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1.

Introduction

Brain mapping through a direct cortical and subcortical electrical stimulation during an awake craniotomy has gained an increasing popularity as a powerful tool to prevent neurological deficit while increasing extent of resection of hemispheric diffuse low-grade gliomas in adults. However, few case reports or very limited series of awake surgery in children are currently available in the literature.

Methods

In this paper, we review the oncological and functional differences between pediatric and adult populations, and the methodological specificities that may limit the use of awake mapping in pediatric low-grade glioma surgery.

Results

This could be explained by the fact that pediatric low-grade gliomas have a different epidemiology and biologic behavior in comparison to adults, with pilocytic astrocytomas (WHO grade I glioma) as the most frequent histotype, and with WHO grade II gliomas less prone to anaplastic transformation than their adult counterparts. In addition, aside from the issue of poor collaboration of younger children under 10 years of age, some anatomical and functional peculiarities of children developing brain (cortical and subcortical myelination, maturation of neural networks and of specialized cortical areas) can influence direct electrical stimulation methodology and sensitivity, limiting its use in children.

Conclusions

Therefore, even though awake procedure with cortical and axonal stimulation mapping can be adapted in a specific subgroup of children with a diffuse glioma from the age of 10 years, only few pediatric patients are nonetheless candidates for awake brain surgery.
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2.

Purpose of Review

Neuroimaging plays a critical role in diagnosis of brain tumors and in assessment of response to therapy. However, challenges remain, including accurately and reproducibly assessing response to therapy, defining endpoints for neuro-oncology trials, providing prognostic information, and differentiating progressive disease from post-therapeutic changes particularly in the setting of antiangiogenic and other novel therapies.

Recent Findings

Recent advances in the imaging of brain tumors include application of advanced MRI imaging techniques to assess tumor response to therapy and analysis of imaging features correlating to molecular markers, grade, and prognosis.

Summary

This review aims to summarize recent advances in imaging as applied to current diagnostic and therapeutic neuro-oncologic challenges.
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3.

Purpose

The aims of this study are to analyze how the nature and the behavior of low-grade glial tumors (LGGT) in children may correlate with the anatomy of the cerebral hemispheres and to evaluate the consequent impact of diffusion tensor imaging (DTI) techniques in the presurgical assessment.

Methods

This is a combined review of a series of 155 cases of LGGT and of the recent literature on the subject.

Results

The cases retrieved from our data bank were divided in central hemispheric tumors (basal ganglia and thalami) (36 cases), glioneuronal cortical-based tumors (49 cases), and glial tumors of the cerebral mantle (70 cases). A close correlation was found in the thalamus between the primary location of the tumor (juxta-ventricular, inferior, lateral, bilateral) and its extension (ventricular lumen, midbrain and mesial temporal, globus pallidus, respectively) which may relate to the connectivity. Among the glioneuronal tumors, most gangliogliomas were located in the temporal lobe and especially in the mesial temporal structures. In addition, the morphologic feature of the ganglioglioma was different there from the neocortical areas. As a complementary approach, DTI data may assist in evaluating the structure and the extension of the LGGT, in addition to planning the surgical strategy.

Conclusions

In the cerebral hemispheres like in the rest of the central nervous system, there is some degree of correlation between the anatomy and the nature, appearance, and behavior of the LGGT in children.
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4.

Purpose

In the present study, we evaluated the preoperative demographic, clinical, and neuropsychological variables that could predict postoperative seizure outcome in a group of pediatric epileptic patients.

Materials and methods

We studied 40 consecutive pediatric patients, ages ranging from 6 to 16 years, that underwent resective surgery for the treatment of medically intractable epilepsy at the Clinical Hospital of Ribeirão Preto School of Medicine. We performed ictal electroencephalography (EEG), interictal EEG, magnetic resonance imaging (MRI), and a preoperative neuropsychological assessment in the presurgical workup.

Results

The following factors were correlated with seizure outcome: (1) duration of epilepsy, (2) surgery localization, (3) localized Neuropsychological (NPS) Evaluation, (4) ictal EEG, (5) interictal EEG, and (6) MRI. Mental retardation, NPS tests, and the other demographic variables failed to correlate with seizure reduction.

Conclusions

The identification of predictor variables of epilepsy surgery outcome could improve the epileptic prognosis and guarantee the children's full potential development.
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5.

Background

Contrast-induced encephalopathy (CIE) is a syndrome that may be clinically unrecognized and misdiagnosed as cerebral edema.

Methods

Case report and review.

Results

A 72-year-old woman was admitted for elective endovascular embolization of a 10-mm left anterior communicating artery aneurysm. One hour post-procedure, she acutely developed global aphasia. Emergent head computed tomography (CT) and computed tomography-angiography (CTA) showed high attenuation of the left hemispheric subarachnoid spaces interpreted as hemispheric edema; emergent magnetic resonance imaging revealed left hemispheric punctate infarcts. At 12 h, she developed right hemiparesis and encephalopathy. Repeat CTA and CT perfusion revealed decreased left hemisphere cerebral blood flow and diminutive caliber of distal left middle cerebral artery territory vasculature. Repeated angiography with intra-arterial verapamil and systemic blood pressure augmentation were performed for presumed vasospasm. At 20 h, head CT was concerning for worsening left hemispheric edema, but dual-energy, iodine-subtracting sequences revealed significant contrast extravasation contributing to the appearance of sulcal effacement but without actual edema. Out of concern for blood–brain barrier breakdown from CIE, pressor augmentation was discontinued and the patient gradually improved to full neurological recovery within 72 h of symptom onset.

Conclusions

Our case is the first known to report the use of dual-energy, iodine-subtracting CT as a diagnostic tool in differentiating between cerebral edema and pseudoedema in CIE.
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6.

Background

Localization of the source of brain activity using an electroencephalograph (EEG) enables non-invasive visualization of the generators of epilepsy in an anatomical 3?D space. Initially the localization of interictal epileptic discharges with high-density EEG (hdEEG) was established, before techniques are also now being looked for, in order to localize ictal events.

Objective

This article summarizes the current state of the literature on localization of interictal discharges and gives an overview of the current questions.

Material and methods

The most important articles on various parameters, such as documentation of the discharge, marking the time point, role of head models and number of electrodes are discussed and the most important studies with patient cohorts are presented.

Results

In several studies with large patient cohorts, the localization of the source of interictal epileptic discharges was successfully proven with hdEEG. When this is combined with a head model based on the magnetic resonance imaging (MRI) of the patient, a high sensitivity (84%) and specificity (88%) can be achieved, which is superior to the other imaging techniques, such as MRI, PET and SPECT.

Conclusion

The EEG source localization of interictal epileptic discharges is precise and powerful. In view of the clinical relevance EEG source localization should be an established component in the presurgical clarification of patients with drug-refractory epilepsy, in addition to the other widely used imaging techniques.
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7.

Purpose

Paroxysmal sympathetic hyperactivity is a complication of brain injury that has mainly been described in the adult brain injury literature.

Methods

We present a case series of three pediatric patients that developed paroxysmal sympathetic hyperactivity of varying severity following hypoxic brain injury.

Results

Comparison of brain magnetic resonance imaging revealed bilateral and symmetric global ischemic changes in all three cases. However, the thalamus was not affected in the patient with the mild case of paroxysmal sympathetic hyperactivity. In contrast, bilateral and symmetric damage to the thalamus was observed in the two severe cases.

Conclusions

Our case series suggests that in hypoxic brain injury, evidence of bilateral ischemic injury to the thalamus on magnetic resonance imaging may be an important early predictor of severity and length of paroxysmal sympathetic hyperactivity. While this is an interesting observation, definite proof of our hypothesis requires further research including analysis of larger numbers of patients and comparison of MRI findings in children with hypoxic brain injury that do not develop paroxysmal sympathetic hyperactivity.
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8.

Objective

The objective of the present study was to retrospectively evaluate the relationship between tumor grading and a selective evaluation of neurocognitive and behavioral functions in children with supratentorial hemispheric brain tumors.

Methods

Children admitted with a diagnosis of supratentorial hemispheric tumors involving the cerebral hemispheres or the thalamus at the Pediatric Neurosurgery Unit of the Catholic University of Rome between January 2008 and January 2014 were considered for the present study. Exclusion criteria were represented by age less than 2 years, severe neurological deficits, seizures, and a metastatic disease. A selective neurocognitive and behavioral workout was used for children aged less and more than 5 years.

Results

Global cognitive functions as well as selective neurocognitive and behavioral profiles were found to be significantly worse in children with low-grade tumors, compared with those affected by higher-grades histotypes. Frontal locations for cortical tumors and thalamic lesions were significantly related with worse results, with a clear contribution of dominant vs. nondominant hemisphere involvement and an age higher than 5 years.

Conclusions

Preoperative global and selective neurocognitive evaluation might contribute to the prediction of the tumor aggressiveness. Due to a longer clinical history, more benign tumors more frequently arrive to the diagnosis with a neurocognitive compromise in spite of an apparently mild presence of neurological symptoms and signs.
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9.

Introduction

The treatment of low-grade gliomas (LGGs) in pediatric age is still controversial. However, most authors report longer life expectancy in case of completely removed cerebral gliomas. Intraoperative magnetic resonance imaging (iMRI) is increasingly utilized in the surgical management of intra-axial tumor in adults following the demonstration of its effectiveness. In this article, we analyze the management of LGG using iMRI focusing on its impact on resection rate and its limits in the pediatric population.

Methods

We performed review of the literature regarding the treatment of LGG using iMRI focusing on its impact on resection rate and its limits in the pediatric population. Some exemplary cases are also described.

Results

Intraoperative MRI allowed extension of tumor resection after the depiction of residual tumor at the intraoperative imaging control from 21 to 52 % of the cases in the published series. Moreover, the early reoperation rate was significantly lower when compared with the population treated without this tool (0 % vs 7–14 %). Some technical difficulties have been described in literature regarding the use of iMRI in the pediatric population especially for positioning due to the structure of the headrest coil designed for adult patients.

Conclusion

The analysis of the literature and our own experience with iMRI in children indicates significant advantages in the resection of LGG offered by the technique. All these advantages are obtained without elongation of the surgical times or increased risk for complications, namely infection. The main limit for a wider diffusion of iMRI for the pediatric neurosurgical center is the cost required, for acquisition of the system, especially for high-field magnet, and the environmental and organizational changes necessary for its use.
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10.

Introduction

Most functional magnetic resonance imaging (fMRI) studies of taste deliver small quantities of liquids over roughly 45 min to repeatedly sample brain response to tastants. Within this time participants frequently report that their need to urinate increases.

Methods

Since both gustatory and interoceptive information are represented in the anterior insular cortex, we evaluated whether perceived need to urinate influenced insular responses to the receipt of a small bolus of milkshake in two datasets (n?=?45).

Results

Change in pre- to post-scan ratings of desire to urinate was inversely related to anterior insular response to milkshake.

Conclusion

This finding demonstrates that micturition drive influences insular response to milkshake and supports previous reports of overlapping gustatory and visceral representation within human anterior insular cortex.
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11.

Introduction

Despite recent improvements in perinatal care, the incidence of neonatal brachial plexus palsy (NBPP) remains relatively common. CT myelography is currently considered to be the optimal imaging modality for evaluating nerve root integrity. Recent improvements in MRI techniques have made it an attractive alternative to evaluate nerve root avulsions (preganglionic injuries).

Aim

We demonstrate the utility of MRI for the evaluation of normal and avulsed spinal nerve roots. We also show the utility of ultrasound in providing useful preoperative evaluation of the postganglionic brachial plexus in patients with NBPP.
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12.

Objective

L-2-hydroxyglutaric aciduria is a genetic metabolic disorder. Its clinical features include elevated levels of hydroxyglutaric acid in body fluids and abnormal magnetic resonance imaging (MRI) in the subcortical white matter, which are affected by the accumulation of L-2-hydroxyglutaric acid.

Method

A boy with psychomotor retardation and progressive ataxia accompanied by abnormal brain MRI findings was tested using whole-exome sequencing.

Results

Next-generation sequencing (NGS) revealed two novel compound heterozygous frameshift mutations, c.407 del A (p.K136SfsTer3) and c.699_c700 ins A (p.D234RfsTer42), in the L-2-hydroxyglutarate dehydrogenase (L2HGDH) gene, leading to premature termination codons and truncated FAD/NAD(P)-binding domain of L2HGDH protein. Further laboratory testing revealed an increase in the 2-hydroxyglutaric acid level in the urine.

Conclusion

The results suggested that NGS could provide clues for identifying patients with abnormal neuroradiological findings in the subcortical white matter.
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13.
14.

Background

Growing skull fracture (GSF) is a rare complication of pediatric head trauma and causes delayed onset neurological deficits and cranial defect. GSF usually develops following linear fracture with underlying dural tear resulting in herniation of the brain. Early diagnosis and treatment are essential to avoid complications. However, there are no clear-cut guidelines for the early diagnosis of GSF. The present study was conducted to identify the criteria for the early diagnosis of GSF.

Material and methods

From 2010 to 2015, all pediatric patients of head trauma with linear fracture were evaluated. Patients of age <5 years with cephalhematoma, bone diastasis of 4 mm or more with underlying brain contusion were subjected to contrast brain MRI to find out the dural tear and herniation of the brain matter. Patients with contrast MRI showing dural tear and herniation of the brain matter were considered high risk for the development of GSF and treated surgically within 1 month of trauma. Patients with contrast brain MRI not showing dural tear and herniation of the brain matter were regularly followed for any signs of GSF.

Results

A total of 20 patients were evaluated, out of which 16 showed dural defects with herniation of the brain matter and were subjected to duraplasty. Four patients in which MRI did not show dural tear and herniation of the brain matter were regularly followed-up and have not shown any sign of GSF later on follow-up.

Conclusion

Early diagnosis of GSF can be made based on the four criteria, i.e., (1) age <5 year with cephalhematoma, (2) bone diastasis 4 mm or more (3) underlying brain contusion (4) contrast MRI showing dural tear and herniation of the brain matter. Dural tear with herniation of the brain matter is the main etiopathogenic factor for the development of GSF. Early diagnosis and treatment of GSF can yield a good outcome.
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15.

Background

Brain imaging is one of the most important diagnostic methods for evaluating headache during pregnancy. In this study, we aimed to identify anamnestic and clinical predictors for pathological brain imaging findings in pregnant women suffering from acute headache.

Methods

We conducted a retrospective chart review of 151 pregnant women with acute headache between 2010 and 2016. We screened the medical records of these patients and analyzed radiological variables, including brain imaging frequency and modality, delay to imaging and imaging findings. In patients with brain imaging, we compared several anamnestic and clinical features between those with and without symptomatic pathological findings.

Results

Half of the patients (50.3%) underwent brain imaging, mainly magnetic resonance imaging (MRI) including venography (53.9%) or MRI including both venography and arteriography (31.6%). Symptomatic pathological results could be observed in 27.6% of the patients with a brain scan. Patients in the first trimester with acute headache had a statistically higher risk for a symptomatic pathological imaging finding (p?=?0.024). Strong pain intensity, a reduced level of consciousness and seizures were significantly associated with a symptomatic pathological imaging outcome across all stages of pregnancy.

Conclusion

Brain imaging may reveal a secondary headache etiology in more than 25% of pregnant women presenting with acute headache. In particular, when the acute headache is of severe intensity, and the headache is accompanied by seizures or a change in consciousness, brain imaging is required.
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16.

Background

About 23 million people worldwide suffer from medically refractory epilepsy. Surgery might be the best treatment option with a reasonable chance of seizure freedom. Surgical success depends on the exact definition of the epileptogenic zone (EZ). Magnetoencephalography (MEG) is one of the newer additions to the noninvasive presurgical work-up.

Objectives

This study gives an overview of the impact of MEG on the management of epilepsy patients, focusing on (1) the influence on presurgical evaluation, (2) the identification of patients with the greatest benefit, and (3) possible surgical outcome predictors.

Methods

An extensive Medline literature search was conducted for studies published from 1990.

Results

MEG is in clinical use in the presurgical evaluation of epilepsies for the identification of the EZ and outcome prediction. In cases of failed surgery, it serves as a means to locate the remaining epileptogenic cortex. The usefulness of MEG has been reported for a wide range of localizations including challenging areas like the insula. In cases of multiple possible culprit lesions, MEG can mark the epileptogenic lesion, whereas in cases of nonlesional magnetic resonance imaging (MRI) findings, MEG can pinpoint a lesional or nonlesional epileptogenic cortex area. The role of MEG in the presurgical evaluation of epilepsy was shown with rates of modified approaches in 20–35% of cases. This holds true especially for cases with extratemporal epilepsy.

Discussion

The value of MEG source localization is highest in extratemporal epilepsy, in MRI-negative or multilesional cases, if other modalities yield contradictory or inconclusive results, or in cases of suspected multifocal epilepsy. There is clear evidence that MEG yields nonredundant information and influences the therapeutic course of patients. Various patient groups likely to benefit from MEG were identified. Considering the poor chances of seizure freedom with continued medical treatment, these patients should not be denied source localization, which could result in surgery with favorable outcomes.
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17.

Purpose

The aim of this study was to highlight the challenges for early diagnosis and the difficulties observed in surgical treatment of patients with transsphenoidal meningoencephalocele associated with cleft lip and/or palate.

Method

We evaluated six male patients treated over the course of 4 years. Five patients presented encephalic herniation with nonfunctional brain tissue; one of these presented herniation of the pituitary gland and cerebral ventricles.

Results

All the patients received surgical treatment for the cleft lip and/or palate. Only one patient underwent repair of the meningoencephalocele, via nasal endoscopy. There were no postprocedural clinical or surgical complications.

Conclusion

The tendency is to avoid neurosurgery, opting for periodic follow-up with magnetic resonance imaging. In the presence of cleft palate, palatoplasty is essential to protect the meningoencephalocele.
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18.

Background

Ictal electroencephalogram (EEG) source imaging has both advantages and disadvantages compared with source imaging of interictal epileptiform discharges. Ictal source imaging estimates the seizure onset zone directly. However, the rapid propagation of the ictal activity and the low signal-to-noise ratio impose additional challenges on ictal source imaging. Several methods have been developed to circumvent these challenges.

Objectives

To summarize and explain in plain terms the methods of ictal EEG source imaging and to review the published evidence on its accuracy.

Materials and methods

We systematically searched Medline for studies of ictal EEG source imaging. In addition, we summarize our clinical experience with ictal EEG source imaging and we present illustrative examples for the analysis process.

Results

Pooled data from 77 operated patients, from four clinical studies, showed that ictal EEG source imaging had a sensitivity of 83.3% (95% confidence interval: 69.8–92.5%) and specificity of 72.4% (95% confidence interval: 52.8–87.3%).

Conclusion

Ictal EEG source imaging is accurate and it should be added to the multimodal presurgical evaluation of patients with drug-resistant focal epilepsy.
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19.

Purpose of Review

Previous literature has shown inconsistent findings regarding the effects of neurosteroids on the brain in postmenopausal women. The goal of this paper is to examine how and whether advances in neuroimaging have helped elucidate the relationship between the withdrawal of and/or treatment with neurosteroids and cognition at menopause.

Recent Findings

Neuroimaging techniques such as structural and functional MRI have been used in recent studies to examine the relationship between neurosteroids and brain structure and functioning. However, the recent literature shows that different formulations of postmenopausal hormones given at different times, through different routes of administration, and in different combinations with progestins result in a variety of relationships with the brain outcomes.

Summary

We suggest that still further research is needed to understand how the structural changes resulting from estrogen withdrawal or therapy at menopause can influence cognitive functioning. However, imaging studies are time-, resource-, and expertise-intensive. We believe that this information will help uncover the mechanisms and relationships that can aid in the explanation of the individual differences in the effects of menopause on the brain as well as how this menopause-related hormone change influences risk for pathological aging.
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20.

Purpose of Review

Clinical trials using agents directed at neuroprotection and remyelination in multiple sclerosis (MS) are needed. As optic neuritis (ON) is common in people with MS and the pathology of ON is similar to other MS lesions in the brain, measurements of the anterior visual system are frequently utilized in neuroprotection and remyelination trials. Understanding the strengths and weaknesses of the measurements is vital when interpreting the results of this research.

Recent Findings

Techniques such as visual evoked potentials (VEP) and optical coherence tomography (OCT) are well established in MS and are thought to measure axonal integrity and myelination. Novel imaging techniques can also be used in conjunction with these measurements to provide better insight into optic nerve structure and function. Magnetization transfer imaging (MTR) together with optic nerve area and volume measures neurodegeneration; diffusion tensor imaging (DTI) measures myelination status and neurodegeneration. However, these techniques require various levels of experience to interpret, and all can be confounded by ocular motion and surrounding fat and bone.

Summary

This article provides a review of established and novel techniques to measure the anterior visual system in multiple sclerosis with a focus on the evidence to support their use as outcome measures in clinical trials focused on neuroprotection and remyelination therapies.
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