G-quadruplexes are a source of vulnerability in deficient granule cell progenitors and medulloblastoma.

Biallelic pathogenic variants in the essential DNA repair gene causes Fanconi anemia, complementation group FA-D1. Patients in this group are highly prone to develop embryonal tumors, most commonly medulloblastoma arising from the cerebellar granule cell progenitors (GCPs). GCPs undergo high proliferation in the postnatal cerebellum under SHH activation, but the type of DNA lesions that require the function of the BRCA2 to prevent tumorigenesis remains unknown.

Structural and functional connectivity in hydrocephalus: a scoping review.

Optimizing the treatment of hydrocephalus remains a major challenge in adult and pediatric neurosurgery. Currently, clinical treatment relies heavily on anatomic imaging of ventricular size and clinical presentation. The emergence of functional and structural brain connectivity imaging has provided the basis for a new paradigm in the management of hydrocephalus.

Natural history of craniovertebral abnormalities in a single-center study in 54 patients with Hurler syndrome.

Objective: Craniovertebral junction (CVJ) abnormalities are common and well documented in mucopolysaccharidosis type I-Hurler syndrome (MPS IH), often causing severe spinal canal narrowing. However, the requirement for surgical decompression and/or fusion is uncommon. Although hematopoietic cell transplant (HCT) has been shown to prolong the lives of patients with MPS IH, its effect in halting or reversing musculoskeletal abnormalities is less clear.

Current state and future direction of quality improvement in pediatric neurosurgery: a survey of pediatric neurosurgeons.

Objective: Quality improvement (QI) is a methodology used to implement sustainable, meaningful change to improve patient outcomes. Given the complex pathologies observed in pediatric neurosurgery, QI projects could potentially improve patient care. Overall, there is a need to characterize the degree of QI opportunities, training, and initiatives within the field of pediatric neurosurgery.

Wide Arterial Sparing Encephalo-Duro-Synangiosis for Moyamoya: Surgical Technique and Outcomes.

Background: Moyamoya is managed by surgical revascularization, but no standardized method has yet been universally adopted.

Objective: To describe a new indirect bypass technique for pediatric moyamoya, wide arterial sparing encephalo-duro-synangiosis (WASEDS), which provides a much wider area of revascularization with minimal compromise to the middle meningeal arterial tree compared with traditional procedures. Initially used as a salvage technique after failed encephalo-duro-arterio-synangiosis, its success later motivated its use as a first-line procedure.

Anterior versus Posterior Ventricular Catheter Placement in Pediatric Patients: A Systematic Review and Meta-Analysis.

Background: Ventriculoperitoneal shunt placement is the mainstay of treatment for hydrocephalus, but there are relatively high rates of malfunction. Shunt catheter entry can be performed anteriorly or posteriorly, with the body of evidence from randomized controlled trials and retrospective studies suggesting conflicting findings.

Methods: A systematic review of PubMed, Medline, Scopus, and Web of Science was performed adherent to PRISMA guidelines, searching for clinical studies examining outcomes for anterior or frontal and posterior or occipital ventriculoperitoneal shunt placement.

Learning Curve Associated with ClearPoint Neuronavigation System: A Case Series.

Background: The ClearPoint neuronavigation system affords real-time magnetic resonance imaging (MRI) guidance during stereotactic procedures. While such information confers potential clinical benefits, additional operative time may be needed.

Methods: We conducted a retrospective analysis of procedural time associated with ClearPoint Stereotaxis, with hypothesis that this procedural time is comparable with that associated with frame-based biopsy.

Stereo-electroencephalography (SEEG) in pediatric epilepsy: Utility in children with and without prior epilepsy surgery failure.

Background: When noninvasive modalities fail to adequately localize the seizure onset zone (SOZ) in children with medically refractory epilepsy, invasive interrogation with stereo-electroencephalography (SEEG) or subdural electrodes may be required. Our center utilizes SEEG for invasive monitoring in a carefully selected population of children, many of whom have seizures despite a prior surgical resection. We describe the cohort of patients who underwent SEEG in the first 5 years of its employment in our institution, almost half of which had a history of a failed epilepsy surgery.

Autologous Calvarial Bone Remodeling Technique for Small to Medium-Sized Cranial Defects in Young Children: The "Switch-Cranioplasty" Technique.

Introduction: Reconstruction of cranial defects in children less than 2 years of age, particularly when there is an associated dural defect, is challenging due to the need to accommodate active skull growth, limited options for autologous bone graft and thin calvarial bones. We use a simple remodeling technique that exploits the normal dura's inherent potential for new bone growth while covering the dural defect with adjacent skull.

Case Presentation: We describe an alternating, two-piece craniotomy or "switch-cranioplasty technique" to repair an occipital meningocele.

Outcomes in children undergoing posterior fossa decompression and duraplasty with and without tonsillar reduction for Chiari malformation type I and syringomyelia: a pilot prospective multicenter cohort study.

Objective: Despite significant advances in diagnostic and surgical techniques, the surgical management of Chiari malformation type I (CM-I) with associated syringomyelia remains controversial, and the type of surgery performed is surgeon dependent. This study's goal was to determine the feasibility of a prospective, multicenter, cohort study for CM-I/syringomyelia patients and to provide pilot data that compare posterior fossa decompression and duraplasty (PFDD) with and without tonsillar reduction.

Methods: Participating centers prospectively enrolled children suffering from both CM-I and syringomyelia who were scheduled to undergo surgical decompression.

Presurgical hyperconnectivity of the ablation volume is associated with seizure-freedom after magnetic resonance-guided laser interstitial thermal therapy.

Purpose: Magnetic Resonance-guided Laser Interstitial Thermal Therapy (MRgLITT) is an emerging minimally-invasive alternative to resective surgery for medically-intractable epilepsy. The precise lesioning effect produced by MRgLITT supplies opportunities to glean insights into epileptogenic regions and their interactions with functional brain networks. In this exploratory analysis, we sought to characterize associations between MRgLITT ablation zones and large-scale brain networks that portended seizure outcome using resting-state fMRI.

Non contrast, Pseudo-Continuous Arterial Spin Labeling and Accelerated 3-Dimensional Radial Acquisition Intracranial 3-Dimensional Magnetic Resonance Angiography for the Detection and Classification of Intracranial Arteriovenous Shunts.

Objectives: The aim of this study was to assess the sensitivity and specificity of pseudo-continuous arterial spin labeling (PCASL) magnetic resonance angiography (MRA) with 3-dimensional (3D) radial acquisition for the detection of intracranial arteriovenous (AV) shunts.

Materials And Methods: A total of 32 patients who underwent PCASL-MRA, clinical magnetic resonance imaging (MRI)/MRA exam, and digital subtraction angiography (DSA) were included in this retrospective analysis. Twelve patients presented with AV shunts.

Shunt-dependent hydrocephalus: management style among members of the American Society of Pediatric Neurosurgeons.

OBJECTIVE The authors conducted a survey to evaluate differences in the understanding and management of shunt-dependent hydrocephalus among members of the American Society of Pediatric Neurosurgeons (ASPN). METHODS Surveys were sent to all 204 active ASPN members in September 2014. One hundred thirty responses were received, representing a 64% response rate.

Histopathologic Validation of Grayscale Carotid Plaque Characteristics Related to Plaque Vulnerability.

Inflammation and angiogenesis play major roles in carotid plaque vulnerability. The purpose of this study was to determine whether gray-scale features of carotid plaques are associated with histologic markers for inflammation. Thirty-eight individuals completed a dedicated research carotid ultrasound exam before carotid endarterectomy.

Cognitive Deficits in Symptomatic and Asymptomatic Carotid Endarterectomy Surgical Candidates.

The role played by vessel disease in stroke-related cognition dysfunction is unclear. We assessed the impact of significant atherosclerotic disease on cognition-even in patients asymptomatic for stroke. We hypothesized that patients would perform poorly relative to controls, but that symptomatic/asymptomatic status (history of stroke/transient ischemic attack) would have no effect.

4D DSA a new technique for arteriovenous malformation evaluation: a feasibility study.

Background: The angioarchitectural features of an arteriovenous malformation (AVM) provide key information regarding natural history and treatment planning. Because of rapid filling and vascular overlap, two-dimensional (2D) and three-dimensional (3D) digital subtraction angiography (DSA) are often suboptimal for evaluation of these features. We have developed an algorithm that derives a series of fully time-resolved 3D DSA volumes (four-dimensional (4D) DSA) at up to 30 frames/s from a conventional 3D DSA.

Immune modulation by Lacto-N-fucopentaose III in experimental autoimmune encephalomyelitis.

Parasitic infections frequently lead to immune deviation or suppression. However, the application of specific parasitic molecules in regulating autoimmune responses remains to be explored. Here we report on the immune modulatory function of Lacto-N-fucopentaose III (LNFPIII), a schistosome glycan, in an animal model for multiple sclerosis.

Plasticity of Ly-6C(hi) myeloid cells in T cell regulation.

CD11b(+)Ly-6C(hi) cells, including inflammatory monocytes (IMCs) and inflammatory dendritic cells (IDCs), are important in infectious, autoimmune, and tumor models. However, their role in T cell regulation is controversial. In this article, we show that T cell regulation by IMCs and IDCs is determined by their activation state and is plastic during an immune response.

Validation study of human figure drawing test in a Colombian school children population.

The aim of this article was to assess the validity of the emotional and developmental components of the Koppitz human figure drawing test. 2420 children's drawings available in a database resulting from a previous cross sectional study designed to determine the prevalence of neurological diseases in children between 0 and 12 years old in Bogota schools were evaluated. They were scored using the criteria proposed by Koppitz, and classified into 16 groups according to age, gender, and presence/absence of learning or attention problems.