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Brain abscesses in infants are extremely rare and occur as an uncommon complication of bacterial meningitis or sepsis. This case is of a 29 week male premature infant with a diagnosis of a brain abscess at 63 days of life secondary to methicillin-resistant staphylococcus aureus bacteria (MRSA) which evolved after a fairly unremarkable neonatal intensive care unit course. Cultures of the cerebrospinal fluid and blood yielded no growth. He was found to be colonized with methicillin-resistant staphylococcus aureus on routine surveillance cultures after transfer from an outside hospital. The infant required a temporary external ventricular drain for hydrocephalus and compression of the brainstem secondary to the mass effect. He is currently developing favorably at 14 months after undergoing prolonged treatment with Vancomycin and Rifampin. [ABSTRACT FROM AUTHOR]

Abstract: Purpose: Lennox–Gastaut syndrome (LGS) is an epileptogenic disorder that arises in childhood and is typically characterized by multiple seizure types, slow spike-and-wave complexes on EEG and cognitive impairment. If medical treatment fails, patients can proceed to one of two palliative surgeries, vagus nerve stimulation (VNS) or corpus callosotomy (CC). Their relative seizure control rates in LGS have not been well studied. The purpose of this paper is to compare seizure reduction rates between VNS and CC in LGS using meta-analyses of published data. Methods: A systematic search of Pubmed, Ovidsp, and Cochrane was performed to find articles that met the following criteria: (1) prospective or retrospective study, (2) at least one patient diagnosed with Lennox–Gastaut syndrome, and (3) well-defined measure of seizure frequency reduction. Seizure reduction rates were divided into seizure subtypes, as well as total seizures, and categorized as 100%, >75%, and >50%. Patient groups were compared using chi-square tests for categorical variables and t-test for continuous measures. Pooled proportions with 95% confidence interval (95% CI) of seizure outcomes were estimated for total seizures and seizure subtypes using random effects methods. Results: 17 VNS and 9 CC studies met the criteria for inclusion. CC had a significantly better outcome than VNS for >50% atonic seizure reduction (80.0% [67.0–90.0%] vs. 54.1% [32.1–75.4%], p <0.05) and for >75% atonic seizure reduction (70.0% [48.05–87.0%] vs. 26.3% [5.8–54.7%], p <0.05). All other seizure types, as well as total number of seizures, showed no statistically significant difference between VNS and CC. Conclusions: CC may be more beneficial for LGS patients whose predominant disabling seizure type is atonic. For all other seizure types, VNS offers comparable rates to CC. [Copyright &y& Elsevier]

Background: Arteriovenous malformations (AVMs) are the most common cause of intracerebral hemorrhage in children. Different options exist for their successful management consisting of surgery, endovascular embolization, stereotactic radiosurgery, or a combination of these treatments. Discussion: In this paper, we discuss the different treatment modalities in the treatment of pediatric cerebral AVMs emphasizing the role of surgery and endovascular embolization as a preoperative strategy. [ABSTRACT FROM AUTHOR]

The cellular and molecular events that initiate and promote malignant glioma development are not completely understood. The treatment modalities designed to promote its demise are all ultimately ineffective, leading to disease progression. In this issue of the JCI, Kioi et al. demonstrate that vasculogenesis and angiogenesis potentially play distinct roles in the etiology of primary and recurrent malignant gliomas, suggesting that patient therapy should perhaps be tailored specifically against the predominant vasculature pathway at a given specific stage of gliomagenesis. [ABSTRACT FROM AUTHOR]

Objective: Infusion of intraventricular chemotherapy requires precise ventricular catheter placement in oncology patients to avoid intraparenchymal infusion. We assessed the safety and precision of using frameless surgical navigation for the placement of Ommaya reservoir catheters. Methods: In 20 consecutive patients who required Ommaya reservoirs for the administration of intrathecal chemotherapy, the catheters were positioned with the guidance of frameless stereotaxy. Precision of catheter placement, operative time, operative complications, and postoperative complications were assessed. Results: All catheters were completely within cerebrospinal fluid and 90% were within 5 mm of the intended target, the ipsilateral foramen of Monro. The average operative time was 47 min, and there were no intraoperative complications. There were no reports of leukoencephalopathy, and 3 patients had minor delayed complications. Conclusion: Frameless surgical navigation is a useful and efficient method for ensuring accurate catheter placement during Ommaya reservoir implantation, and it reduces the risk of potential parenchymal toxicity. Copyright © 2007 S. Karger AG, Basel [ABSTRACT FROM AUTHOR]

Chiari malformation type 1 (CM1) is a structural defect that involves the herniation of the cerebellar tonsils through the foramen magnum, causing mild to severe neurological symptoms. Little is known about the molecular and developmental mechanisms leading to its pathogenesis, prompting current efforts to elucidate genetic drivers. Inherited genetic disorders are reported in 2–3% of CM1 patients; however, CM1, including familial forms, is predominantly non-syndromic. Recent work has focused on identifying CM1-asscoiated variants through the study of both familial cases and de novo mutations using exome sequencing. This article aims to review the current understanding of the genetics of CM1. We discuss three broad classes of CM1 based on anatomy and link them with genetic lesions, including posterior fossa-linked, macrocephaly-linked, and connective tissue disorder-linked CM1. Although the genetics of CM1 are only beginning to be understood, we anticipate that additional studies with diverse patient populations, tissue types, and profiling technologies will reveal new insights in the coming years. [ABSTRACT FROM AUTHOR]

Obtaining postmortem tissue from pediatric oncology patients is critical to research and may help grieving families heal. Since 2019, the national Gift from a Child program has made significant progress in collecting postmortem tissue from pediatric patients with central nervous system tumors to advance research. This progress was at risk during the onset of the severe acute respiratory syndrome coronavirus 2 pandemic, when some autopsy programs came to a halt. We retrospectively reviewed autopsies of four patients treated at Memorial Sloan Kettering Cancer Center who underwent postmortem examination at Weill Cornell Medicine from June 2020 to March 2021. We collected patient demographics, Do not resuscitate status, time of death and procedure, restrictions due to the coronavirus disease 2019 (COVID-19) pandemic, and results of the tissue analysis. Three of four specimens were processed within 12 hours of the time of death. Two families required interpreter services to obtain consent. In all cases, tumor aliquots were flash frozen for further study. Cell line generation was successful in one case. All families expressed gratitude both for the opportunity to participate and for the handling of the procedures. Despite the sensitive nature of these cases and the challenges presented by COVID-19 restrictions, clinicians should offer the option of a rapid autopsy to caregivers of pediatric patients based on the scientific need and the positive effect it has on grieving families. This article outlines the logistic efforts required for these donations to take place and provides a framework for providers to offer rapid autopsy as an option for families through this program. [ABSTRACT FROM AUTHOR]

Background: The prognosis for metastatic and recurrent tumors of the central nervous system (CNS) remains dismal, and the need for newer therapeutic targets and modalities is critical. The cell surface glycoprotein B7H3 is expressed on a range of solid tumors with a restricted expression on normal tissues. We hypothesized that compartmental radioimmunotherapy (cRIT) with the anti-B7H3 murine monoclonal antibody omburtamab injected intraventricularly could safely target CNS malignancies. Patients and methods: We conducted a phase I trial of intraventricular 131I-omburtamab using a standard 3 + 3 design. Eligibility criteria included adequate cerebrospinal fluid (CSF) flow, no major organ toxicity, and for patients > dose level 6, availability of autologous stem cells. Patients initially received 74 MBq radioiodinated omburtamab to evaluate dosimetry and biodistribution followed by therapeutic 131I-omburtamab dose-escalated from 370 to 2960 MBq. Patients were monitored clinically and biochemically for toxicity graded using CTCAEv 3.0. Dosimetry was evaluated using serial CSF and blood sampling, and serial PET or gamma-camera scans. Patients could receive a second cycle in the absence of grade 3/4 non-hematologic toxicity or progressive disease. Results: Thirty-eight patients received 100 radioiodinated omburtamab injections. Diagnoses included metastatic neuroblastoma (n = 16) and other B7H3-expressing solid tumors (n = 22). Thirty-five patients received at least 1 cycle of treatment with both dosimetry and therapy doses. Acute toxicities included < grade 4 self-limited headache, vomiting or fever, and biochemical abnormalities. Grade 3/4 thrombocytopenia was the most common hematologic toxicity. Recommended phase 2 dose was 1850 MBq/injection. The median radiation dose to the CSF and blood by sampling was 1.01 and 0.04 mGy/MBq, respectively, showing a consistently high therapeutic advantage for CSF. Major organ exposure was well below maximum tolerated levels. In patients developing antidrug antibodies, blood clearance, and therefore therapeutic index, was significantly increased. In patients receiving cRIT for neuroblastoma, survival was markedly increased (median PFS 7.5 years) compared to historical data. Conclusions: cRIT with 131I-omburtamab is safe, has favorable dosimetry and may have a therapeutic benefit as adjuvant therapy for B7-H3-expressing leptomeningeal metastases. Trial registration: clinicaltrials.gov NCT00089245, August 5, 2004. [ABSTRACT FROM AUTHOR]

Virtual learning has made neurosurgery education more available to medical students (MSs) of all stages than ever before. We aimed to evaluate the impact of on-site learning in order to assess whether a return to this method of teaching, when safe, is warranted for MSs of all years. Registrants of the 2019 MS Neurosurgery Training Camps were sent precourse and postcourse surveys to assess changes in self-assessed confidence in concrete neurosurgical skills. Data were analyzed using a 2-tailed paired Student's t -test for continuous variables. A P value <0.05 was considered significant. The 2019 Training Camp had 105 attendees, of whom 94 (89.5%) completed both surveys. Students reported statistically significant improvements in every surveyed skill area, except for understanding what is and is not sterile in an operating room. The cohort of MS 3/4 students indicated a postcourse decrease in confidence in their ability to understand what is and is not sterile in an operating room (93.69 ± 16.41 vs. 86.20 ± 21.18; P < 0.05). MS 3/4 students did not benefit in their ability to perform a neurologic examination or tie knots using a 1-handed technique. Neurosurgical education initiatives for MSs should continue to be developed. Hands-on neurosurgical training experiences for MSs serve as a valuable educational experience. Improvement in training models will lead to capitalizing on MS education to better improve readiness for neurosurgical residency without concern for patient safety. [ABSTRACT FROM AUTHOR]

Studies the critical role of presenilins in the proteolysis of beta-amyloid precursor protein (beta-APP) to generate beta-amyloid, a peptide important in the pathogenesis of Alzheimer's disease. Biogenesis of beta-APP-containing vesicles from the trans-Golgi network and the endoplasmic reticulum; Analysis of pertinent topics and relevant issues; Implications on membrane transport, structure, function and biogenesis.

Radiographic characterization of Chiari malformation (CM) has historically focused on caudal tonsillar herniation (CH) below the foramen magnum. Previously, we published evidence linking ventral tonsillar herniation (VH) and medullary symptoms in very young children. We sought to extend that investigation by studying the radiographic and clinical significance of VH in adults diagnosed with CM. We retrospectively reviewed adults with cerebellar ectopia who underwent posterior fossa decompression with or without duraplasty (PFD/D) at our institution. VH was defined as tonsils crossing a line bisecting the caudal medulla at the level of the foramen magnum on axial MRI. Degree of VH was measured as distance between this bisecting line and the ventral tip of the herniated tonsil. Dorsal brainstem compression was qualitatively determined by assessing for obliteration of CSF space between the dorsal brainstem and the tonsils. Out of 89 cases reviewed, 54 had some degree of VH. Compared with those without VH, the VH group was significantly older in age and more likely to also present with dorsal brainstem compression and headaches. No correlation was observed between degrees of CH and VH in the VH group. The degree of VH significantly decreased 3 months after PFD/D. VH is relatively common in CM patients and might be an important independent radiographic metric to evaluate and consider as part of the decision-making process, especially in those presenting with Chiari-like symptomatology referable to the medulla but who do not meet the traditional criteria of cerebellar ectopia greater than 5 mm. [ABSTRACT FROM AUTHOR]

Introduction: Tethered cord syndrome (TCS) is an amalgamation of neurological, urological, orthopedic, and dermatologic signs and symptoms with radiographic evidence of a thickened filum and low-lying conus. Surgical sectioning of the filum and disconnection of any tethering entities such as dermal sinus tracts or lipomas has been shown to improve outcomes. The manifestation of TCS symptoms in the absence of a low-lying conus has been referred to as occult tethered cord syndrome (OTCS) and is much less well reviewed in the literature. To date, there has only been one randomized controlled trial examining the effect of intervention in OTCS; therefore, contemporary data is often elicited from limited cohorts. Objective: To perform a comprehensive literature review of management in OTCS and evaluate treatment response rates to sectioning of the filum terminale. Results: Seventeen papers met inclusion criteria for our review. Sample sizes ranged from 8 to 60 children, and results were mixed, often dependent on study design, definition of typical OTCS symptoms, and follow-up intervals. Symptomatic improvement was observed in > 50% of patients for all but one study; however, the recurrence rates were highly variable. Conclusion: The data regarding the efficacy of surgical treatment in OTCS is mixed and merits more rigorous scientific examination with strict and clear parameters regarding symptomatic operationalization and follow-up time points to monitor for TCS recurrence. [ABSTRACT FROM AUTHOR]

The coronavirus disease 2019 (COVID-19) pandemic has created significant obstacles within medical education. For medical students interested in pursuing neurosurgery as a specialty, the educational policies surrounding COVID-19 have resulted in unique challenges. The present study used a nationwide survey to identify the concerns of medical students interested in pursuing neurosurgery during the COVID-19 pandemic. Students who had previously registered for medical student neurosurgery training camps were sent an online Qualtrics survey requesting them to assess how the COVID-19 pandemic was affecting their neurosurgical education. The Pearson χ2 test and post hoc pairwise Fisher exact test were used for analysis of categorical variables, and the 2-tailed paired Student t test was used for continuous variables. The survey was distributed to 852 medical students, with 127 analyzed responses. Concerns regarding conferences and networking opportunities (63%), clinical experience (59%), and board examination scores (42%) were most frequently cited. Of the third-year medical students, 76% reported ≥1 cancelled or postponed neurosurgery rotation. On average, students were more likely to take 1 year off from medical school after than before the start of the COVID-19 pandemic, measured from 0 to 100 (25.3 ± 36.0 vs. 39.5 ± 37.5; P = 0.004). Virtual mentorship pairing was the highest rated educational intervention suggested by first- and second-year medical students. The third- and fourth-year medical students had cited virtual surgical skills workshops most frequently. The results from the present nationwide survey have highlighted the concerns of medical students regarding their neurosurgery education during the COVID-19 pandemic. With these findings, neurosurgery organizations can consider targeted plans for students of each year to continue their education and development. [ABSTRACT FROM AUTHOR]

The factors that attract and concern medical students about a career in neurosurgery have never been clearly characterized or delineated in a large nationwide cohort of medical students intending to pursue a career in neurosurgery. The objective of the present study was to characterize the factors that influence medical student interest in neurosurgery and assess the effects of a formal neurosurgery training course on participants' perceptions of a career in neurosurgery. Before the Medical Student Neurosurgery Training Camp for subinternship preparation, registered students were surveyed about their interest level in neurosurgery, factors that attracted or concerned them about a career in neurosurgery, attendance at a national neurosurgery conference or course, formal clinical neurosurgery exposure in medical school, and whether they had a resident or attending mentor in neurosurgery. At the end of the course, all the participants completed the surveyed again. P < 0.05 was considered significant on Pearson's χ2 and Fisher's exact tests for categorical variables and 2-tailed paired Student's t tests for continuous variables. Of the training camp attendees, >95% completed both pre- and postcourse surveys, including 41 first-year, 19 second-year, 30 third-year, and 5 fourth-year medical school students. The most common factors that concerned students about a career in neurosurgery were work–life balance (76%) and competitiveness (56%). All factors of concern were decreased in the postcourse survey, except for competitiveness. A small cohort (8.4%) of students had no concerns about a career in neurosurgery; this cohort had doubled to 17% after the course (P < 0.05). The students that indicated no concern had a greater postcourse interest level in neurosurgery (95.8 ± 8.7 vs. 86.7 ± 20.5; P < 0.05). Student reasons for an interest in neurosurgery included intellectually stimulating work (94%), interest in neurosciences (93%), effect on patients (84%), innovation and new technology (80%), research opportunities (77%), and prestige (24%). All reasons increased after the course, with the exception of prestige, which decreased to 22%. A training camp for students pursuing a neurosurgery subinternship was effective in providing transparency and positively influencing the factors that attract and concern students about a career in neurosurgery. Characterization of medical student perceptions of neurosurgery from a large, nationwide cohort of students pursuing a subinternship has provided novel data and could help identify factors protecting against burnout later in life. [ABSTRACT FROM AUTHOR]

Patients who undergo decompression surgery for Chiari malformation frequently require occipitocervical fixation. This is typically performed with occipital plates, which may cause intracranial injuries due to multiple fixation points. We undertook this study to assess the feasibility of occipital condyle (OC) screw placement as an alternative method of occipitocervical fixation in this patient population. Using a cadaveric model with navigational assistance, we performed the complete surgical procedure for occipitocervical fixation with OC screws. We then performed a morphometric analysis using measurements from computed tomography scans of 49 patients (32 adult, 17 pediatric) who had undergone occipitocervical fusion with instrumentation following decompression surgery for Chiari malformation. Bilateral morphometric data were analyzed for the adult and pediatric subgroups separately, as well as for the overall group. The surgical procedure was successfully performed in the cadaveric model, demonstrating the feasibility of the proposed method. Ninety-eight OCs were studied in the morphometric analysis, and 80 (81.6%) met our eligibility criteria for OC screw placement. However, in 14.1% of adult OCs and 26.5% of pediatric OCs studied, placement of condylar screws would have been challenging or unsafe, according to our criteria. Our findings suggest that OC screws provide a useful option for occipitocervical fixation in a substantial proportion of patients with Chiari malformation. However, rigorous preoperative analysis would be essential to identify appropriate candidates for this technique and exclude those in whom it should not be attempted. Additional study is warranted. [ABSTRACT FROM AUTHOR]

Cervicomedullary compression in young children has been described in the context of Chiari type 1 malformation, with symptoms associated with the extent of tonsillar herniation below McRae line. Historically, Chiari type 1 malformation has been defined by tonsillar herniation of at least 5 mm. However, in certain populations, including very young children, Chiari symptoms may be present without this finding. A new Chiari classification is thus necessary. Cases involving patients up to 5 years of age evaluated for possible posterior fossa decompression were retrospectively reviewed. Preoperative symptoms, magnetic resonance imaging findings, surgical management, and short- and long-term outcome and follow-up were recorded. Tonsillar descent and presence of ventral herniation (VH) were recorded. We define VH as the tonsils crossing a line that bisects the caudal medulla at the level of the foramen magnum, thus creating a novel entity, Chiari type 0.5 malformation. Patients with ventrally herniated tonsils were compared with patients exhibiting more typical Chiari morphology. Of 41 cases retrospectively reviewed, 20 met criteria for VH. These differed from cases without VH because of the predominance of medullary symptoms. In the VH cohort, 11 patients underwent surgical decompression with symptom resolution; 9 were initially managed conservatively, but 3 subsequently required surgery. We define a novel Chiari entity, Chiari type 0.5 malformation, characterized by ventral tonsillar wrapping around the medulla in young children in the absence of classic Chiari type 1 malformation imaging findings. These patients are more likely to present with medullary symptoms than patients without VH. They are also more likely to require surgical decompression and respond favorably to intervention. [ABSTRACT FROM AUTHOR]

Germ cell tumors are rare malignant tumors frequently located in the suprasellar region. Definitive treatment is chemotherapy and radiation. However, in some circumstances, surgery is indicated for biopsy or resection. There are limited reports of the role of the endonasal endoscopic approach (EEA) in the management of this tumor. We present two cases in which EEA was utilized for successful management of germ cell tumor. The most challenging aspect of germ cell tumor management for the treating physician is knowing the proper indications for surgery. In this paper, we highlight two specific instances, namely diagnosis and tumor refractory to chemoradiation. Given the suprasellar location, EEA is an ideal approach. [ABSTRACT FROM AUTHOR]

Historically, medical student education in neurological surgery has generally limited student involvement to assisting in research projects with minimal formal clinical exposure before starting sub-internships and application for the neurosurgery match. Consequently, students have generally had little opportunity to acquire exposure to clinical neurosurgery and attain minimal proficiency. A medical student training camp was created to improve the preparation of medical students for the involvement in neurological surgery activities and sub-internships. A 1-day course was held at Weill Cornell Medicine, which consisted of a series of morning lectures, an interactive resident lunch panel, and afternoon hands-on laboratory sessions. Students completed self-assessment questionnaires regarding their confidence in several areas of clinical neurosurgery before the start of the course and again at its end. A significant increase in self-assessed confidence was observed in all skill areas surveyed. Overall, rising fourth year students who were starting sub-internships in the subsequent weeks reported a substantial increase in their preparedness for the elective rotations in neurosurgery. The preparation of medical students for clinical neurosurgery can be improved. Single-day courses such as the described training camp are an effective method for improving knowledge and skill gaps in medical students entering neurosurgical careers. Initiatives should be developed, in addition to this annual program, to increase the clinical and research skills throughout medical student education. [ABSTRACT FROM AUTHOR]

Background: The association between mid-facial clefts and Chiari malformation in the medical literature has been restricted to patients with syndromic craniofacial abnormalities. A common shared developmental pathway including causative factors for facial clefts and “complex” Chiari malformations, both midline skull base pathologies, seems logical but has not been reported. The coincident presentation of these findings in a single patient, and our subsequent discovery of other patients harboring these mutual findings prompted further investigation.Case illustration: We describe the case of a patient born with a cleft palate which was repaired during his first year of life, subsequently presenting as a teenager to our hospital with a severe and symptomatic complex Chiari malformation. We discuss his treatment strategy, suboccipital decompression with occipitocervical fusion and endoscopic anterior decompression surgeries, as well as his favorable radiological and clinical outcome, demonstrated at long-interval follow-up. Furthermore, we review his two pathologies, cleft palate and Chiari malformation, and posit a common embryological linkage.Conclusions: The embryologic interaction between the paraxial mesoderm and ectoderm may explain the co-occurrence of cleft palate and complex Chiari malformation in a single patient. Complete radiological, clinical, and genetic evaluation and counseling is advised in this situation and raises the question of whether the presence of a cleft palate independently increases the risk for other skull base developmental abnormalities. [ABSTRACT FROM AUTHOR]

Leptomeningeal disease (LMD) in pediatric brain tumors (PBTs) is a poorly understood and categorized phenomenon. LMD incidence rates, as well as diagnosis, treatment, and screening practices, vary greatly depending on the primary tumor pathology. While LMD is encountered most frequently in medulloblastoma, reports of LMD have been described across a wide variety of PBT pathologies. LMD may be diagnosed simultaneously with the primary tumor, at time of recurrence, or as primary LMD without a primary intraparenchymal lesion. Dissemination and seeding of the cerebrospinal fluid (CSF) involves a modified invasion-metastasis cascade and is often the result of direct deposition of tumor cells into the CSF. Cells develop select environmental advantages to survive the harsh, nutrient poor and turbulent environment of the CSF and leptomeninges. Improved understanding of the molecular mechanisms that underlie LMD, along with improved diagnostic and treatment approaches, will help the prognosis of children affected by primary brain tumors. [ABSTRACT FROM AUTHOR]