study enrolled 50 pediatric patients (median age=12 years, Q1=10 years, Q3=14 years) undergoing clinical brain MRI. T2-weighted (T2W) FLAIR images were reconstructed by both standard clinical and AI reconstruction algorithms (strong denoising). Images were independently rated by two neuroradiologists on a dedicated research picture archiving and communication system (PACS) to indicate whether AI increased, decreased, or had no effect on image quality compared to standard reconstruction. Quantitative analysis of signal intensities was also performed to calculate apparent signal to noise (aSNR) and apparent contrast to noise (aCNR) ratios. Results: AI reconstruction was better than standard in 99% (reader 1, 49/50; reader 2, 50/50) for overall image quality, 99% (reader 1, 49/50; reader 2, 50/50) for subjective SNR, and 98% (reader 1, 49/50; reader 2, 49/50) for diagnostic preference. Quantitative analysis revealed significantly higher gray matter aSNR (30.6±6.5), white matter aSNR (21.4±5.6), and gray-white matter aCNR (7.1±1.6) in AI-reconstructed images compared to standard reconstruction (18±2.7, 14.2±2.8, 4.4±0.8, p<0.001) respectively. Conclusion: We conclude that AI reconstruction improved T2W FLAIR image quality in most patients when compared with standard reconstruction in pediatric patients. [ABSTRACT FROM AUTHOR]

Purpose: Rasmussen encephalitis (RE) is a very rare chronic neurological disorder of unilateral inflammation of the cerebral cortex. Hemispherotomy provides the best chance at achieving seizure freedom in RE patients, but with significant risks and variable long-term outcomes. The goal of this study is to utilize our multicenter pediatric cohort to characterize if differences in pathology and/or imaging characterization of RE may provide a window into post-operative seizure outcomes, which in turn could guide decision-making for parents and healthcare providers. Methods: This multi-institutional retrospective review of medical record, imaging, and pathology samples was approved by each individual institution's review board. Data was collected from all known pediatric cases of peri-insular functional hemispherotomy from the earliest available electronic medical records. Mean follow-up time was 4.9 years. Clinical outcomes were measured by last follow-up visit using both Engel and ILAE scoring systems. Relationships between categorical and continuous variables were analyzed with Pearson correlation values. Results: Twenty-seven patients met study criteria. No statistically significant correlations existed between patient imaging and pathology data. Pathology stage, MRI brain imaging stages, and a combined assessment of pathology and imaging stages showed no statistically significant correlation to post-operative seizure freedom rates. Hemispherectomy Outcome Prediction Scale scoring demonstrated seizure freedom in only 71% of patients receiving a score of 1 and 36% of patients receiving a score of 2 which were substantially lower than predicted. Conclusions: Our analysis did not find evidence for either independent or combined analysis of imaging and pathology staging being predictive for post peri-insular hemispherotomy seizure outcomes, prompting the need for other biomarkers to be explored. Our data stands in contrast to the recently proposed Hemispherectomy Outcome Prediction Scale and does not externally validate this metric for an RE cohort. [ABSTRACT FROM AUTHOR]

We aimed to objectively compare the effects of wearing newer, higher-ranked football helmets (HRank) vs. wearing older, lower-ranked helmets (LRank) on pre- to post-season alterations to neuroimaging-derived metrics of athletes' white matter. Fifty-four high-school athletes wore an HRank helmet, and 62 athletes wore an LRank helmet during their competitive football season and completed pre- and post-season diffusion tensor imaging (DTI). Longitudinal within- and between-group DTI metrics [fractional anisotropy (FA) and mean/axial/radial diffusivity (MD, AD, RD)] were analyzed using tract-based spatial statistics. The LRank helmet group exhibited significant pre- to post-season reductions in MD, AD, and RD, the HRank helmet group displayed significant pre- to post-season increases in FA, and both groups showed significant pre- to post-season increases in AD (p's <.05 [corrected]). Between-group analyses revealed the pre- to post-season increase in AD was significantly less for athletes wearing HRank compared to LRank (p <.05 [corrected]). These data provide in vivo evidence that wearing an HRank helmet may be efficacious for preserving white matter from head impact exposure during high school football. Future prospective longitudinal investigations with complimentary imaging and behavioral outcomes are warranted to corroborate these initial in vivo findings. [ABSTRACT FROM AUTHOR]

Purpose: Cyclin-dependent kinase-retinoblastoma (CDK-RB) pathway is dysregulated in some diffuse intrinsic pontine gliomas (DIPG). We evaluated safety, feasibility, and early efficacy of the CDK4/6-inhibitor ribociclib, administered following radiotherapy in newly-diagnosed DIPG patients. Methods: Following radiotherapy, eligible patients received ribociclib in 28-day cycles (350 mg/m2; 21 days on/7 days off). Feasibility endpoints included tolerability for at least 6 courses, and a less than 2-week delay in restarting therapy after 1 dose reduction. Early efficacy was measured by 1-year and median overall survival (OS). Patient/parent-by-proxy reported outcomes measurement information system (PROMIS) assessments were completed prospectively. Results: The study included 10 evaluable patients, 9 DIPG and 1 diffuse midline glioma (DMG)—all 3.7 to 19.8 years of age. The median number of courses was 8 (range 3–14). Three patients required dose reduction for grade-4 neutropenia, and 1 discontinued therapy for hematological toxicity following course 4. The most common grade-3/4 toxicity was myelosuppression. After 2 courses, MRI evaluations in 4 patients revealed increased necrotic volume, associated with new neurological symptoms in 3 patients. The 1-year and median OS for DIPG was 89% and 16.1 months (range 10–30), respectively; the DMG patient died at 6 months post-diagnosis. Five patients donated brain tissue and tumor; 3 were RB+. Conclusions: Ribociclib administered following radiotherapy is feasible in DIPG and DMG. Increased tumor necrosis may represent a treatment effect. These data warrant further prospective volumetric analyses of tumors with necrosis. Feasibility and stabilization findings support further investigation of ribociclib in combination therapies. Trial registration: NCT02607124. [ABSTRACT FROM AUTHOR]

Venous-related brain injury is a common form of cerebrovascular injury in children and encompasses a diverse group of cerebrovascular diagnoses. The purpose of this pictorial essay is to introduce the relevant anatomy, pathophysiology and various imaging patterns of venous-related cerebral injury in children. Unifying concepts to better understand the effects of venous hypertension in the developing brain will be emphasized. These unifying concepts will provide the imaging professional with a conceptual framework to better understand and confidently identify imaging patterns of venous-related cerebral injury. [ABSTRACT FROM AUTHOR]

Background: Cortical dysplasia is the most common cause of pediatric refractory epilepsy. MRI detection of epileptogenic lesion is associated with good postsurgical outcome. Additional electrophysiological information is suggested to be helpful in localization of cortical dysplasia. Educational measures were taken to increase the awareness of cortical dysplasia at our institution in the context of a recent International League Against Epilepsy (ILAE 2011) classification of cortical dysplasia.Objective: To determine changes in the rate of prospective identification of cortical dysplasia on an initial radiology report and also evaluate the benefit of MRI review as part of a multidisciplinary epilepsy conference in identifying previously overlooked MRI findings.Materials and Methods: We retrospectively evaluated surgically treated children with refractory epilepsy from 2007 to 2014 with cortical dysplasia on histopathology. We analyzed the initial radiology report, preoperative MRI interpretation at multidisciplinary epilepsy conference and subsequent retrospective MRI review with knowledge of the resection site. We recorded additional electrophysiological data and the presence of lobar concordance with the MRI findings.Results: Of 78 children (44 MRI lesional) evaluated, 18 had initially overlooked MRI findings. Comparing 2007-2010 to 2011-2014, there was improvement in the rate of overlooked findings on the initial radiology report (54% vs. 13% of lesional cases, respectively; P = 0.008). The majority (72%) were identified at a multidisciplinary conference with lobar concordance of findings with at least one additional electrophysiological investigation in 89%.Conclusion: Awareness of current classification schemes of cortical dysplasia and image review in the context of a multidisciplinary conference can lead to improved MRI detection of cortical dysplasia in children. [ABSTRACT FROM AUTHOR]

Developmental venous anomalies (DVAs) are the most common vascular malformation of the brain and are commonly identified on routine imaging of the brain. They are typically considered incidental findings, usually with no clinical significance. However the increasing identification of DVAs as a result of improved imaging technology has led to recognition of their association with a variety of abnormal imaging findings and clinically important conditions. This pictorial essay explores the suspected embryological origin, associated imaging features, and proposed pathophysiological mechanisms of DVAs in the pediatric population. This paper emphasizes newer physiological imaging data, which suggest that DVA drainage has less physiological flexibility than otherwise normal venous drainage development. [ABSTRACT FROM AUTHOR]

Background: Alagille syndrome is a pediatric multisystem disease with increased prevalence of cerebrovascular disease. The spectrum of cerebrovascular disease in Alagille syndrome includes cerebral aneurysms, moyamoya arteriopathy and dolichoectasia. The prevalence of cerebrovascular disease in Alagille syndrome varies widely in the literature.Objective: To determine the prevalence of cerebrovascular disease in our institution's Alagille patient population by employing a full primary review of all available neuroimaging.Materials and Methods: An institutional review board-approved retrospective review of all Alagille syndrome patients seen at a tertiary care children's hospital from January 2000 to January 2014 was performed. All neuroimaging studies were reviewed for arterial or venous abnormalities. The prevalence of arterial and venous abnormalities was calculated and clinical outcomes were determined.Results: Fifty-two patients with Alagille syndrome ranging in age from 11 months to 27 years were studied. Nineteen (37%) had dedicated vascular neuroimaging. Six (32%) had cerebral arterial disease, 4 with dolichoectasia, 3 with aneurysm(s) and 2 with moyamoya arteriopathy. Three of the four patients with dolichoectasia had associated aneurysm(s). Venous anomalies were present in 4 (21%) patients. One patient with moyamoya arteriopathy underwent revascularization procedures. No deaths were attributable to cerebrovascular disease.Conclusion: Cerebral vasculopathy is an important feature of Alagille syndrome and includes dolichoectasia, cerebral aneurysms and moyamoya arteriopathy. The high prevalence identified in our study suggests noninvasive vascular neuroimaging screening should be performed in this patient population. In addition to cerebral arterial abnormalities, alterations of venous development may be a feature of Alagille syndrome. [ABSTRACT FROM AUTHOR]

We aimed to perform external validation of the recently developed survival prediction model for diffuse intrinsic pontine glioma (DIPG), and discuss its utility. The DIPG survival prediction model was developed in a cohort of patients from the Netherlands, United Kingdom and Germany, registered in the SIOPE DIPG Registry, and includes age <3 years, longer symptom duration and receipt of chemotherapy as favorable predictors, and presence of ring-enhancement on MRI as unfavorable predictor. Model performance was evaluated by analyzing the discrimination and calibration abilities. External validation was performed using an unselected cohort from the International DIPG Registry, including patients from United States, Canada, Australia and New Zealand. Basic comparison with the results of the original study was performed using descriptive statistics, and univariate- and multivariable regression analyses in the validation cohort. External validation was assessed following a variety of analyses described previously. Baseline patient characteristics and results from the regression analyses were largely comparable. Kaplan-Meier curves of the validation cohort reproduced separated groups of standard (n = 39), intermediate (n = 125), and high-risk (n = 78) patients. This discriminative ability was confirmed by similar values for the hazard ratios across these risk groups. The calibration curve in the validation cohort showed a symmetric underestimation of the predicted survival probabilities. In this external validation study, we demonstrate that the DIPG survival prediction model has acceptable cross-cohort calibration and is able to discriminate patients with short, average, and increased survival. We discuss how this clinico-radiological model may serve a useful role in current clinical practice. [ABSTRACT FROM AUTHOR]

Diffuse intrinsic pontine glioma (DIPG), a rare, often fatal childhood brain tumor, remains a major therapeutic challenge. In 2012, investigators, funded by the DIPG Collaborative (a philanthropic partnership among 29 private foundations), launched the International DIPG Registry (IDIPGR) to advance understanding of DIPG. Comprised of comprehensive deidentified but linked clinical, imaging, histopathological, and genomic repositories, the IDIPGR uses standardized case report forms for uniform data collection; serial imaging and histopathology are centrally reviewed by IDIPGR neuro-radiologists and neuro-pathologists, respectively. Tissue and genomic data, and cell cultures derived from autopsies coordinated by the IDIPGR are available to investigators for studies approved by the Scientific Advisory Committee. From April 2012 to December 2016, 670 patients diagnosed with DIPG have been enrolled from 55 participating institutions in the US, Canada, Australia and New Zealand. The radiology repository contains 3558 studies from 448 patients. The pathology repository contains tissue on 81 patients with another 98 samples available for submission. Fresh DIPG tissue from seven autopsies has been sent to investigators to develop primary cell cultures. The bioinformatics repository contains next-generation sequencing data on 66 tumors. Nine projects using data/tissue from the IDIPGR by 13 principle investigators from around the world are now underway. The IDIPGR, a successful alliance among philanthropic agencies and investigators, has developed and maintained a highly collaborative, hypothesis-driven research infrastructure for interdisciplinary and translational projects in DIPG to improve diagnosis, response assessment, treatment and outcome for patients. [ABSTRACT FROM AUTHOR]

Introduction: The purpose of this study was to determine the diagnostic accuracy of synthetic MR sequences generated through post-acquisition processing of a single sequence measuring inherent R1, R2, and PD tissue properties compared with sequences acquired conventionally as part of a routine clinical pediatric brain MR exam. Methods: Thirty-two patients underwent routine clinical brain MRI with conventional and synthetic sequences acquired (22 abnormal). Synthetic axial T1, T2, and T2 fluid attenuation inversion recovery or proton density-weighted sequences were made to match the comparable clinical sequences. Two exams for each patient were de-identified. Four blinded reviewers reviewed eight patients and were asked to generate clinical reports on each exam (synthetic or conventional) at two different time points separated by a mean of 33 days. Exams were rated for overall and specific finding agreement (synthetic/conventional and compared to gold standard consensus review by two senior reviewers with knowledge of clinical report), quality, and diagnostic confidence. Results: Overall agreement between conventional and synthetic exams was 97%. Agreement with consensus readings was 84% (conventional) and 81% (synthetic), p = 0.61. There were no significant differences in sensitivity, specificity, or accuracy for specific imaging findings involving the ventricles, CSF, brain parenchyma, or vasculature between synthetic or conventional exams ( p > 0.05). No significant difference in exam quality, diagnostic confidence, or noise/artifacts was noted comparing studies with synthetic or conventional sequences. Conclusions: Diagnostic accuracy and quality of synthetically generated sequences are comparable to conventionally acquired sequences as part of a standard pediatric brain exam. Further confirmation in a larger study is warranted. [ABSTRACT FROM AUTHOR]

Introduction: Synthetic magnetic resonance imaging is a quantitative imaging technique that measures inherent T1-relaxation, T2-relaxation, and proton density. These inherent tissue properties allow synthesis of various imaging sequences from a single acquisition. Clinical use of synthetic MR imaging has been described in adult populations. However, use of synthetic MR imaging has not been previously reported in children. The purpose of this study is to report our assessment of diagnostic image quality using synthetic MR imaging in children. Methods: Synthetic MR acquisition was obtained in a sample of children undergoing brain MR imaging. Image quality assessments were performed on conventional and synthetic T1-weighted, T2-weighted, and FLAIR images. Standardized linear measurements were performed on conventional and synthetic T2 images. Estimates of patient age based upon myelination patterns were also performed. Results: Conventional and synthetic MR images were evaluated on 30 children. Using a 4-point assessment scale, conventional imaging performed better than synthetic imaging for T1-weighted, T2-weighted, and FLAIR images. When the assessment was simplified to a dichotomized scale, the conventional and synthetic T1-weighted and T2-weighted images performed similarly. However, the superiority of conventional FLAIR images persisted in the dichotomized assessment. There were no statistically significant differences between linear measurements made on T2-weighted images. Estimates of patient age based upon pattern of myelination were also similar between conventional and synthetic techniques. Conclusion: Synthetic MR imaging may be acceptable for clinical use in children. However, users should be aware of current limitations that could impact clinical utility in the software version used in this study. [ABSTRACT FROM AUTHOR]

Although bevacizumab has not proven effective in adults with newly diagnosed high-grade gliomas (HGG), feasibility in newly diagnosed children with diffuse intrinsic pontine gliomas (DIPG) or HGG has not been reported in a prospective study. In a safety and feasibility study, children and young adults with newly diagnosed HGG received radiotherapy (RT) with bevacizumab (10 mg/kg: days 22, 36) and temozolomide (75-90 mg/m/day for 42 days) followed by bevacizumab (10 mg/kg, days 1, 15), irinotecan (125 mg/m, days 1, 15) and temozolomide (150 mg/m/day days 1-5). DIPG patients did not receive temozolomide. Telomerase activity, quality of life (QOL), and functional outcomes were assessed. Among 27 eligible patients (15 DIPG, 12 HGG), median age 10 years (range 3-29 years), 6 discontinued therapy for toxicity: 2 during RT (grade 4 thrombocytopenia, grade 3 hepatotoxicity) and 4 during maintenance therapy (grade 3: thrombosis, hypertension, skin ulceration, and wound dehiscence). Commonest ≥grade 3 toxicities included lymphopenia, neutropenia and leukopenia. Grade 3 hypertension occurred in 2 patients. No intracranial hemorrhages occurred. For DIPG patients, median overall survival (OS) was 10.4 months. For HGG patients, 3-year progression free survival and OS were 33 % (SE ± 14 %) and 50 % (SE ± 14 %), respectively. All 3 tested tumor samples, demonstrated histone H3.3K27M (n = 2 DIPG) or G34R (n = 1 HGG) mutations. QOL scores improved over the course of therapy. A bevacizumab-based regimen is feasible and tolerable in newly diagnosed children and young adults with HGG and DIPG. [ABSTRACT FROM AUTHOR]

It has been postulated that mitochondrial dysfunction may be an important factor in epileptogenesis of intractable epilepsy. The current study tests the hypothesis that mitochondrial Complex IV (CIV) or cytochrome c oxidase dysfunction is associated with the seizure onset zone (SOZ) in patients with focal cortical dysplasia (FCD). Subjects were selected based on: age <19y; epilepsy surgery between May, 2010 and October, 2011; pathological diagnosis of isolated focal cortical dysplasia Type I (FCDI) or Type II (FCDII); and sufficient residual cortical tissue to conduct analysis of electron transport chain complex (ETC) activity in SOZ and adjacent cortical regions. In this retrospective study, patients were identified who had sufficient unfixed, frozen brain tissue for biochemical analysis in tissue homogenates. Specimens were subtyped using ILAE classification for FCD, and excluded if diagnosed with FCD Type III or dual pathology. Analysis of ETC activity in resected tissues was conducted independently and without knowledge of the identity, diagnosis, or clinical status of individual subjects. Seventeen patients met the inclusion criteria, including 6 FCDI and 11 FCDII. Comparison of adjacent cortical resections showed decreased CIV activity in the SOZ of the FCDII group ( P = 0.003), but no significant CIV difference in adjacent tissues of the FCDI group. Because of the importance of CIV as the terminal and rate-limiting complex in the mitochondrial electron transport chain, these authors conclude that 1) a deficit of CIV is associated with the SOZ of patients with FCDII; 2) CIV deficiency may contribute to the spectrum of FCD neuropathology; and 3) further investigation of CIV in FCD may lead to the discovery of new targets for neuroprotective therapies for patients with intractable epilepsy. [ABSTRACT FROM AUTHOR]

In contemporary dance, cognitive events are not necessarily restricted 'to the skin or skull of an individual' (Hutchins in Int Encycl Soc Behav Sci 2068-2072, ) but distributed across dancers during collaborative improvisation. There is some experimental evidence of greater output when people perform problem-solving tasks alone. However, when a task is challenging and paired participants are familiar with each other, pairwise and emergent outcomes are more plentiful than solo outcomes. We investigate these factors in the context of dance with the broad hypothesis that innovation is enhanced when dancers improvise together compared with when they improvise alone. Dancers ( N = 10) in a professional company improvised for 2 min alone and then with another dancer. Dancer familiarity (familiar, unfamiliar) and task (expressive, non-expressive) were crossed (within-subjects). The improvisations were video-recorded over 2 h in the dancers' usual improvisation space. After each improvisation, the dancers: stated the number of movement ideas expressed and rated task ease, satisfaction, interest, novelty, originality and clarity. In both tasks, there was a tendency for self-report of a greater number of movement ideas expressed in familiar and unfamiliar pairs than alone. Ratings of task ease, satisfaction, interest, clarity, etc. were slightly higher in the unfamiliar pair condition. In the non-expressive task, ratings of the task were higher in pairs ( M = 3.02, SD 0.82) than in the solo ( M = 2.67, SD 0.96) condition. Distributed creativity, relational cognition and social facilitation are used to interpret the results. [ABSTRACT FROM AUTHOR]

MRI is a powerful tool to evaluate brain anatomy and function in normal children and its use in research applications has steadily increased. As imaging technology improves, and sensitivity to brain pathology increases, unanticipated (and potentially clinically important) findings on research neuroimaging studies will also increase. We evaluated the prevalence and type of unanticipated and potentially clinically significant imaging findings in a group of 114 normal children enrolled in an ongoing MRI imaging study of normal brain development for the Pediatric Functional Neuroimaging Research Network. Brain imaging findings were classified using standardized scales developed for the Network and findings were reported to participants and their primary healthcare provider according to a standard reporting pathway. Classification scales, reporting processes, and illustrated examples of findings are included and discussed. Unanticipated imaging findings were identified in approximately 12.5 % of children participating in this study. [ABSTRACT FROM AUTHOR]

There is a paucity of data regarding patterns of progression in children with high-grade glioma (HGG) or diffuse intrinsic pontine glioma (DIPG) treated with bevacizumab (BVZ) at diagnosis. We performed a retrospective study of 20 children with HGG or DIPG who received BVZ-based therapy at diagnosis on, or according to, a bi-institutional study. Magnetic resonance imaging (MRI) characteristics of first and most recent progressions were reviewed. Comparison was made to a control group of 19 patients who never received BVZ. Imaging definitions of progressive disease (PD) were local: at primary site or within 2 cm, contiguous; diffuse: >2 cm away but contiguous with primary site, ill-defined and infiltrative; distant: new, non-contiguous disease. In the BVZ-treated group, 14 patients had DIPG, six patients had HGG. Median age was 7 years (range: 3-21). Median time to PD and follow-up were 8.8 months (range 4-21) and 11 months (range: 6-25), respectively. Among 14 patients with PD, 8 (57.1 %) had local PD, 6 (42.9 %) had local and diffuse/distant PD, at initial progression. At most recent progression, a median of 10.8 months (range 6-25) from diagnosis, 10 of 14 (71.4 %) had at least diffuse (n = 8), or distant (n = 6) PD. In the comparable control group, 15 patients had PD: 11(73.3 %) local, 4 (26.7 %) local and diffuse/distant PD at first and most recent progressions. Based on these data, we postulate that BVZ may lead to a higher incidence of distant and diffuse disease in newly-diagnosed children with HGG or DIPG who received BVZ-based therapy. [ABSTRACT FROM AUTHOR]

Objective: Children suffering from epilepsy with suspected low-grade tumors may benefit from a surgical approach that considers the epileptogenic zone, which can be more extensive than the tumor region. This study aimed to determine the prevalence of epilepsy in children undergoing supratentorial tumor resection and the factors predictive of postoperative seizure freedom in children with low-grade tumors. Methods: Subjects 3 months to 21 years undergoing supratentorial brain tumor resection between 2007 and 2011 were included in this retrospective study. Children with supratentorial, cortically based tumors and a preoperative diagnosis of epilepsy were considered epilepsy surgery candidates. Pre- and postoperative MRI were reviewed and scored for extent of resection, adjacent dysplasia, and remaining abnormal cortex postoperatively. Results: The prevalence of seizures in all cases of supratentorial tumors was 46/87 (53 %). Eighteen were epilepsy surgery candidates. Eight of 18 (44 %) were seizure-free postoperatively with a mean follow-up of 39 months. Children who were seizure free postoperatively had tried fewer anticonvulsants than those with continued seizures (1.7 v. 2.9, p = 0.01). Presurgical evaluation was nonstandardized, and a more extensive workup and resection were performed in children who continued to have seizures postoperatively. Conclusions: All epilepsy surgery candidates had low-grade tumors on histological evaluation, indicating that a surgical approach that takes into consideration the epileptogenic zone is reasonable in this population. Gross total resection should be the goal, with additional attention to resection of the epileptogenic zone when located in the noneloquent cortex. [ABSTRACT FROM AUTHOR]

Introduction: Rotational vertebral artery occlusion (RVAO), sometimes known as 'Bow hunter syndrome,' is an important and diagnostically challenging cause of posterior circulation stroke in children. It is caused by impingement of osseous and/or ligamentous structures on the vertebral artery. Discussion: We review the differential diagnosis and recommended workup for posterior circulation stroke in children. The anatomical cause for pediatric RVAO is most commonly an anomalous bone formation of the rostral vertebral column. Many bony anomalies have been associated with RVAO, including the 'ponticulus posticus,' a common anomaly of the C1 vertebrae. The definitive diagnosis of RVAO is made with dynamic angiography, and surgical decompression of the vertebral artery prevents recurrent strokes. Illustrative case: We report a 15-year-old boy with recurrent posterior circulation ischemic strokes caused by an anomalous ossification of the C1 vertebrae. Three-dimensional (3D) and multiplanar reconstructed CT images showed the anomalous C1 ossification, and cervicocerebral digital subtraction angiography (DSA) revealed an associated vertebral artery (VA) dissection. The patient was initially managed by head immobilization in a hard collar and anticoagulation for 3 months. After healing of the dissection that was confirmed angiographically, dynamic DSA demonstrated reversible occlusion of the VA during contralateral head turn. Surgical decompression of the VA was performed and confirmed by intra-operative DSA with head turning. Conclusions: RVAO is an important diagnosis in children with posterior circulation stroke. Our case demonstrates the need for high clinical suspicion and careful analysis of 3D cervical spine CT images in children with posterior circulation stroke. [ABSTRACT FROM AUTHOR]

Controversy exists as to whether sinus thrombosis is the cause or the result of dural arteriovenous fistula (DAVF) and to whether DAVF are congenital or acquired lesions, especially in children. An infant presented with rupture of an anterior communicating artery aneurysm diagnosed with computed tomography angiography and catheter angiography. Pretreatment hospital course was complicated by extensive dural sinus thrombosis. Subsequent arteriography showed a new adult-type dural arteriovenous fistula to the previously thrombosed right sigmoid sinus. This is the first report of definitive angiographic documentation of the development of an adult-type DAVF after recanalization of a thrombosed dural sinus in a child. This case confirms the acquired etiology of at least one type of DAVF in children, even at this young age. We review the previously documented cases of formation of DAVF subsequent to sinus thrombosis with serial angiography in adults. [ABSTRACT FROM AUTHOR]

Functional MRI has become a critical research tool for evaluating brain function and developmental trajectories in children. Its clinical use in children is becoming more common. This presentation will review the basic underlying physiologic and technical aspects of fMRI, review research applications that have direct clinical relevance, and outline the current clinical uses of this technology. [ABSTRACT FROM AUTHOR]