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Hydrocephalus (HC) is a heterogenous disease characterized by alterations in cerebrospinal fluid (CSF) dynamics that may cause increased intracranial pressure. HC is a component of a wide array of genetic syndromes as well as a secondary consequence of brain injury (intraventricular hemorrhage (IVH), infection, etc.) that can present across the age spectrum, highlighting the phenotypic heterogeneity of the disease. Surgical treatments include ventricular shunting and endoscopic third ventriculostomy with or without choroid plexus cauterization, both of which are prone to failure, and no effective pharmacologic treatments for HC have been developed. Thus, there is an urgent need to understand the genetic architecture and molecular pathogenesis of HC. Without this knowledge, the development of preventive, diagnostic, and therapeutic measures is impeded. However, the genetics of HC is extraordinarily complex, based on studies of varying size, scope, and rigor. This review serves to provide a comprehensive overview of genes, pathways, mechanisms, and global impact of genetics contributing to all etiologies of HC in humans.

Preoperative risk stratification of patients undergoing epilepsy surgery remains challenging. Recently, the efforts to look beyond age alone as an outcomes predictor has resulted in the development of measures of physiological reserve, or ‘frailty indices.’ The most frequently cited index in neurosurgery is the 11-item or 5-item modified frailty index (mFI11 or mFI-5). The present study aimed to use a large national registry to evaluate the effect of frailty (as measured by mFI-5 versus age on postoperative outcomes of patients undergoing epilepsy surgery. The National Surgical Quality Improvement Program (NSQIP) database, overseen by the American College of Surgeons (ACS), was used to extract data for patients undergoing epilepsy surgery from 2015 to 2019. Univariate and multivariate analyses for age and mFI-5 were performed for the following 30-day outcomes of extended length of hospital stay (eLOS) and non-home discharge (NHD). The effect sizes were summarized by odds ratio and associated 95 % confidence intervals. Receiver operating characteristic (ROC) curve analysis, including area under the curve (AUC), was used to quantify the discrimination. Univariate and multivariate analyses demonstrated that frailty statuses from mFI-5, not age, were significantly predictive of eLOS and NHD. On ROC curve analysis, mFI-5 was a stronger predictor of eLOS (C = 0.59, 95 % CI 0.54–0.64, p < 0.001) and NHD (C = 0.69, 95 % CI 0.64–0.76, p < 0.001) than age (C = 0.53, 95 % CI 0.48–0.58, p = 0.21 and C = 0.53, 95 % CI 0.46–0.59, p = 0.44, respectively). Frailty, not age, is an independent risk factor for poor postoperative outcomes, particularly eLOS and NHD, in patients undergoing epilepsy surgery. Usage of mFI-5 for preoperative risk stratification of epilepsy surgery patients can help in prognostication. •Frailty predicts length of stay and non-home discharge after epilepsy surgery.•Frailty is a stronger predictor than age for epilepsy surgery outcomes.•mFI-5 is an effective tool to assess preoperative frailty.

Background Rheumatoid arthritis (RA) is a chronic inflammatory autoimmune disease that causes persistent synovitis, bone damage, and progressive joint destruction. Neuroimmune modulation through electrical stimulation of the vagus nerve activates the inflammatory reflex and has been shown to inhibit the production and release of inflammatory cytokines and decrease clinical signs and symptoms in RA. The RESET-RA study was designed to determine the safety and efficacy of an active implantable device for treating RA. Methods The RESET-RA study is a randomized, double-blind, sham-controlled, multi-center, two-stage pivotal trial that enrolled patients with moderate-to-severe RA who were incomplete responders or intolerant to at least one biologic or targeted synthetic disease-modifying anti-rheumatic drug. A neuroimmune modulation device (SetPoint Medical, Valencia, CA) was implanted on the left cervical vagus nerve within the carotid sheath in all patients. Following post-surgical clearance, patients were randomly assigned (1:1) to active stimulation or non-active (control) stimulation for 1 min once per day. A predefined blinded interim analysis was performed in patients enrolled in the study’s initial stage (Stage 1) that included demographics, enrollment rates, device implantation rates, and safety of the surgical procedure, device, and stimulation over 12 weeks of treatment. Results Sixty patients were implanted during Stage 1 of the study. All device implant procedures were completed without intraoperative complications, infections, or surgical revisions. No unanticipated adverse events were reported during the perioperative period and at the end of 12 weeks of follow-up. No study discontinuations were due to adverse events, and no serious adverse events were related to the device or stimulation. Two serious adverse events were related to the implantation procedure: vocal cord paresis and prolonged hoarseness. These were reported in two patients and are known complications of surgical implantation procedures with vagus nerve stimulation devices. The adverse event of vocal cord paresis resolved after vocal cord augmentation injections with filler and speech therapy. The prolonged hoarseness had improved with speech therapy, but mild hoarseness persists. Conclusions The surgical procedures for implantation of the novel neuroimmune modulation device for the treatment of RA were safe, and the device and its use were well tolerated. Trial registration NCT04539964; August 31, 2020.

The nematode Onchocerca lupi is an emerging human pathogen. Though its life cycle is not well studied, it likely infects humans after a bite from a black fly vector, which in turn acquires infective microfilariae from an infected canid. These microfilariae mature into an infective larval stage within the fly. Among six reported cases in the United States, five involved children, and all occurred in the southwest. In this report, we present a case of O. lupi infection with cervical spine invasion in a healthy 10-year-old girl. She presented with five months of neurological symptoms from a rural and medically underserved area, highlighting a need for clinical vigilance in such settings for this emerging infectious threat in the American southwest.

•Myelin water fraction (MWF) maps could be used to further characterize febrile seizures.•All subjects in this study with simple febrile seizures had increased MWF.•All subjects in this study with complex febrile seizures had decreased MWF. The objective of this feasibility study was to investigate whether myelin water fraction (MWF) patterns can differentiate children presenting with febrile seizures who will go on to develop nonfebrile epilepsy from those who will not. As part of a prospective study of myelination patterns in pediatric epilepsy, seven subjects with febrile seizures underwent magnetic resonance imaging (MRI) including the following standard sequences—T1-weighted, T2-weighted, fluid-attenuated inversion recovery (FLAIR)—and an additional experimental sequence, multicomponent-derived equilibrium single-pulse observation of T1 and T2 (mcDESPOT) to quantify MWF. For each of these subjects, MWF maps were derived and compared with an age-matched population-averaged MWF atlas. All seven subjects (<5 years old) initially presented with febrile seizures. Of the seven, four had complex seizures and three had simple seizures. All of the children with simple febrile seizures had higher MWF compared with model-derived controls and did not develop epilepsy. All of the children with complex febrile seizures had lower MWF than their model-derived control, and two of these subjects later developed epilepsy. This is the first study in which MWF maps were used to study children with febrile *seizures. This data suggests that relatively higher or stable MWF compared with normative data indicates a lower risk of nonfebrile epilepsy while relatively lower MWF may indicate a pathological condition that could lead to nonfebrile epilepsy.

Patient satisfaction is a key metric used to measure quality in health care. However, patient satisfaction measures in the pediatric population are less studied and understood than in the adult population. The purpose of this study was to evaluate the impact of telephone follow-up on patient satisfaction in an outpatient pediatric neurosurgery clinic. A standardized telephone follow-up call was performed within 1 week of a child’s clinic visit. Pearson’s χ2 or Fisher’s exact tests were used to assess changes in patient satisfaction measures after implementation of the telephone follow-up call initiative. The proportion of overall “top-box” physician rating significantly increased from 85.5% in 2017 to 95.6% in 2018 (P = .04). There was also a nonsignificant upward trend in the proportion of respondents noting that they would recommend this provider, as well as in all measures of physician communication quality and office staff quality. A simple telephone call to new patients after an outpatient pediatric neurosurgery clinic visit resulted in statistically significant and clinically meaningful changes in patient satisfaction scores.

Carotid endarterectomy (CEA) is one of the most commonly performed and studied surgical procedures for extracranial ischemic disease. The authors reviewed the outcome of 39 consecutive carotid endarterectomy procedures performed by a single surgeon with emphasis on the safety of discharging patients the same day of the procedure. Retrospective analysis was performed over a two-year period on patients who were admitted as outpatients and underwent CEA. Following CEA, patients were observed for 4–6h in the recovery room and Duplex ultrasonography was completed to assess the endarterectomy repair. Determination was then made whether patients could be safely discharged home. Over a two year period, CEA was performed 39 times in 37 outpatients. Twenty-five patients (64%) were discharged within 6h of surgery completion. The remaining 14 patients (36%) were admitted to the hospital for varying reasons. Six patients (43%) stayed either due to personal preference or the lack of supervision at home and six other patients (43%) stayed because of mild hemodynamic instability. Of the two remaining patients, one was admitted for chest pain and the other for a small wound hematoma. No patients developed postoperative neurologic deficits. Two-tailed Fisher test analysis of collected variables revealed that patients who had general anesthesia were more likely to be admitted (p<0.02). Patients undergoing CEA can be safely discharged the same day after a brief period of postoperative observation. One factor that may predict the need for postoperative admission is the use of general anesthesia.

•Worse GOS-E Peds scores were associated with higher NLRs at 24 and 48 h.•LOC was associated with higher NLRs on admission and at 24 h post-injury. The brain’s inflammatory reaction to traumatic brain injury (TBI) generally peaks between 24 and 48 h after injury. This inflammatory cascade can be neuroprotective or may mediate secondary brain injury beyond the initial TBI. Therefore, circulating inflammatory markers may be useful for predicting outcomes in pediatric TBI. The goal of this study was to determine whether elevations in peripheral blood neutrophil-to-lymphocyte ratios (NLRs) are associated with adverse outcomes in pediatric TBI patients. 188 pediatric patients (0–18 years) presenting to our institution with TBI from 2007 to 2017 were retrospectively reviewed. Absolute neutrophil and lymphocyte counts from a complete blood count (CBC) were used to calculate NLRs on admission (<12 h) and approximately 24, 48, and 72 h after injury. Data points included Glasgow Coma Scale (GCS) on admission, presence of post-traumatic amnesia (PTA), loss of consciousness (LOC), and Glasgow Outcome Scale Extended Pediatric Version (GOS-E Peds) with a median outcome span of 86 days. A one-way ANOVA demonstrated statistically significant differences in NLR at 24 h (p = 0.004) and 48 h (p=0.003) among patients stratified by GOS-E Peds. No significant differences in NLR were observed at any time point based on GCS or PTA. Patients who experienced LOC had a significantly higher NLR on admission (p=0.013) and at 24 h (p<0.001) than those who did not. In this study, relatively higher NLRs at 24 and 48 h post-TBI were associated with worse outcomes in pediatric patients. This suggests that NLR may be a useful and cost-effective outcome predictor in pediatric TBI as well as a possible future target for therapeutic intervention, warranting larger prospective trials.

A cerebral arteriovenous fistula (CAVF) is a rare abnormality representing only 4.7% of all cerebral arteriovenous malformations. In this report a unique case is presented of a giant holo-hemispheric CAVF in an infant who presented with congestive heart failure and was successfully treated endovascularly with transarterial and transvenous embolization. A 4-day-old girl presented with lethargy and poor feeding and was found to be in congestive heart failure secondary to a large left hemispheric vascular malformation. Embolization was performed via a transarterial route on days of life (DOL) 11 and 18, a transvenous route on DOL 54 and a final transarterial treatment on DOL 76. After all treatment the patient was discharged home on minimal cardiac medications. At 2-year follow-up the patient had moderate motor delays and mild speech delays, but she continued to meet milestones and her cardiac failure was completely resolved. This case demonstrates one of the largest CAVFs treated successfully in the neonatal population.