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Purpose: Hemispherotomy is an effective surgery for intractable pediatric hemispheric epilepsy. Over the years, the surgical goal has shifted from a complete hemispheric resection (anatomical hemispherectomy) to a disconnective hemispherotomy (DH). Multiple techniques for DH have been described, and often, anterior temporal lobectomy (ATL, with hippocampal resection) is performed. The goal of the current study is to assess the role of hippocampal resection in DH., Methods: We retrospectively collected all clinical data of children (< 18 years old) who underwent DH between 2001 and 2022 at two tertiary large centers. Epilepsy status and surgical outcome were compared, based on whether the hippocampus was resected (as part of an ATL) or disconnected at the amygdala and atrial segment of the fornix (with no ATL)., Results: A total of 86 patients (32 females) were included. The most common epilepsy etiologies were stroke (31), Rasmussen's encephalitis (16), cortical dysplasia (10), and hemimegaloencephaly (9). The mean age at surgery was 7 (± 4.9) years. The average number of anti-seizure medications (ASMs) at surgery was 3 (± 1.2). Hemispherotomy techniques included peri-insular (54), vertical (23 [19 endoscopic + 4 parasagittal]), and trans-sylvian (9). The mean follow-up was 41.5 (± 38) months. Forty-three patients had hippocampal resection, and 43 patients had a hippocampal disconnection. Both groups had similar Engel outcome scores (p = 0.53)., Conclusions: Disconnective hemispherotomy is highly effective for pediatric intractable hemispheric epilepsy. Our data suggest that the inclusion of hippocampal resection does not provide additional benefit., (© 2024. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: The intrathecal baclofen pump is an effective treatment option for patients with severe spasticity. In children, subfascial pump placement is often preferred given concerns for infection and wound healing. However, this approach is not without risk, and rare complications, such as peritoneal pump migration, can occur., Observations: The authors describe three pediatric cases of peritoneal pump migration at their institution over the past 14 years (3/545, 0.5%). All three patients had low body weight (below the 39th percentile), and two had scoliosis requiring surgery. All pumps had been placed using the subfascial technique. The first case occurred 6 months postplacement, and the pump was not replaced. Cases 2 and 3 occurred at 2 and 3 years postplacement, respectively, and both pumps were replaced., Lessons: The authors conclude that peritoneal pump migration, although uncommon, can occur in patients with subfascial pump placement, and providers should have a low threshold of suspicion for repeat imaging prior to refilling if the pump's location has migrated. Potential contributing factors to pump migration include a patient's small size, a larger pump size (40-mL pump), and scoliosis. All these factors should be considered during pump placement, and surgeons can consider using a 20-ml pump for smaller patients. https://thejns.org/doi/abs/10.3171/CASE24290.

Purpose: Stereoelectroencephalography (SEEG) is widely performed on individuals with medically refractory epilepsy for whom invasive seizure localization is desired. Despite increasing adoption in many centers across the world, no standardized electrode naming convention exists, generating confusion among both clinical and research teams., Methods: We have developed a novel nomenclature, named the Standardized Electrode Nomenclature for SEEG Applications system. Concise, unique, informative, and unambiguous labels provide information about entry point, deep targets, and relationships between electrodes. Inter-rater agreement was evaluated by comparing original electrode names from 10 randomly sampled cases (including 136 electrodes) with those prospectively assigned by four additional blinded raters., Results: The Standardized Electrode Nomenclature for SEEG Application system was prospectively implemented in 40 consecutive patients undergoing SEEG monitoring at our institution, creating unique electrode names in all cases, and facilitating implantation design, SEEG recording and mapping interpretation, and treatment planning among neurosurgeons, neurologists, and neurophysiologists. The inter-rater percent agreement for electrode names among two neurosurgeons, two epilepsy neurologists, and one neurosurgical fellow was 97.5%., Conclusions: This standardized naming convention, Standardized Electrode Nomenclature for SEEG Application, provides a simple, concise, reproducible, and informative method for specifying the target(s) and relative position of each SEEG electrode in each patient, allowing for successful sharing of information in both the clinical and research settings. General adoption of this nomenclature could pave the way for improved communication and collaboration between institutions., Competing Interests: The authors have no funding or conflicts of interest to disclose., (Copyright © 2024 by the American Clinical Neurophysiology Society.)

Objective: Selective dorsal rhizotomy (SDR) is a neurosurgical procedure to reduce spasticity in children with cerebral palsy and spastic diplegia. The authors developed a procedure called focal SDR for children with spasticity predominantly in the L5 or S1 motor distribution, which can be combined with orthopedic correction of fixed soft-tissue or bony deformity. The authors describe in detail the technique of minimally invasive focal SDR and propose selection criteria., Methods: The authors conducted a retrospective study of patients who underwent focal SDR at their institution and underwent baseline and 1-year postoperative 3D gait analysis. Modified Ashworth scale (MAS) and Gait Deviation Index (GDI) scores were the primary outcome measures., Results: Ten patients met the study criteria, all with an underlying diagnosis of cerebral palsy. All underwent focal SDR at the unilateral or bilateral S1 level, and 4 additionally underwent focal SDR at the L5 level unilaterally or bilaterally. All but 1 patient underwent concurrent orthopedic surgery. The improvement in spasticity of the plantar flexors, as measured by the MAS score, was 2.2 (p < 0.001). In the patients who underwent L5 focal SDR, there was an improvement in the hamstring MAS score of 1.4 (p = 0.004). The mean improvement in the GDI score following focal SDR was 11 (range -6 to 29, p < 0.001)., Conclusions: Focally impairing spasticity in the gastrocsoleus complex and/or hamstrings muscle group in the setting of less functionally impactful proximal tone is extremely common in cerebral palsy. The novel technique of focal SDR, combined with orthopedic intervention, improves spasticity scores and overall gait mechanics. Further investigation is warranted to define the ideal candidacy and outcomes.

Objective: Responsive neurostimulation (RNS) is a US FDA-approved form of neuromodulation to treat patients with focal-onset drug-resistant epilepsy (DRE) who are ineligible for or whose condition is refractory to resection. However, the FDA approval only extends to use in patients with one or two epileptogenic foci. Recent literature has shown possible efficacy of thalamic RNS in patients with Lennox-Gastaut syndrome and multifocal epilepsy. The authors hypothesized that RNS of thalamic nuclei may be effective in seizure reduction for patients with multifocal or regionalized-onset DRE., Methods: The authors performed a retrospective chart review of all patients who had an RNS device managed at Texas Children's Hospital between July 2016 and September 2023, with at least one active electrode in the thalamic nuclei and ≥ 12 months of postimplantation follow-up. Information conveyed by the patient or their caregiver provided data on the change in the clinical seizure frequency, quality of life (QOL), and seizure severity between the preimplantation baseline visit and the last office visit (LOV)., Results: Thirteen patients (ages 8-24 years) were identified with active RNS leads in thalamic nuclei (11 centromedian and 2 anterior nucleus). At LOV, 46% of patients reported 50%-100% clinical seizure reduction (classified as responders), 15% reported 25%-49% reduction, and 38% reported < 25% reduction or no change. Additionally, 42% of patients reported subjective improvement in QOL and 58% reported improved seizure severity. Patients with focal cortical dysplasia (FCD) responded strongly: 3 of 5 (60%) reported ≥ 80% reduction in seizure burden and improvement in seizure severity and QOL. Patients with multifocal epilepsy and bilateral thalamocortical leads also did well, with all 3 reporting ≥ 50% reduction in seizures., Conclusions: RNS of thalamic nuclei shows promising results in reducing seizure burden for patients with multifocal or regional-onset DRE, particularly in a bilateral thalamocortical configuration or when addressing an underlying FCD.

Neuromodulation through implantable pulse generators (IPGs) represents an important treatment approach for neurological disorders. While the field has observed the success of state-of-the-art interventions, such as deep brain stimulation (DBS) or responsive neurostimulation (RNS), implantable systems face various technical challenges, including the restriction of recording from a limited number of brain sites, power management, and limited external access to the assessed neural data in a continuous fashion. To the best of our knowledge, for the first time in this study, we investigated the feasibility of recording human intracranial EEG (iEEG) using a benchtop version of the Brain Interchange (BIC) unit of CorTec, which is a portable, wireless, and externally powered implant with sensing and stimulation capabilities. We developed a MATLAB/SIMULINK-based rapid prototyping environment and a graphical user interface (GUI) to acquire and visualize the iEEG captured from all 32 channels of the BIC unit. We recorded prolonged iEEG (~ 24 h) from three human subjects with externalized depth leads using the BIC and commercially available clinical amplifiers simultaneously in the epilepsy monitoring unit (EMU). The iEEG signal quality of both streams was compared, and the results demonstrated a comparable power spectral density (PSD) in all the systems in the low-frequency band (< 80 Hz). However, notable differences were primarily observed above 100 Hz, where the clinical amplifiers were associated with lower noise floor (BIC-17 dB vs. clinical amplifiers <  - 25 dB). We employed an established spike detector to assess and compare the spike rates in each iEEG stream. We observed over 90% conformity between the spikes rates and their spatial distribution captured with BIC and clinical systems. Additionally, we quantified the packet loss characteristic in the iEEG signal during the wireless data transfer and conducted a series of simulations to compare the performance of different interpolation methods for recovering the missing packets in signals at different frequency bands. We noted that simple linear interpolation has the potential to recover the signal and reduce the noise floor with modest packet loss levels reaching up to 10%. Overall, our results indicate that while tethered clinical amplifiers exhibited noticeably better noise floor above 80 Hz, epileptic spikes can still be detected successfully in the iEEG recorded with the externally powered wireless BIC unit opening the road for future closed-loop neuromodulation applications with continuous access to brain activity., (© 2024. The Author(s).)

Pediatric epilepsy has a worldwide prevalence of approximately 1% (Berg et al., Handb Clin Neurol 111:391-398, 2013) and is associated with not only lower quality of life but also long-term deficits in executive function, significant psychosocial stressors, poor cognitive outcomes, and developmental delays (Schraegle and Titus, Epilepsy Behav 62:20-26, 2016; Puka and Smith, Epilepsia 56:873-881, 2015). With approximately one-third of patients resistant to medical control, surgical intervention can offer a cure or palliation to decrease the disease burden and improve neurological development. Despite its potential, epilepsy surgery is drastically underutilized. Even today only 1% of the millions of epilepsy patients are referred annually for neurosurgical evaluation, and the average delay between diagnosis of Drug Resistant Epilepsy (DRE) and surgical intervention is approximately 20 years in adults and 5 years in children (Solli et al., Epilepsia 61:1352-1364, 2020). It is still estimated that only one-third of surgical candidates undergo operative intervention (Pestana Knight et al., Epilepsia 56:375, 2015). In contrast to the stable to declining rates of adult epilepsy surgery (Englot et al., Neurology 78:1200-1206, 2012; Neligan et al., Epilepsia 54:e62-e65, 2013), rates of pediatric surgery are rising (Pestana Knight et al., Epilepsia 56:375, 2015). Innovations in surgical approaches to epilepsy not only minimize potential complications but also expand the definition of a surgical candidate. In this chapter, three alternatives to classical resection are presented. First, laser ablation provides a minimally invasive approach to focal lesions. Next, both central and peripheral nervous system stimulation can interrupt seizure networks without creating permanent lesions. Lastly, focused ultrasound is discussed as a potential new avenue not only for ablation but also modulation of small, deep foci within seizure networks. A better understanding of the potential surgical options can guide patients and providers to explore all treatment avenues., (© 2024. The Author(s), under exclusive license to Springer Nature Switzerland AG.)

Background: Surgery has become integral in treating children with tuberous sclerosis complex (TSC)-related drug-resistant epilepsy (DRE)., Objective: To describe outcomes of a multimodal diagnostic and therapeutic approach comprising invasive intracranial monitoring and surgical treatment and compare the complementary techniques of open resection and magnetic resonance-guided laser interstitial thermal therapy., Methods: Clinical and radiographic data were prospectively collected for pediatric patients undergoing surgical evaluation for TSC-related DRE at our tertiary academic hospital. Seizure freedom, developmental improvement, and Engel class were compared., Results: Thirty-eight patients (20 females) underwent treatment in January 2016 to April 2019. Thirty-five underwent phase II invasive monitoring with intracranial electrodes: 24 stereoencephalography, 9 craniotomy for grid/electrode placement, and 2 grids + stereoencephalography. With the multimodal approach, 33/38 patients (87%) achieved >50% seizure freedom of the targeted seizure type after initial treatment; 6/9 requiring secondary treatment and 2/2 requiring a third treatment achieved >50% freedom. The median Engel class was II at last follow-up (1.65 years), and 55% of patients were Engel class I/II. The mean age was lower for children undergoing open resection (2.4 vs 4.9 years, P = .04). Rates of >50% reduction in seizures (86% open resection vs 88% laser interstitial thermal therapy) and developmental improvement (86% open resection vs 83% magnetic resonance-guided laser interstitial thermal therapy) were similar., Conclusion: This hybrid approach of using both open surgical and minimally invasive techniques is safe and effective in treating DRE secondary to TSC. Clinical trials focused on treatment method with longer follow-up are needed to determine the optimal candidates for each approach and compare the treatment modalities more effectively., (Copyright © Congress of Neurological Surgeons 2022. All rights reserved.)

Introduction: Vagus nerve stimulation (VNS) is an adjunctive treatment in children with intractable epilepsy. When lead replacement becomes necessary, the old leads are often truncated and retained and new leads are implanted at a newly exposed segment of the nerve. Direct lead removal and replacement are infrequently described, with outcomes poorly characterized. We aimed to describe our experience with feasibility of VNS lead removal and replacement in pediatric patients., Methods: Retrospective review examined 14 patients, at a single, tertiary-care, children's hospital, who underwent surgery to replace VNS leads, with complete removal of the existing lead from the vagus nerve and placement of a new lead on the same segment of the vagus nerve, via blunt and sharp dissection without use of electrocautery. Preoperative characteristics, stimulation parameters, and outcomes were collected., Results: Mean age at initial VNS placement was 7.6 years (SD 3.5, range 4.5-13.4). Most common etiologies of epilepsy were genetic (5, 36%) and cryptogenic (4, 29%). Lead replacement was performed at a mean of 6.0 years (SD 3.8, range 2.1-11.7) following initial VNS placement. Reasons for revision included VNS lead breakage or malfunction. There were no perioperative complications, including surgical site infection, voice changes, dysphagia, or new deficits postoperatively. Stimulation parameters after replacement surgery at last follow-up were similar compared to preoperatively, with final stimulation parameters ranging from 0.25 mA higher to 1.5 mA lower to maintain baseline seizure control. The mean length of follow-up was 7.9 years (SD 3.5, range 3.1-13.7)., Conclusion: Removal and replacement of VNS leads are feasible and can be safely performed in children. Further characterization of surgical technique, associated risk, impact on stimulation parameters, and long-term outcomes are needed to inform best practices in VNS revision., (© 2022. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Background: Neuroimaging has evolved from anatomical imaging toward a multi-modality comprehensive anatomical and functional imaging in the past decades, important functional data like perfusion-weighted imaging, permeability imaging, diffusion-weighted imaging (DWI), and diffusion tensor imaging (DTI), tractography, metabolic imaging, connectomics, event-related functional imaging, resting state functional imaging, and much more is now being offered., Summary: Precision diagnostics has proven to be essential for precision treatment. Many minimal invasive techniques have been developed, taking advantage of digital subtraction angiography and interventional neuroradiology. Furthermore, intraoperative CT and/or MRI and more recently MR-guided focused ultrasound have complemented the diagnostic and therapeutic armamentarium., Key Messages: In the current manuscript, we discuss standard imaging sequences including advanced techniques like DWI, DTI, susceptibility-weighted imaging, and 1H magnetic resonance spectroscopy, various perfusion weighted imaging approaches including arterial spin labeling, dynamic contrast enhanced imaging, and dynamic susceptibility contrast imaging. Pre-, intra, and postoperative surgical imaging including visualize imaging will be discussed. The value of connectomics will be presented for its value in neuro-oncology. Minimal invasive therapeutic possibilities of interventional neuroradiology and image-guided laser ablation and MR-guided high-intensity-focused ultrasound will be presented for treatment of pediatric brain and spinal cord tumors. Finally, a comprehensive review of spinal cord tumors and matching neuropathology has been included., (© 2023 The Author(s). Published by S. Karger AG, Basel.)

Introduction: In carefully selected patients with medically refractory epilepsy, disconnective hemispherotomy can result in significant seizure freedom; however, incomplete disconnection can result in ongoing seizures and poses a significant challenge. Completion hemispherotomy provides an opportunity to finish the disconnection. We describe the use of magnetic resonance-guided laser interstitial thermal ablation (MRgLITT) for completion hemispherotomy., Methods: Patients treated with completion hemispherotomy using MRgLITT at our institution were identified. Procedural and seizure outcomes were evaluated retrospectively., Results: Five patients (3 males) underwent six MRgLITT procedures (one child treated twice) for completion hemispherotomy at a median age of 6 years (range 1.8-12.9). Two children had hemimegalencephaly, two had Rasmussen encephalitis, and one had polymicrogyria. All five children had persistent seizures likely secondary to incomplete disconnection after their functional hemispherotomy. The mean time from open hemispherotomy to MRgLITT was 569.5 ± 272.4 days (median 424, range 342-1,095). One patient underwent stereoelectroencephalography before MRgLITT. The mean number of ablation targets was 2.3 ± 0.47 (median 2, range 2-3). The mean length of the procedure was 373 min ± 68.9 (median 374, range 246-475). Four of the five patients were afforded improvement in their neurocognitive functioning and speech performance after ablation, with mean daily seizure frequency at 1 year of 1.03 ± 1.98 (median 0, range 0-5). Two patients achieved Engel Class I outcomes at 1 year after ablation, one was Engel Class III, and two were Engel Class IV. The mean follow-up time was 646.8 ± 179.5 days (median 634, range 384-918). No MRgLITT-related complications occurred. Delayed retreatment (&gt;1 year) occurred in three patients: one child underwent redo ablation and two underwent anatomic hemispherectomy., Conclusion: We have demonstrated the feasibility of a minimally invasive approach for completion hemispherotomy using MRgLITT. Delayed retreatment was needed in three patients; thus, further study of this technique with comparison to other surgical techniques is warranted., (© 2023 S. Karger AG, Basel.)

Objective: Since the Accreditation Council for Graduate Medical Education (ACGME) implemented duty-hour restrictions in 2003, many residency programs have adopted a night float system to comply with time constraints. However, some surgical subspecialities have been concerned that use of a night float system deprives residents of operative experience. In this study, the authors describe their training program's transition to a night float system and its impact on resident operative experience., Methods: The authors conducted a single-program study of resident surgical case volume before and after implementing the night float system at 3 of their 5 hospitals from 2014 to 2020. The authors obtained surgical case numbers from the ACGME case log database., Results: Junior residents received a concentrated educational experience, whereas senior residents saw a significant decrease from 112 calls/year to 17. Logged cases significantly increased after implementation of the night float system (8846 vs 10,547, p = 0.04), whereas cases at non-night float hospitals remained the same. This increase was concurrent with an increase in hospital cases. This difference was mainly driven by senior resident cases (p = 0.010), as junior and chief residents did not show significant differences in logged cases (p > 0.40). Lead resident cases increased significantly after implementation of the night float system (6852 vs 8860, p = 0.04). When normalized for increased hospital cases, resident case increases were not statistically significant., Conclusions: Transitioning to a night float call system at the authors' institution increased overall resident operative cases, particularly for lead resident surgeons. Based on the results of this study, the authors recommend the use of a night float call system to consolidate night calls, which increases junior resident-level educational opportunities and senior resident cases.

Aims: To identify clinical features and protein biomarkers associated with bladder cancer (BC) in individuals with type 2 diabetes mellitus presenting with haematuria., Materials and Methods: Data collected from the Haematuria Biomarker (HaBio) study was used in this analysis. A matched sub-cohort of patients with type 2 diabetes and patients without diabetes was created based on age, sex, and BC diagnosis, using approximately a 1:2 fixed ratio. Randox Biochip Array Technology and ELISA were applied for measurement of 66 candidate serum and urine protein biomarkers. Hazard ratios and 95% confidence intervals were estimated by chi-squared and Wilcoxon rank sum test for clinical features and candidate protein biomarkers. Diagnostic protein biomarker models were identified using Lasso-based binominal regression analysis., Results: There was no difference in BC grade, stage, and severity between individuals with type 2 diabetes and matched controls. Incidence of chronic kidney disease (CKD) was significantly higher in patients with type 2 diabetes (p = 0.008), and CKD was significantly associated with BC in patients with type 2 diabetes (p = 0.032). A biomarker model, incorporating two serum (monocyte chemoattractant protein 1 and vascular endothelial growth factor) and three urine (interleukin 6, cytokeratin 18, and cytokeratin 8) proteins, predicted incidence of BC with an Area Under the Curve (AUC) of 0.84 in individuals with type 2 diabetes. In people without diabetes, the AUC was 0.66., Conclusions: We demonstrate the potential clinical utility of a biomarker panel, which includes proteins related to BC pathogenesis and type 2 diabetes, for monitoring risk of BC in patients with type 2 diabetes. Earlier urology referral of patients with type 2 diabetes will improve outcomes for these patients., Trial Registration: http://www.isrctn.com/ISRCTN25823942., (© 2022 The Authors. Diabetes/Metabolism Research and Reviews published by John Wiley & Sons Ltd.)

Objective. High-frequency oscillations (HFOs) are considered a biomarker of the epileptogenic zone in intracranial EEG recordings. However, automated HFO detectors confound true oscillations with spurious events caused by the presence of artifacts. Approach. We hypothesized that, unlike pseudo-HFOs with sharp transients or arbitrary shapes, real HFOs have a signal characteristic that can be represented using a small number of oscillatory bases. Based on this hypothesis using a sparse representation framework, this study introduces a new classification approach to distinguish true HFOs from the pseudo-events that mislead seizure onset zone (SOZ) localization. Moreover, we further classified the HFOs into ripples and fast ripples by introducing an adaptive reconstruction scheme using sparse representation. By visualizing the raw waveforms and time-frequency representation of events recorded from 16 patients, three experts labeled 6400 candidate events that passed an initial amplitude-threshold-based HFO detector. We formed a redundant analytical multiscale dictionary built from smooth oscillatory Gabor atoms and represented each event with orthogonal matching pursuit by using a small number of dictionary elements. We used the approximation error and residual signal at each iteration to extract features that can distinguish the HFOs from any type of artifact regardless of their corresponding source. We validated our model on sixteen subjects with thirty minutes of continuous interictal intracranial EEG recording from each. Main results. We showed that the accuracy of SOZ detection after applying our method was significantly improved. In particular, we achieved a 96.65% classification accuracy in labeled events and a 17.57% improvement in SOZ detection on continuous data. Our sparse representation framework can also distinguish between ripples and fast ripples. Significance. We show that by using a sparse representation approach we can remove the pseudo-HFOs from the pool of events and improve the reliability of detected HFOs in large data sets and minimize manual artifact elimination., (© 2022 IOP Publishing Ltd.)

Objective: To investigate the differences in delivery mode, daily dose, and catheter tip location in pediatric patients using intrathecal baclofen (ITB) pumps with spasticity plus dystonia versus spasticity alone., Methods: A single-center, cross-sectional study was performed by collecting retrospective data from electronic medical records. Demographic and diagnostic information was obtained, comparing patients with spasticity with or without dystonia. The data were analyzed for group differences using a two-tailed Student's t-test. Categorical data were analyzed for group differences using Pearson's χ 2 test., Results: A total of 137 patients met the criteria. The majority (114) had spasticity plus dystonia whereas only 23 were documented as spasticity alone. Simple continuous dosing was the most common delivery mode, but flex dosing was used more than twice as frequently with spasticity plus dystonia compared with spasticity alone (42% vs 17%). Patients with spasticity plus dystonia also had more rostral catheter tip locations., Conclusions: While it has been discussed anecdotally, this study confirms the supposition that patients with spasticity plus dystonia have increased dose requirements when compared with those with spasticity alone. Although there are no clear standards of care when managing these patients, they are often on higher daily dosages, are more likely to require flexed dosing method, and have higher catheter placements. Still, there are few studies that demonstrate improvements in dystonia with the use of ITB. In general, these patients would benefit from the development of universal standardizations as well as the confirmation that this is an appropriate treatment., Competing Interests: Competing interests: None declared., (© Author(s) (or their employer(s)) 2022. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ.)

Objective: Periventricular nodular heterotopia (PVNH) is a result of disrupted neuronal migration from the ventricular system and can be a rare cause of refractory focal epilepsy. The goal of this case series was to describe the treatment of pediatric PVNH-related epilepsy with MR-guided laser interstitial thermal ablation., Methods: Patients treated at a single institution with MR-guided laser interstitial thermal therapy (MRgLITT) for PVNH-related epilepsy were identified. Preoperative and postoperative seizure outcomes and procedural information were evaluated., Results: Five children with PVNH treated with MRgLITT were reviewed; 1 child was treated twice. Three patients were female; the median age was 10.9 years. Five of 6 treatments were preceded by stereoelectroencephalography phase II monitoring. Three children experienced unilateral PVNH, and 2 had bilateral seizures. The median number of seizures recorded during phase II monitoring was 2; the median number of ablation targets was 2 (range 1-4). All patients experienced a decrease in seizure frequency; 4 patients (80%) had an Engel class ≤ III at the last follow-up (range I-IV). One child experienced right hemianopia posttreatment., Conclusions: This case series investigation has illustrated a novel, minimally invasive approach for treating pediatric PVNH-related epilepsy. Further study of this technique with comparison with other surgical techniques is warranted.

Texas Children's Hospital opened its doors in 1954, and since that time the institution has remained dedicated to a three-part mission: patient care, education, and research. Dr. William R. Cheek developed an early interest in pediatric neurosurgery, which led to his efforts in building and developing a service at Texas Children's Hospital at a time when the field was just emerging. His work with other early pioneers in the field led to the establishment of organized societies, educational texts, and governing bodies that have led to significant advances in the field over the past 50 years.

Background: Spastic cerebral palsy is caused by an insult to the developing brain. Various medical and surgical procedures are used to reduce tone., Objective: To describe a novel method of magnetic resonance-guided laser interstitial thermal ablation for palliative rhizotomy., Methods: Patients treated at a single institution with percutaneous rhizotomy using magnetic resonance-guided laser interstitial thermal therapy were identified. Preoperative and postoperative Modified Ashworth Scale scores were collected as well as procedural information., Results: Two male children (7.8 and 19 yr, respectively) with spastic quadriparesis were treated using this technique. Neither patient experienced surgical or perioperative complications, and both were discharged from the hospital within 48 h. Each of them demonstrated improvement in his Modified Ashworth Scale score with no need for retreatment for spasticity at last follow-up., Conclusion: These 2 cases illustrate a novel technique for treating spasticity in the setting of cerebral palsy. Further study of this technique in additional patients, and comparison with traditional methods of surgical tone reduction, are warranted., (© Congress of Neurological Surgeons 2020.)

Introduction: Hypothalamic hamartoma is rarely associated with epileptic spasms. We describe epileptic spasms in a large cohort of hypothalamic hamartoma patients., Methods: We performed a retrospective chart review between March 2011 and March 2020 to identify patients with hypothalamic hamartoma and epilepsy., Results: We identified 114 patients with hypothalamic hamartoma and epilepsy, only 3 male patients (2.6%) also had epileptic spasms. The epileptic spasms developed between 6 and 18 months of age. Epileptic spasms resolved with oral prednisolone in 1 and with vigabatrin in the second patient. The third patient continued epileptic spasms despite multiple antiepileptic drugs and partial resection of hypothalamic hamartoma. All 3 patients underwent laser-ablation of hypothalamic hamartoma at the age of 14, 29, and 63 months. The seizure burden decreased by 100%, 84%, and 93% at follow-up (3-47 months)., Conclusions: Epileptic spasms are rare in hypothalamic hamartoma patients and early laser-ablation could potentially treat epileptic spasms and all other seizure types associated with hypothalamic hamartoma.

Objective: The grid-based orthogonal placement of depth electrodes (DEs), initially defined by Jean Talairach and Jean Bancaud, is known as stereo-electroencephalography (sEEG). Although acceptance in the United States was initially slow, advances in imaging and technology have spawned a proliferation of North American epilepsy centers offering sEEG. Despite publications highlighting minimal access techniques and varied indications, standard work for phase I targeted DE has not been defined. In this article, the authors propose the term "dynamic sEEG" and define standard work tools and related common data elements to promote uniformity in the field., Methods: A multidisciplinary approach from July to August 2016 resulted in the production of 4 standard work tools for dynamic sEEG using ROSA: 1) a 34-page illustrated manual depicting a detailed workflow; 2) a planning form to collocate all the phase I data; 3) a naming convention for DEs that encodes the data defining it; and 4) a reusable portable perioperative planning and documentation board. A retrospective review of sEEG case efficiency was performed comparing those using standard work tools (between July 2016 and April 2017) with historical controls (between March 2015 and June 2016). The standard work tools were then instituted at another epilepsy surgery center, and the results were recorded., Results: The process for dynamic sEEG was formally reviewed, including anesthesia, positioning, perioperative nursing guidelines, surgical steps, and postoperative care for the workflow using cranial fixation and ROSA-guided placement. There was a 40% improvement in time per electrode, from 44.7 ± 9.0 minutes to 26.9 ± 6.5 minutes (p = 0.0007) following the development and use of the manual, the naming convention, and the reusable portable perioperative planning and documentation board. This standardized protocol was implemented at another institution and yielded a time per electrode of 22.3 ± 4.4 minutes., Conclusions: The authors propose the term dynamic sEEG for stereotactic depth electrodes placed according to phase I workup data with the intention of converting to ablation. This workflow efficiency can be optimized using the standard work tools presented. The authors also propose a novel naming convention that encodes critical data and allows portability among providers. Use of a planning form for common data elements optimizes research, and global adoption could facilitate multicenter studies correlating phase I modality and seizure onset zone identification.

Objective: Several small series have described stereotactic MRI-guided laser interstitial thermal therapy for partial callosotomy of astatic and generalized tonic-clonic (GTC) seizures, especially in association with Lennox-Gastaut syndrome. Larger case series and comparison of distinct stereotactic methods for stereotactic laser corpus callosotomy (SLCC), however, are currently lacking. The objective of this study was to report seizure outcomes in a series of adult patients with epilepsy following anterior, posterior, and complete SLCC procedures and to compare the results achieved with a frameless stereotactic surgical robot versus direct MRI guidance frames., Methods: The authors retrospectively reviewed sequential adult epilepsy surgery patients who underwent SLCC procedures at a single institution. They describe workflows, stereotactic errors, percentage disconnection, hospitalization durations, adverse events, and seizure outcomes after performing anterior, posterior, and complete SLCC procedures using a frameless stereotactic surgical robot versus direct MRI guidance platforms., Results: Thirteen patients underwent 15 SLCC procedures. The median age at surgery was 29 years (range 20-49 years), the median duration of epilepsy was 21 years (range 9-48 years), and median postablation follow-up was 20 months (range 4-44 months). Ten patients underwent anterior SLCC with a median 73% (range 33%-80%) midsagittal length of callosum acutely ablated. Following anterior SLCC, 6 of 10 patients achieved meaningful (> 50%) reduction of target seizures. Four patients underwent posterior (completion) SLCC following prior anterior callosotomy, and 1 patient underwent complete SLCC as a single procedure; 3 of these 5 patients experienced meaningful reduction of target seizures. Overall, 8 of 10 patients in whom astatic seizures were targeted and treated by anterior and/or posterior SLCC experienced meaningful improvement. SLCC procedures with direct MRI guidance (n = 7) versus a frameless surgical robot (n = 8) yielded median radial accuracies of 1.1 mm (range 0.2-2.0 mm) versus 2.4 mm (range 0.6-6.1 mm; p = 0.0011). The most serious adverse event was a clinically significant intraparenchymal hemorrhage in a patient who underwent the robotic technique., Conclusions: This is the largest reported series of SLCC for epilepsy to date. SLCC provides seizure outcomes comparable to open surgery outcomes reported in the literature. Direct MRI guidance is more accurate, which has the potential to reduce the risks of SLCC. Methodological advancements and larger studies are needed.

Epilepsy occurs in nearly all patients with tuberous sclerosis and is often refractory to medical treatment. The definition of surgical candidacy in these patients has broadened in recent years due to philosophical and technological advances. The goals of surgery have shifted to focusing on quality of life and maximizing neurodevelopmental potential in patients unable to obtain seizure freedom. Novel diagnostic, ablative, and neuromodulatory techniques have been developed that may help patients that were previously considered inoperable to have an improved quality of life. In the coming years, it is expected that these techniques will be further refined and lead to an improvement of neurological prognosis in patients with tuberous sclerosis.

Background: Hypothalamic hamartomas (HH) are a challenging pathology that cause gelastic seizures. Magnetic Resonance Imaging-guided Laser Interstitial Thermal Therapy (MRgLITT) offers a safe and effective treatment for HHs via a minimally invasive technique., Objective: To determine how clinical outcome correlates to residual tumor volume and surgical strategy by analyzing radiographic data and reconstructing volumetric imaging., Methods: Clinical and radiographic information of 58 pediatric patients who underwent MRgLITT for HH with at least 6 mo of follow-up were retrospectively reviewed. MR imaging was volumetrically reconstructed to analyze the impact of hamartoma and ablation volumes on outcome. Primary outcome measure was freedom from gelastic seizures., Results: Eighty-one percent of patients were completely free of gelastic seizures at last follow-up; of 22 patients with secondary nongelastic epilepsy, 15 were free of additional seizures. Postoperative complication rate was low. There was no significant difference in gelastic seizure outcome related to pre- or postoperative hamartoma size. Residual hamartoma percentage in those free of gelastic seizures was 43% compared to 71% in those with continued seizures (P = .021). Larger hamartomas required multiple ablations to achieve seizure freedom., Conclusion: This large series of patients confirms the safety and efficacy of MRgLITT for pediatric HH and describes morphological considerations that predict success. Our data suggest that complete ablation of the lesion is not necessary, and that the focus should be on appropriate disconnection of the epileptogenic network. We have found that a staged approach to hamartoma ablation allows adequate disconnection of the hamartoma while mitigating risk to surrounding structures., (Copyright © 2019 by the Congress of Neurological Surgeons.)

This article overviews the surgical options for hypertonia management in cerebral palsy, both spasticity and dystonia. We review the history and use of intrathecal baclofen. We contrast its use with the indications for selective dorsal rhizotomy and review how it is the optimal technique to lower tone in the ambulatory spastic diplegic patient with cerebral palsy. This article reviews the advent of deep brain stimulation, with an emphasis on selection criteria and expected outcomes in this population. The article reviews the principles and use of selective peripheral neurotomy as it is applied to focal spasticity not requiring systemic tone reduction., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Objective: To describe the findings of histopathology and genotyping studies in affected brain tissue from an individual with phacomatosis pigmentovascularis (PPV)., Methods: A retrospective chart review of a 2-year 10-month-old male with a clinical diagnosis of PPV cesiomarmorata (or type V) was performed. Clinical features, brain imaging and histopathology findings, and genotyping studies in his affected brain tissue are summarized., Results: The proband had a clinically severe neurologic phenotype characterized by global developmental delay, generalized hypotonia, and recurrent episodes of cardiac asystole in the setting of status epilepticus. A somatic pathogenic variant in GNA11 (c.547C>T, p.Arg183Cys) was detected in his skin tissue but not in blood (previously published). He underwent an urgent left posterior quadrantectomy for his life-threatening seizures. Histopathology of resected brain tissue showed an increase in leptomeningeal melanocytes and abnormal vasculature, and the exact pathogenic variant in GNA11 (c.547C>T, p.Arg183Cys), previously isolated from his skin tissue but not blood, was detected in his resected brain tissue., Conclusions: The finding of this variant in affected skin and brain tissue of our patient with PPV supports a unifying genetic diagnosis of his neurocutaneous features., (Copyright © 2019 The Author(s). Published by Wolters Kluwer Health, Inc. on behalf of the American Academy of Neurology.)

Objective: Postoperative resting-state functional magnetic resonance imaging (MRI) in children with intractable epilepsy has not been quantified in relation to seizure outcome. Therefore, its value as a biomarker for epileptogenic pathology is not well understood., Methods: In a sample of children with intractable epilepsy who underwent prospective resting-state seizure onset zone (SOZ)-targeted epilepsy surgery, postoperative resting-state functional MRI (rs-fMRI) was performed 6 to 12 months later. Graded normalization of the postoperative resting-state SOZ was compared to seizure outcomes, patient, surgery, and anatomical MRI characteristics., Results: A total of 64 cases were evaluated. Network-targeted surgery, followed by postoperative rs-fMRI normalization was significantly (p < 0.001) correlated with seizure reduction, with a Spearman rank correlation coefficient of 0.83. Of 39 cases with postoperative rs-fMRI SOZ normalization, 38 (97%) became completely seizure free. In contrast, of the 25 cases without complete rs-fMRI SOZ normalization, only 3 (5%) became seizure free. The accuracy of rs-fMRI as a biomarker predicting seizure freedom is 94%, with 96% sensitivity and 93% specificity., Interpretation: Among seizure localization techniques in pediatric epilepsy, network-targeted surgery, followed by postoperative rs-fMRI normalization, has high correlation with seizure freedom. This study shows that rs-fMRI SOZ can be used as a biomarker of the epileptogenic zone, and postoperative rs-fMRI normalization is a biomarker for SOZ quiescence. ANN NEUROL 2019;86:344-356., (© 2019 The Authors. Annals of Neurology published by Wiley Periodicals, Inc. on behalf of American Neurological Association.)

Background: Tethered cord syndrome (TCS) refers to a clinical constellation of signs and symptoms associated with tension in the spinal cord and associated with pathologies related to abnormally low-lying conus medullaris. As minimally invasive spinal operations have become more prevalent in the past decade, we applied expanding indications to selected intradural procedures, such as tethered cord release (TCR). We present the first series of minimally invasive muscle-sparing TCR in the pediatric population., Methods: A retrospective review of the Texas Children's Hospital, Houston, neurosurgical database was conducted for patients who underwent TCR between 2010 and 2017. Charts were reviewed to determine the source of TCS and whether surgery was conducted in a minimally invasive fashion. To establish a cohort of control population, age-matched cases of open TCR were selected in the same. Exclusion criterion was non-fatty filum sources of TCS. The length of stay, operative time, estimated blood loss, and postoperative complications were recorded., Results: Eleven patients underwent minimally invasive TCR. Mean age of the patients was 10.1 years. All patients underwent L4-5 tubular laminotomy and cord detethering by 2 pediatric neurosurgeons at Texas Children's Hospital. The average length of stay in the hospital was 2.6 days. The average operative time was 167 minutes and average estimated blood loss was 8.2 mL. Ten patients reported clinical improvement and 1 patient was lost to follow-up., Conclusions: Minimally invasive TCR is a safe and represents a viable alternative to the open approach with advantages such as minimal estimated blood loss, shortened length of stay, and postoperative pain control., (Copyright © 2019 Elsevier Inc. All rights reserved.)

In Brief: The study compared two types of functional MRI (fMRI) to see which side of the brain is most responsible for language: traditional task-based fMRI, which requires a high level of patient interaction, and resting-state fMRI, which is typically performed with the patient under light sedation and has no interaction requirement. The authors found that the test correlation was 93%, indicating resting state fMRI has potential to locate language in those unable to participate in task-based fMRI.

Objective: The purpose of this study is to investigate the outcomes of epilepsy surgery targeting the subcentimeter-sized resting state functional magnetic resonance imaging (rs-fMRI) epileptogenic onset zone (EZ) in hypothalamic hamartoma (HH)., Methods: Fifty-one children with HH-related intractable epilepsy received anatomical MRI-guided stereotactic laser ablation (SLA) procedures. Fifteen of these children were control subjects (CS) not guided by rs-fMRI. Thirty-six had been preoperatively guided by rs-fMRI (RS) to determine EZs, which were subsequently targeted by SLA. The primary outcome measure for the study was a predetermined goal of 30% reduction in seizure frequency and improvement in class I Engel outcomes 1 year postoperatively. Quantitative and qualitative volumetric analyses of total HH and ablated tissue were also assessed., Results: In the RS group, the EZ target within the HH was ablated with high accuracy (>87.5% of target ablated in 83% of subjects). There was no difference between the groups in percentage of ablated hamartoma volume (P = 0.137). Overall seizure reduction was higher in the rs-fMRI group: 85% RS versus 49% CS (P = 0.0006, adjusted). The Engel Epilepsy Surgery Outcome Scale demonstrated significant differences in those with freedom from disabling seizures (class I), 92% RS versus 47% CS, a 45% improvement (P = 0.001). Compared to prior studies, there was improvement in class I outcomes (92% vs 76%-81%). No postoperative morbidity or mortality occurred., Significance: For the first time, surgical SLA targeting of subcentimeter-sized EZs, located by rs-fMRI, guided surgery for intractable epilepsy. Our outcomes demonstrated the highest seizure freedom rate without surgical complications and are a significant improvement over prior reports. The approach improved freedom from seizures by 45% compared to conventional ablation, regardless of hamartoma size or anatomical classification. This technique showed the same or reduced morbidity (0%) compared to recent non-rs-fMRI-guided SLA studies with as high as 20% permanent significant morbidity., (© 2018 The Authors. Epilepsia published by Wiley Periodicals, Inc. on behalf of International League Against Epilepsy.)

Hypothalamic hamartoma is an archetypal example of subcortical epilepsy that can be associated with intractable gelastic epilepsy, secondary epilepsy, and epileptic encephalopathy. The history of its surgical treatment is fraught with mislocalization of the seizure focus, modest efficacy and a high complication rate. Many minimally invasive techniques have been described to mitigate this high complication profile of which MR-guided laser ablation is one. The technology combines instant effect of thermal coagulation with stereotactic precision and guidance with real time MR thermography. This article presents a series of 71 hypothalamic hamartoma patients operated with laser ablation. Ninety-three percent (93%) were free of gelastic seizures at one year with 23% of the patients requiring more than one ablation. One patient experienced a significant memory deficit and one patient experienced worsening diabetes insipidus. Stereotactic laser ablation appears to be a safe and effective surgical option in the treatment of hypothalamic hamartoma., (Copyright © 2018 Elsevier B.V. All rights reserved.)

Resting-state functional magnetic resonance imaging (rsfMRI) is emerging as a useful tool in the multimodal assessment of patients with epilepsy. In pediatric patients who cannot perform task-based fMRI, rsfMRI may present an adjunct and alternative. Although changes in brain activation during task-based fMRI have been described after surgery for epilepsy, there is limited data on the role of postoperative rsfMRI. In this short review, we discuss the role of postoperative rsfMRI after laser ablation of seizure foci. By establishing standardized anesthesia protocols and imaging parameters, we have been able to perform serial rsfMRI at postoperative follow-up. The development of in-house software that can merge rsfMRI images to surgical navigation systems has allowed us to enhance the clinical applications of this technique. Resting-state fMRI after laser ablation has the potential to identify changes in connectivity, localize new seizure foci, and guide antiepileptic therapy. In our experience, rsfMRI complements conventional MR imaging and task-based fMRI for the evaluation of patients with seizure recurrence after laser ablation, and represents a potential noninvasive biomarker for functional connectivity., (Copyright © 2017 The Authors. Published by Elsevier B.V. All rights reserved.)

OBJECTIVE Intrathecal baclofen pumps are generally placed in children for the treatment of spasticity and dystonia. Use of implants in this pediatric population with comorbidities is reported to have a high risk of complications and infections. With the aim of reducing baclofen pump-related infections, a quality improvement project was instituted at the authors' institution. METHODS A workflow paradigm unique to baclofen pump implantation aimed at decreasing implant-related infections was implemented. All baclofen pump operations performed at the authors' institution between August 2012 and June 2016 were reviewed. An infection prevention protocol was created and implemented in August 2014 based on a literature review and the consensus opinion of the pediatric neurosurgeons in the group. Compliance with the prevention bundle was tracked. Case outcomes before and after implementation of the protocol with a minimum of 3 months of follow-up were retrospectively reviewed. Univariate and multivariate analyses were performed to assess the association of the steps in the prevention bundle with infection and complication outcomes. RESULTS A total of 128 baclofen pump surgeries were performed (64 preprotocol and 64 postprotocol). The patient age range was 3 to 27 years. The overall compliance rate with the infection prevention bundle was 82%. The pre- and postimplementation infection rates were 12.5% and 6.3%, respectively (p = 0.225). The total pre- and postimplementation complication rates were 23.4% and 9.4%, respectively (p = 0.032). The absolute and relative risk reductions for infections were 6.3% (95% CI 3.8%-16.3%) and 50%, respectively; for complications, the absolute and relative risk reductions were 14.1% (95% CI 1.5%-26.7%) and 60%, respectively. CONCLUSIONS The total complication rate following intrathecal baclofen pump surgery was significantly lower after implementation of the quality improvement protocol. This study is an example of using checklist standardization to diminish special cause variability.

High-frequency oscillations in local field potentials recorded with intracranial EEG are putative biomarkers of seizure onset zones in epileptic brain. However, localized 80-500 Hz oscillations can also be recorded from normal and non-epileptic cerebral structures. When defined only by rate or frequency, physiological high-frequency oscillations are indistinguishable from pathological ones, which limit their application in epilepsy presurgical planning. We hypothesized that pathological high-frequency oscillations occur in a repetitive fashion with a similar waveform morphology that specifically indicates seizure onset zones. We investigated the waveform patterns of automatically detected high-frequency oscillations in 13 epilepsy patients and five control subjects, with an average of 73 subdural and intracerebral electrodes recorded per patient. The repetitive oscillatory waveforms were identified by using a pipeline of unsupervised machine learning techniques and were then correlated with independently clinician-defined seizure onset zones. Consistently in all patients, the stereotypical high-frequency oscillations with the highest degree of waveform similarity were localized within the seizure onset zones only, whereas the channels generating high-frequency oscillations embedded in random waveforms were found in the functional regions independent from the epileptogenic locations. The repetitive waveform pattern was more evident in fast ripples compared to ripples, suggesting a potential association between waveform repetition and the underlying pathological network. Our findings provided a new tool for the interpretation of pathological high-frequency oscillations that can be efficiently applied to distinguish seizure onset zones from functionally important sites, which is a critical step towards the translation of these signature events into valid clinical biomarkers.awx374media15721572971001.

MRI-guided laser interstitial thermal therapy for epilepsy (LITT-E) has become an established, minimally invasive alternative to traditional epilepsy surgery. LITT-E is particularly valuable in cases in which open surgery poses unacceptably high morbidity or patient preference precludes craniotomy. Here we present a focused review of technical details and application of LITT to both focal and generalized epilepsy., (Copyright © 2017 Elsevier Inc. All rights reserved.)

The purpose of this study was to prospectively investigate the agreement between the epileptogenic zone(s) (EZ) localization by resting-state functional magnetic resonance imaging (rs-fMRI) and the seizure onset zone(s) (SOZ) identified by intracranial electroencephalogram (ic-EEG) using novel differentiating and ranking criteria of rs-fMRI abnormal independent components (ICs) in a large consecutive heterogeneous pediatric intractable epilepsy population without an a priori alternate modality informing EZ localization or prior declaration of total SOZ number. The EZ determination criteria were developed by using independent component analysis (ICA) on rs-fMRI in an initial cohort of 350 pediatric patients evaluated for epilepsy surgery over a 3-year period. Subsequently, these rs-fMRI EZ criteria were applied prospectively to an evaluation cohort of 40 patients who underwent ic-EEG for SOZ identification. Thirty-seven of these patients had surgical resection/disconnection of the area believed to be the primary source of seizures. One-year seizure frequency rate was collected postoperatively. Among the total 40 patients evaluated, agreement between rs-fMRI EZ and ic-EEG SOZ was 90% (36/40; 95% confidence interval [CI], 0.76-0.97). Of the 37 patients who had surgical destruction of the area believed to be the primary source of seizures, 27 (73%) rs-fMRI EZ could be classified as true positives, 7 (18%) false positives, and 2 (5%) false negatives. Sensitivity of rs-fMRI EZ was 93% (95% CI 78-98%) with a positive predictive value of 79% (95% CI, 63-89%). In those with cryptogenic localization-related epilepsy, agreement between rs-fMRI EZ and ic-EEG SOZ was 89% (8/9; 95% CI, 0.52-99), with no statistically significant difference between the agreement in the cryptogenic and symptomatic localization-related epilepsy subgroups. Two children with negative ic-EEG had removal of the rs-fMRI EZ and were seizure free 1 year postoperatively. Of the 33 patients where at least 1 rs-fMRI EZ agreed with the ic-EEG SOZ, 24% had at least 1 additional rs-fMRI EZ outside the resection area. Of these patients with un-resected rs-fMRI EZ, 75% continued to have seizures 1 year later. Conversely, among 75% of patients in whom rs-fMRI agreed with ic-EEG SOZ and had no anatomically separate rs-fMRI EZ, only 24% continued to have seizures 1 year later. This relationship between extraneous rs-fMRI EZ and seizure outcome was statistically significant (p = 0.01). rs-fMRI EZ surgical destruction showed significant association with postoperative seizure outcome. The pediatric population with intractable epilepsy studied prospectively provides evidence for use of resting-state ICA ranking criteria, to identify rs-fMRI EZ, as developed by the lead author (V.L.B.). This is a high yield test in this population, because no seizure nor particular interictal epilepiform activity needs to occur during the study. Thus, rs-fMRI EZ detected by this technique are potentially informative for epilepsy surgery evaluation and planning in this population. Independent of other brain function testing modalities, such as simultaneous EEG-fMRI or electrical source imaging, contextual ranking of abnormal ICs of rs-fMRI localized EZs correlated with the gold standard of SOZ localization, ic-EEG, across the broad range of pediatric epilepsy surgery candidates, including those with cryptogenic epilepsy.

Epilepsy associated with cavernous malformation (CM) often requires surgical resection of seizure focus to achieve seizure-free outcome. High-frequency oscillations (HFOs) in intracranial electroencephalogram (EEG) are reported as potential biomarkers of epileptogenic regions, but to our knowledge there are no data on the existence of HFOs in CM-caused epilepsy. Here we report our experience of the identification of the seizure focus in a 3-year-old pediatric patient with intractable epilepsy associated with CM. The electrocorticographic recordings were obtained from a 64-contact grid over 2 days in the epilepsy monitoring unit (EMU). The spatial distribution of HFOs and epileptic spikes were estimated from recording segments right after the electrode placement, during sleep and awake states separately. The HFO distribution showed consistency with the perilesional region; the location of spikes varied over days and did not correlate with the lesion. The HFO spatial distribution was more compact in sleep state and pinpointed the contacts sitting on the CM border. Following the resection of the CM and the hemosiderin ring, the patient became seizure-free. This is the first report describing HFOs in a pediatric patient with intractable epilepsy associated with CM and shows their potential in identifying the seizure focus.

Purpose: Preoperative antibiosis contributes up to one third of total antibiotic use in major hospitals. Choice of antibiotic is not uniformly standardized, and polypharmacy regimens may be used without knowing the effect on rates of surgical site infection, nonsurgical infections, or antibiotic resistance. Careful examination of trends in surgical prophylaxis is warranted. In this study, we aimed to examine our institution's experience with vagus nerve stimulator (VNS) implantation, focusing on association between perioperative antibiotic practices and postoperative infectious outcomes., Methods: We conducted a single-center case-control study using a retrospective chart review of 50 consecutively operated patients undergoing VNS implantation over 24 months by two experienced surgeons at our institution from April 2014 to March 2016. In each surgery, the technical procedure, operating room, and surgical team were the same, while surgeon's preference in antibiotic prophylaxis differed. Group 1 received a single dose of intravenous (IV) cefazolin (n = 26), and Group 2 received IV cefazolin, paired with one or both of gentamicin/vancomycin, in addition to a 10-day outpatient oral course of clindamycin (n = 24). Patient demographics, perioperative details, and minimum 3-month follow-up for infection and healthcare utilization were recorded. Student t tests were computed for significance., Results: Group 1 patients on average were older than group 2 patients (10.2, 7.1 years, p = 0.01), and length of surgery was longer (115.5, 91.9 min, p = 0.007). There were no differences in number of surgeons gowned (p = 0.11), presence of tracheostomy (p = 0.43) or gastrostomy (p = 0.20) tube, nonsurgical infections (p = 0.32), and number of postoperative emergency department (ED) visits (p = 0.22) or readmissions (p = 0.23). Neither group had VNS infections in the follow-up period., Conclusion: Single preoperative dosing of one antibiotic appropriately chosen to cover typical skin flora conferred equal benefit to perioperative prophylactic polypharmacy in this study. There were no differences in postoperative infection events or ED visits/readmissions. Restraint with preoperative antibiosis shows equipoise in postoperative infection and overall resource utilization.

Background: Patients sustaining a fractured neck of the femur are typically of advanced age with multiple comorbidities. As a consequence, the proportion of these patients receiving warfarin therapy is approximately 10%. There are currently few studies investigating outcomes in this subset of patients., Questions/purposes: The purpose of this study was to assess the association between warfarin therapy and time to surgery, length of hospital stay, and survival in patients sustaining a fractured neck of the femur., Methods: Data for 2036 patients admitted to our center between July 2009 and July 2014 with a fractured neck of the femur were extracted from the National Hip Fracture Database. Fifty-seven patients received no surgical treatment and were excluded from analysis. Multivariable ordinary least squares regression was performed to test the association between warfarin treatment on time to surgery and length of stay, and Cox proportional hazards to test followup survival. Variables included in the regression model were age, sex, American Society of Anesthesiologists (ASA) score, admission Abbreviated Mental Test Score (AMTS), fracture type, operation type, and premorbid Work Ability Index (WAI). One hundred fifty-two of 1979 surgically treated patients (8%) were receiving warfarin therapy at the time of admission., Results: After controlling for age, sex, ASA score, AMTS, fracture type, operation type, and WAI, we found that patients taking warfarin were less likely to go to surgery by 36 hours (odds ratio [OR], 0.20; 95% CI, 0.14-0.30), and less likely to go to surgery by 48 hours (OR, 0.17; 95% CI, 0.11-0.24). Patients taking warfarin had a longer length of stay (median, 15 days; interquartile range [IQR], 12-22 days) compared with patients not taking warfarin (median, 13 days; IQR, 9-20 days; p < 0.001). Survival analysis to June 2015 showed a higher mortality for patients taking warfarin (12-month survival, 66% vs 76%; hazard ratio, 1.57; 95% CI, 1.21-2.04; p < 0.001)., Conclusions: After controlling for multiple prognostic factors such as age, ASA score, AMTS, and WAI, warfarin therapy at the time of injury is associated with increased time to surgery, length of stay, and decreased survival. This study highlights the need to view warfarin therapy as a 'red flag' in patients presenting with a fractured neck of the femur. Preoperatively, prompt warfarin reversal together with adequate investigation and optimization of the patient should ensure timely, safe surgery. Early involvement of the anesthesia team should ensure an appropriate level of postoperative care for these patients., Level of Evidence: Level III, therapeutic study.

Objective: The aims of this study were to evaluate a clinically practical functional connectivity protocol designed to blindly identify the corresponding areas of initial seizure propagation and also to differentiate these areas from remote secondary areas affected by seizure. The patients in this cohort had intractable epilepsy caused by intrahypothalamic hamartoma, which is the location of the ictal focus. The ictal propagation pathway is homogeneous and established, thus creating the optimum situation for the proposed method validation study., Methods: Twelve patients with seizures from hypothalamic hamartoma and 6 normal control patients underwent resting state functional MRI, using independent component analysis to identify network differences in patients. This was followed by seed-based connectivity measures to determine the extent of functional connectivity derangement between hypothalamus and these areas. The areas with significant change in connectivity were compared with the results of prior studies' modalities used to evaluate seizure propagation., Results: The left amygdala-parahippocampal gyrus area, cingulate gyrus, and occipito-temporal gyrus demonstrated the highest derangement in connectivity with the hypothalamus, p < 0.01, corresponding to the initial seizure propagation areas established by prior modalities. Areas of secondary ictal propagation were differentiated from these initial locations by first being identified as an abnormal neuronal signal source via independent component analysis, but did not show significant connectivity directly with the known ictal focus., Conclusion: Non-invasive connectivity measures correspond to areas of initial ictal propagation and differentiate such areas from secondary ictal propagation, which may aid in ictal focus surgical disconnection planning and support the use of this newer modality for adjunctive information in epilepsy surgery evaluation., Competing Interests: Author Disclosure Statement No competing financial interests exist.

Aims: We present an audit comparing our level I major trauma centre's data for a cohort of patients with hip fractures in the National Hip Fracture Database (NHFD) with locally held data on these patients., Patients and Methods: A total of 2036 records for episodes between July 2009 and June 2014 were reviewed., Results: The demographics of nine patients were recorded incorrectly. The rate of incorrect data in operation codes was most significant with overall accuracy of 0.637 (95% CI 0.615 to 0.658). The sensitivity of NHFD coding ranged from 0.250 to 1.000 and the specificity 0.879 to 0.999. The recording of cementation had a sensitivity of 0.932 and specificity of 0.713. The recording of total hip arthroplasty had a sensitivity of 0.739 and specificity of 0.983. The overall accuracy of mortality data was 0.942 (95% CI 0.931 to 0.952), with sensitivity of 0.967 and specificity of 0.419., Conclusion: This paper highlights the need for local audit of the integrity of data uploaded to the NHFD. Cite this article: Bone Joint J 2016;98-B:1406-9., (©2016 The British Editorial Society of Bone & Joint Surgery.)

The vagus nerve stimulator (VNS) has been shown to provide a safe, albeit costly, treatment for intractable epilepsy. We aimed to analyze the incidence, timing, and clinical/demographic associations of revision surgery post-VNS implantation in epilepsy patients. The Thomson Reuters MarketScan database, containing data from 23-50million individuals, was used. Epilepsy patients receiving VNS implantations from 2003 to 2009 were identified by Current Procedural Terminology and International Classification Of Diseases Ninth Revision codes. Incidence and timing of subsequent implant-related surgeries were recorded. Events were described using time-to-event methodology, with Kaplan-Meier failure estimation/Cox proportional hazard models adjusted for clinical/demographic factors. In 1234 patients, average incidence of revision surgeries over 6years of follow-up were <1%, <3%, 4-10%, and <1% for VNS electrode revision, battery revision/removal, battery replacement/implantation, and infection washout, respectively. For electrode revision and battery revision/replacement, the incidence was higher in the first year and for battery replacement in later years. Age, sex, insurance type, or geographic region did not significantly impact event occurrence. Implant-related revision surgeries are rare. Some events occur more often in certain follow-up years than others; none are significantly impacted by age, sex, insurance type, or geographic region. The most common reason for revision was battery replacement several years after VNS placement., (Copyright © 2016 Elsevier Ltd. All rights reserved.)

A male patient in his fifties presented to his local hospital with numbness and weakness of the right leg which left him unable to mobilise. He reported injecting heroin the previous morning. Following an initial diagnosis of acute limb ischaemia the patient was transferred to a tertiary centre where Computed Tomography Angiography was reported as normal. Detailed neurological examination revealed weakness in hip flexion and extension (1/5 on the Medical Research Council scale) with complete paralysis of muscle groups distal to this. Sensation to pinprick and light touch was globally reduced. Blood tests revealed acute kidney injury with raised creatinine kinase and the patient was treated for rhabdomyolysis. Orthopaedic referral was made the following day and a diagnosis of gluteal compartment syndrome (GCS) was made. Emergency fasciotomy was performed 56 hours after the onset of symptoms. There was immediate neurological improvement following decompression and the patient was rehabilitated with complete nerve recovery and function at eight-week follow-up. This is the first documented case of full functional recovery following a delayed presentation of GCS with sciatic nerve palsy. We discuss the arguments for and against fasciotomy in cases of compartment syndrome with significant delay in presentation or diagnosis.

Deep brain stimulation (DBS) has improved the prospects for many individuals with diseases affecting motor control, and recently it has shown promise for improving cognitive function as well. Several studies in individuals with Alzheimer disease and in amnesic rats have demonstrated that DBS targeted to the fimbria-fornix, the region that appears to regulate hippocampal activity, can mitigate defects in hippocampus-dependent memory. Despite these promising results, DBS has not been tested for its ability to improve cognition in any childhood intellectual disability disorder. Such disorders are a pressing concern: they affect as much as 3% of the population and involve hundreds of different genes. We proposed that stimulating the neural circuits that underlie learning and memory might provide a more promising route to treating these otherwise intractable disorders than seeking to adjust levels of one molecule at a time. We therefore studied the effects of forniceal DBS in a well-characterized mouse model of Rett syndrome (RTT), which is a leading cause of intellectual disability in females. Caused by mutations that impair the function of MeCP2 (ref. 6), RTT appears by the second year of life in humans, causing profound impairment in cognitive, motor and social skills, along with an array of neurological features. RTT mice, which reproduce the broad phenotype of this disorder, also show clear deficits in hippocampus-dependent learning and memory and hippocampal synaptic plasticity. Here we show that forniceal DBS in RTT mice rescues contextual fear memory as well as spatial learning and memory. In parallel, forniceal DBS restores in vivo hippocampal long-term potentiation and hippocampal neurogenesis. These results indicate that forniceal DBS might mitigate cognitive dysfunction in RTT.

Objective: Hemispherectomy is an established surgical treatment for carefully selected pediatric patients with intractable epilepsy. Published perioperative data report low mortality rates and seizure reduction rates of 50-89%. This study investigates trends in the demographics, hospital utilization, and in-hospital complication rates of patients undergoing hemispherectomy over the past decade in the United States, using the nationally representative Kids' Inpatient Database (KID)., Methods: The KID was queried for all discharges with the International Classification of Diseases, Ninth Revision, Clinical Modification (ICD-9-CM) procedure code for hemispherectomy in the years 2000, 2003, 2006, and 2009. The patient cohorts from these four time points were compared, analyzing differences in demographic data, insurance and payer status, total cost, length of stay, in-hospital mortality, and complications. National estimates and 95% confidence intervals are reported given the weighted sample design of KID., Results: This study identified an estimated total of 552 hospital admissions for hemispherectomy surgery during the years studied in this cohort. The incidence of this procedure increased from 1.2/100,000 admissions in 2000 to 2.2/100,000 in 2009 (p=0.05). Mean age was 6.7 years (range 0-20). There were no significant changes in demographics (age, gender, or race), hospital descriptors (size or type), insurance type, or zip code income quartile. There was a significant increase in total cost, from $42,807 in 2003 to $57,443 in 2009 (p=0.015) (adjusted to 2009 dollars). There were no trends in postoperative complications. In-hospital mortality occurred in five subjects (0.9%). Ventricular shunt placement during hemispherectomy hospitalizations increased over time from 6.7% to 16.5% (p=0.056). Hospitals that performed two or more hemispherectomies yearly had a significantly decreased incidence of in-hospital mortality (odds ratio [OR] 0.08, p=0.04) and an increased incidence of blood transfusion (OR 3.7, p=0.01) compared to hospitals that performed 0-1 procedures a year., Significance: Hemispherectomy procedures increased slightly in frequency over the past decade, with no change in demographic characteristics of the patients over time. Rates of mortality and perioperative complications remained low. Total costs have increased significantly over time. In-hospital mortality was lower in higher volume hospitals., (Wiley Periodicals, Inc. © 2015 International League Against Epilepsy.)