Brain-wide neuronal circuit connectome of human glioblastoma.

Glioblastoma (GBM) infiltrates the brain and can be synaptically innervated by neurons, which drives tumor progression. Synaptic inputs onto GBM cells identified so far are largely short-range and glutamatergic. The extent of GBM integration into the brain-wide neuronal circuitry remains unclear.

Transorbital intraoperative electrocorticography-guided surgical resection for medically refractory developmental epileptic encephalopathy with spike-wave activation in sleep.

Developmental epileptic encephalopathy with spike-wave activation in sleep (DEE-SWAS) is an epilepsy syndrome of childhood characterized by developmental delay or regression with significant abnormal epileptiform activity during sleep. In some cases, DEE-SWAS with an identified focal lesion is treated with surgical resection. The authors report an unusual case of focal DEE-SWAS that was successfully treated via transorbital resection with intraoperative electrocorticography (iECoG).

Endoscopic endonasal resection of olfactory tract hamartoma for pediatric epilepsy.

Background: Non-hypothalamic glioneural hamartomas are rare entities known to cause medically refractory epilepsy. Olfactory bulb hamartomas, in particular, are exceptionally rare.

Methods: We describe a case of an olfactory bulb hamartoma that was surgically resected at our institution.

Intracranial complications secondary to acute bacterial sinusitis requiring neurosurgical intervention before and after the onset of the COVID-19 pandemic.

Objective: Intracranial complications of acute bacterial sinusitis are rare pathologies that occur in children, and are associated with significant neurological morbidity and mortality. There is a subjective concern among neurosurgeons that the incidence of this rare disease has increased since the onset of the novel COVID-19 pandemic. The primary objective of this study was to review the presentation and management of patients admitted at the authors' institution with intracranial extension of sinusitis, to better understand the local disease burden relative to the COVID-19 pandemic.

Development of Consensus-Based Best Practice Guidelines for the Perioperative and Postoperative Care of Pediatric Patients With Spinal Deformity and Programmable Implanted Devices.

Study Design: Modified Delphi consensus study.

Objective: To develop consensus-based best practices for the care of pediatric patients who have implanted programmable devices (IPDs) and require spinal deformity surgery.

Summary Of Background Data: Implanted programmable devices (IPDs) are often present in patients with neuromuscular or syndromic scoliosis who require spine surgery.

Short report: Twins with 20p13 duplication. Case report and comprehensive literature review.

Background: Trisomy 20p is a rare genetic condition caused by a duplication of the short arm of chromosome 20.

Methods: We employed clinical observation and molecular genetic testing (SNP microarray), to study identical twin males with an unknown dysmorphic syndrome. We conducted a literature review of trisomy 20p and collated the clinical and molecular genetic findings on 20 affected subjects reported since 2000.

Timing of intraoperative neurophysiological monitoring (IONM) recovery and clinical recovery after termination of pediatric spinal deformity surgery due to loss of IONM signals.

Background Context: Intraoperative neurophysiological monitoring (IONM) is used to reduce the risk of spinal cord injury during pediatric spinal deformity surgery. Significant reduction and/or loss of IONM signals without immediate recovery may lead the surgeon to acutely abort the case. The timing of when monitorable signals return remains largely unknown.

Advanced approaches in Pediatric Epilepsy surgery.

While recent technological advancements are reshaping the landscape of surgical epilepsy management, the established techniques of resective and disconnective surgeries guided by electrographic monitoring remain the workhorse interventions for the management of refractory seizures and have the highest likelihood of achieving complete seizure resolution. Here we discuss examples of recent developments in surgical approaches and techniques for resective and disconnective surgeries with discussion of their indications and potential advantages.

Systematic review and cumulative analysis of clinical properties of BRAF V600E mutations in PLNTY histological samples.

Purpose: Polymorphous low-grade neuroepithelial tumors of the young (PLNTY) represent a rare pediatric-type tumor that most commonly presents as medically refractory epilepsy. PLNTY has only recently been recognized as a distinct clinical entity, having been first described in 2016 and added to the World Health Organization classification of CNS tumors in 2021. Molecular studies have determined that PLNTY is uniformly driven by aberrant MAPK pathway activation, with most tumors carrying either a BRAF V600E mutation or activating FGFR2 or FGFR3 fusion protein.

Middle meningeal artery embolization in the management of chronic subdural hematoma in medically complex pediatric neurosurgical patients: technical note.

Middle meningeal artery (MMA) embolization has gained acceptance as a treatment for chronic subdural hematoma (cSDH) in adult patients but has not been well described in pediatric patients. Standard cSDH treatment has historically consisted of burr hole drainage with or without subdural drain placement. However, due to the high rate of recurrence and frequency of comorbidities within this population, as both pediatric and adult patients with cSDH frequently have concurrent cardiac disease and a need for anticoagulant therapies, MMA embolization has increasingly demonstrated its value as both an adjunctive and primary treatment.

Magnetic resonance imaging-guided stereotactic laser ablation therapy for the treatment of pediatric epilepsy: a retrospective multiinstitutional study.

Objective: The authors of this study evaluated the safety and efficacy of stereotactic laser ablation (SLA) for the treatment of drug-resistant epilepsy (DRE) in children.

Methods: Seventeen North American centers were enrolled in the study. Data for pediatric patients with DRE who had been treated with SLA between 2008 and 2018 were retrospectively reviewed.

A recurrent de novo splice site variant involving DNM1 exon 10a causes developmental and epileptic encephalopathy through a dominant-negative mechanism.

Heterozygous pathogenic variants in DNM1 cause developmental and epileptic encephalopathy (DEE) as a result of a dominant-negative mechanism impeding vesicular fission. Thus far, pathogenic variants in DNM1 have been studied with a canonical transcript that includes the alternatively spliced exon 10b. However, after performing RNA sequencing in 39 pediatric brain samples, we find the primary transcript expressed in the brain includes the downstream exon 10a instead.

A single-cell transcriptome atlas of glial diversity in the human hippocampus across the postnatal lifespan.

The molecular diversity of glia in the human hippocampus and their temporal dynamics over the lifespan remain largely unknown. Here, we performed single-nucleus RNA sequencing to generate a transcriptome atlas of the human hippocampus across the postnatal lifespan. Detailed analyses of astrocytes, oligodendrocyte lineages, and microglia identified subpopulations with distinct molecular signatures and revealed their association with specific physiological functions, age-dependent changes in abundance, and disease relevance.

Stereoelectroencephalography before 2 years of age.

Objective: Stereoelectroencephalography (SEEG) is a widely used technique for localizing seizure onset zones prior to resection. However, its use has traditionally been avoided in children under 2 years of age because of concerns regarding pin fixation in the immature skull, intraoperative and postoperative electrode bolt security, and stereotactic registration accuracy. In this retrospective study, the authors describe their experience using SEEG in patients younger than 2 years of age, with a focus on the procedure's safety, feasibility, and accuracy as well as surgical outcomes.

Molecular landscapes of human hippocampal immature neurons across lifespan.

Immature dentate granule cells (imGCs) arising from adult hippocampal neurogenesis contribute to plasticity and unique brain functions in rodents and are dysregulated in multiple human neurological disorders. Little is known about the molecular characteristics of adult human hippocampal imGCs, and even their existence is under debate. Here we performed single-nucleus RNA sequencing aided by a validated machine learning-based analytic approach to identify imGCs and quantify their abundance in the human hippocampus at different stages across the lifespan.

Stereoelectroencephalography in the very young: Case report.

Stereoelectroencephalography (SEEG) is an increasingly popular invasive monitoring approach to epilepsy surgery in patients with drug-resistant epilepsies. The technique allows a three-dimensional definition of the epileptogenic zones (EZ) in the brain. It has been shown to be safe and effective in adults and older children but has been used sparingly in children less than two years old due to concerns about pin fixation in thin bone, registration accuracy, and bolt security.

Risk of ventriculoperitoneal shunt malfunction in operatively treated early onset spinal deformity.

Purpose: Ventriculoperitoneal (VP) shunt placement is a common neurosurgical procedure performed in patients with early onset scoliosis (EOS). To provide insight into the risks of spine lengthening operations, we investigate the rate of VP shunt complications in patients with EOS undergoing spinal deformity correction interventions.

Methods: A retrospective review was performed of all patients with EOS at a single institution undergoing spinal deformity correction procedures from 2007 to 2018.

The Surgical Learning Curve for Modified Lapidus Procedure for Hallux Valgus Deformity.

Background: Hallux valgus is one of the most common orthopaedic deformities of the foot, affecting as much as 23% of the population age 18 to 65. In addition to its high prevalence, it has a complex multifactorial pathogenesis. Surgical correction options have variable rates of success and new techniques are being developed.

Acute neurological injury in pediatric patients with single-ventricle congenital heart disease.

Objective: Single-ventricle congenital heart disease (CHD) in pediatric patients with Glenn and Fontan physiology represents a unique physiology requiring the surgical diversion of the systemic venous return from the superior vena cava (Glenn) and then the inferior vena cava (Fontan) directly to the pulmonary arteries. Because many of these patients are on chronic anticoagulation therapy and may have right-to-left shunts, arrhythmias, or lymphatic disorders that predispose them to bleeding and/or clotting, they are at risk of experiencing neurological injury requiring intubation and positive pressure ventilation, which can significantly hamper pulmonary blood flow and cardiac output. The aim of this study was to describe the complex neurological and cardiopulmonary interactions of these pediatric patients after acute central nervous system (CNS) injury.