Multimodal evaluation of network activity and optogenetic interventions in human hippocampal slices.

Seizures are made up of the coordinated activity of networks of neurons, suggesting that control of neurons in the pathologic circuits of epilepsy could allow for control of the disease. Optogenetics has been effective at stopping seizure-like activity in non-human disease models by increasing inhibitory tone or decreasing excitation, although this effect has not been shown in human brain tissue. Many of the genetic means for achieving channelrhodopsin expression in non-human models are not possible in humans, and vector-mediated methods are susceptible to species-specific tropism that may affect translational potential.

The sensitivity of limited-sequence magnetic resonance imaging in identifying pediatric cervical spine injury: A Western Pediatric Surgery Research Consortium multicenter retrospective cohort study.

Introduction: Clinical clearance of a child's cervical spine after trauma is often challenging because of impaired mental status or an unreliable neurologic examination. Magnetic resonance imaging (MRI) is the criterion standard for excluding ligamentous injury in children but is constrained by long image acquisition times and frequent need for anesthesia. Limited-sequence magnetic resonance imaging (LSMRI) is used in evaluating the evolution of traumatic brain injury and may also be useful for cervical spine clearance while potentially avoiding the need for anesthesia.

Pediatric neuromodulation for drug-resistant epilepsy: Survey of current practices, techniques, and outcomes across US epilepsy centers.

Neuromodulation via Responsive Neurostimulation (RNS) or Deep Brain Stimulation (DBS) is an emerging treatment strategy for pediatric drug-resistant epilepsy (DRE). Knowledge gaps exist in patient selection, surgical technique, and perioperative care. Here, we use an expert survey to clarify practices.

Epilepsy surgery in children with genetic etiologies: A prospective evaluation of current practices and outcomes.

Objective: This study assesses current practices and outcomes of epilepsy surgery in children with a genetic etiology. It explores the pre-surgical workup, types of surgeries, and post-surgical outcomes in a broad array of disorders.

Methods: Patients ≤18 years who completed epilepsy surgery and had a known genetic etiology prior to surgical intervention were extrapolated from the Pediatric Epilepsy Research Consortium (PERC) surgery database, across 18 US centers.

Pediatric neuroblastoma with intraspinal extension: the role of surgical management.

Objective: Neuroblastoma with spinal involvement accounts for up to 30% of pediatric spinal tumors and can cause profound neurological deficits. Chemotherapy is the preferred treatment option, but in select patients resection may be indicated. The goal of this study was to identify preoperative factors that led to early surgical intervention, with a specific emphasis on identifying differences on long-term neurological function and spinal deformity in the recent treatment era.

A multi-center comparison of surgical techniques for corpus Callosotomy in pediatric drug-resistant epilepsy.

Objectives: Corpus callosotomy (CC) is used to reduce seizures, primarily in patients with generalized drug-resistant epilepsy (DRE). The invasive nature of the procedure contributes to underutilization despite its potential superiority to other palliative procedures. The goal of this study was to use a multi-institutional epilepsy surgery database to characterize the use of CC across participating centers.

Responsive Neurostimulation in Drug-Resistant Pediatric Epilepsy: Findings From the Epilepsy Surgery Subgroup of the Pediatric Epilepsy Research Consortium.

Background: Responsive neurostimulation (RNS), a closed-loop intracranial electrical stimulation system, is a palliative surgical option for patients with drug-resistant epilepsy (DRE). RNS is approved by the US Food and Drug Administration for patients aged ≥18 years with pharmacoresistant partial seizures. The published experience of RNS in children is limited.

Clarifying the clinical landscape of pediatric spinal arteriovenous shunts: an institutional experience and individual patient-data meta-analysis.

Background: Pediatric spinal arteriovenous shunts (SAVS) are rare lesions with heterogeneous pathogenesis and clinical manifestations.

Objective: To evaluate the clinical characteristics, angioarchitecture, and technical/clinical outcomes in SAVS through a large single-center cohort analysis and meta-analysis of individual patient data.

Methods: A retrospective institutional database identified children (aged 0-21 years) who underwent digital subtraction spinal angiography (DSA) for SAVS between January 1996 and July 2021.

A comparison of incidental and symptomatic unruptured brain arteriovenous malformations in children.

Objective: Patients with unruptured brain arteriovenous malformations (AVMs) may present with headaches, seizures, and/or neurological deficits. A smaller number of cases may be discovered incidentally. These lesions remain incompletely understood due to their sparse reporting.

somatic mosaicism in a patient with bilateral optic nerve sheath meningiomas: illustrative case.

Background: In the past decade, next-generation sequencing has spurred significant progress in the understanding of cytogenetic alterations that occur in meningiomas. Eighty percent of adult meningiomas harbor pathogenic somatic variants involving , , , , , or Somatic variants in associated with meningiomas usually localize to the gene's WD40 domains but are mutually exclusive to germline mutations, which cause a distinctive autosomal dominant syndrome.

Observations: This case involved a 15-year-old girl with bilateral optic nerve sheath meningiomas, diffuse meningiomatosis, and syndromic features, including craniosynostosis, brain anomalies, syndactyly, brachydactyly, epicanthus, and patent ductus arteriosus.

The Hybrid Operative Suite with Intraoperative Biplane Rotational Angiography in Pediatric Cerebrovascular Neurosurgery: Utility and Lessons Learned.

Introduction: The benefits of performing open and endovascular procedures in a hybrid neuroangiography surgical suite include confirmation of treatment results and reduction in number of procedures, leading to improved efficiency of care. Combined procedural suites are infrequently used in pediatric facilities due to technical and logistical limitations. We report the safety, utility, and lessons learned from a single-institution experience using a hybrid suite equipped with biplane rotational digital subtraction angiography and pan-surgical capabilities.

Repetitive transcranial magnetic stimulation (rTMS) as therapy in an infant with epilepsia partialis continua.

We present a case of a 10-month-old girl undergoing repetitive TMS (rTMS) for the treatment of drug-resistant epilepsy. A 10-month-old girl, later diagnosed with pathogenic POLG1 mutations, presented to our institution with chronic progressive EPC (epilepsia partialis continua) manifesting as a frequent, left-sided, synchronous continuous jerking of the arms and legs. The seizures were drug-resistant to multiple antiseizure medications and epilepsy surgery, responding only to continuous anesthesia.

Superior Long-term Appearance of Strip Craniectomy Compared with Cranial Vault Reconstruction in Metopic Craniosynostosis.

Background: Strip craniectomy with orthotic helmet therapy (SCOT) is an increasingly supported treatment for metopic craniosynostosis, although the long-term efficacy of deformity correction remains poorly defined. We compared the longterm outcomes of SCOT versus open cranial vault reconstruction (OCVR).

Methods: Patients who underwent OCVR or SCOT for isolated metopic synostosis with at least 3 years of follow-up were identified at our institution.

Impact of the Skull Size on the Normal Brain Radiation Dose During Gamma Knife Radiosurgery: Results of a Pilot Study.

Objective: The objective of the present study was evaluation of the interrelationships between changes in the skull size and variations in the normal brain radiation dose during Gamma Knife surgery (GKS).

Methods: With use of systematic modeling within Leksell GammaPlan (Elekta AB; Stockholm, Sweden) in each of 15 analyzed cases, the skull was "expanded" and "contracted" by variation of its measurement values from 0 to ±3 cm. The mean normal brain radiation dose was then computed for each variant of the adjusted skull size and compared with the original treatment plan.

Navigated transcranial magnetic stimulation mapping of the motor cortex for preoperative diagnostics in pediatric epilepsy.

Objective: Navigated transcranial magnetic stimulation (nTMS) is a noninvasive technique often used for localization of the functional motor cortex via induction of motor evoked potentials (MEPs) in neurosurgical patients. There has, however, been no published record of its application in pediatric epilepsy surgery. In this study, the authors aimed to investigate the feasibility of nTMS-based motor mapping in the preoperative diagnostic workup within a population of children with medically refractory epilepsy.

Direct brain recordings reveal occipital cortex involvement in memory development.

Processing of low-level visual information shows robust developmental gains through childhood and adolescence. However, it is unknown whether low-level visual processing in the occipital cortex supports age-related gains in memory for complex visual stimuli. Here, we examined occipital alpha activity during visual scene encoding in 24 children and adolescents, aged 6.

Comprehensive analysis of diverse low-grade neuroepithelial tumors with FGFR1 alterations reveals a distinct molecular signature of rosette-forming glioneuronal tumor.

The FGFR1 gene encoding fibroblast growth factor receptor 1 has emerged as a frequently altered oncogene in the pathogenesis of multiple low-grade neuroepithelial tumor (LGNET) subtypes including pilocytic astrocytoma, dysembryoplastic neuroepithelial tumor (DNT), rosette-forming glioneuronal tumor (RGNT), and extraventricular neurocytoma (EVN). These activating FGFR1 alterations in LGNET can include tandem duplication of the exons encoding the intracellular tyrosine kinase domain, in-frame gene fusions most often with TACC1 as the partner, or hotspot missense mutations within the tyrosine kinase domain (either at p.N546 or p.

Single-center series of boys with recurrent strokes and rotational vertebral arteriopathy.

Objective: To describe a pediatric stroke syndrome with chronic focal vertebral arteriopathy adjacent to cervical abnormalities.

Methods: At a single pediatric stroke center, we identified consecutive children with stroke and vertebral arteriopathy of the V3 segment with adjacent cervical bony or soft tissue abnormalities. We abstracted clinical presentation, treatment, and follow-up data from medical charts.

Extradural thoracic meningeal cyst without spinal dysraphism causing adulthood myelopathy: Case illustration and review of the literature.

Extradural spinal meningeal cysts are rare lesions in the adult spine and are an uncommon cause of neurologic deficits. We present the case of an adult who presented with myelopathic symptoms related to a dorsally based extradural thoracic meningeal cyst in the absence of any defect in the posterior spinal elements and no history of spinal dysraphism or trauma. We also performed a review of the literature to evaluate the surgical techniques for extradural meningeal cysts.

Meningiomas in children.

Meningiomas in children are poorly understood because they are rare. Recent reports have provided a more complete description of their incidence, genetics, imaging features, and outcome. In general, meningiomas in children are more likely to be higher grade, present in atypical locations, and have a higher risk of recurrence.

Responsive neurostimulation for treatment of pediatric drug-resistant epilepsy.

Responsive neurostimulation for epilepsy involves an implanted device that delivers direct electrical brain stimulation in response to detection of incipient seizures. Responsive neurostimulation is a safe and effective treatment for adults with drug-resistant epilepsy, but although novel treatments are critically needed for younger patients, responsive neurostimulation is currently not approved for children with drug-resistant epilepsy. Here, we report a 16-year-old patient with seizures arising from eloquent cortex, who was successfully treated with responsive neurostimulation.

Complications following pediatric cranioplasty after decompressive craniectomy: a multicenter retrospective study.

OBJECTIVE In children, the repair of skull defects arising from decompressive craniectomy presents a unique set of challenges. Single-center studies have identified different risk factors for the common complications of cranioplasty resorption and infection. The goal of the present study was to determine the risk factors for bone resorption and infection after pediatric cranioplasty.