Faculty Bibliography

OBJECTIVE:Accreditation Council for Graduate Medical Education (ACGME)-accredited epilepsy fellowships, like other ACGME accredited training programs, use Milestones to establish learning objectives and to evaluate how well trainees are achieving these goals. The ACGME began developing the second iteration of the Milestones 6 years ago, and these are now being adapted to all specialties. Here, we describe the process by which Epilepsy Milestones 2.0 were developed and summarize them. METHODS:A work group of nine board-certified, adult and pediatric epileptologists reviewed Epilepsy Milestones 1.0 and revised them using a modified Delphi approach. RESULTS:The new Milestones share structural changes with all other specialties, including a clearer stepwise progression in professional development and the harmonized Milestones that address competencies common to all medical fields. Much of the epilepsy-specific content remains the same, although a major addition is a set of Milestones focused on reading and interpreting electroencephalograms (EEGs), which the old Milestones lacked. Epilepsy Milestones 2.0 includes a Supplemental Guide to help program directors implement the new Milestones. Together, Epilepsy Milestones 2.0 and the Supplemental Guide recognize advances in epilepsy, including stereo-EEG, neurostimulation, genetics, and safety in epilepsy monitoring units. SIGNIFICANCE:Epilepsy Milestones 2.0 address the shortcomings of the old Milestones and should facilitate the assessment of epilepsy fellowships and fellows by program directors, faculty, and fellows themselves.

There is an urgent need for an in-depth and systematic assessment of a wide range of predictive factors related to populations most at risk for delaying and refusing COVID-19 vaccination as cases of the disease surge across the United States. Many studies have assessed a limited number of general sociodemographic and health-related factors related to low vaccination rates. Machine learning methods were used to assess the association of 151 social and health-related risk factors derived from the American Community Survey 2019 and the Centers for Disease Control and Prevention (CDC) BRFSS with the response variables of vaccination rates and unvaccinated counts in 1,555 ZIP Codes in California. The performance of various analytical models was evaluated according to their ability to regress between predictive variables and vaccination levels. Machine learning modeling identified the Gradient Boosting Regressor (GBR) as the predictive model with a higher percentage of the explained variance than the variance identified through linear and generalized regression models. A set of 20 variables explained 72.90% of the variability of unvaccinated counts among ZIP Codes in California. ZIP Codes were shown to be a more meaningful geo-local unit of analysis than county-level assessments. Modeling vaccination rates was not as effective as modeling unvaccinated counts. The public health utility of this model provides for the analysis of state and local conditions related to COVID-19 vaccination use and future public health problems and pandemics.

OBJECTIVE:Recent work has shown that people with common epilepsies have characteristic patterns of cortical thinning, and that these changes may be progressive over time. Leveraging a large multicenter cross-sectional cohort, we investigated whether regional morphometric changes occur in a sequential manner, and whether these changes in people with mesial temporal lobe epilepsy and hippocampal sclerosis (MTLE-HS) correlate with clinical features. METHODS:We extracted regional measures of cortical thickness, surface area, and subcortical brain volumes from T1-weighted (T1W) magnetic resonance imaging (MRI) scans collected by the ENIGMA-Epilepsy consortium, comprising 804 people with MTLE-HS and 1625 healthy controls from 25 centers. Features with a moderate case-control effect size (Cohen d ≥ .5) were used to train an event-based model (EBM), which estimates a sequence of disease-specific biomarker changes from cross-sectional data and assigns a biomarker-based fine-grained disease stage to individual patients. We tested for associations between EBM disease stage and duration of epilepsy, age at onset, and antiseizure medicine (ASM) resistance. RESULTS:), and ASM resistance (area under the curve = .59, p = .043, Mann-Whitney U test). However, associations were driven by cases assigned to EBM Stage 0, which represents MTLE-HS with mild or nondetectable abnormality on T1W MRI. SIGNIFICANCE/CONCLUSIONS:From cross-sectional MRI, we reconstructed a disease progression model that highlights a sequence of MRI changes that aligns with previous longitudinal studies. This model could be used to stage MTLE-HS subjects in other cohorts and help establish connections between imaging-based progression staging and clinical features.

Epilepsy surgery should be considered in all patients with drug-resistant focal epilepsy. The diagnostic presurgical evaluation aims to delineate the epileptogenic zone and its relationship to eloquent brain regions. Genetic testing is not yet routine in presurgical evaluations, despite many monogenic causes of severe epilepsies, including some focal epilepsies. This review highlights genomic data that may inform decisions regarding epilepsy surgery candidacy and strategy. Focal epilepsies due to pathogenic variants in mechanistic target of rapamycin pathway genes are amenable to surgery if clinical, electroencephalography and imaging data are concordant. Epilepsy surgery outcomes are less favourable in patients with pathogenic variants in ion channel genes such as SCN1A. However, genomic data should not be used in isolation to contraindicate epilepsy surgery and should be considered alongside other diagnostic modalities. The additional role of somatic mosaicism in the pathogenesis of focal epilepsies may have implications for surgical planning and prognostication. Here, we advocate for including genomic data in the presurgical evaluation and multidisciplinary discussion for many epilepsy surgery candidates. We encourage neurologists to perform genetic testing in patients with focal non-lesional epilepsy, epilepsy in the setting of intellectual disability and epilepsy due to specific malformations of cortical development. The integration of genomics into the presurgical evaluation assists selection of patients for resective surgery and fosters a personalised medicine approach, where precision or targeted therapies are considered alongside surgical procedures.

This case report describes a novel endovascular method for treating chronically occluded internal carotid artery (COICA). The patient is a 55-year-old male with vascular risk factors who presented to an outside institution with right-sided weakness and dysarthria, was diagnosed as having a stroke, and discharged with medical management. The patient's symptoms failed to improve throughout the week prompting him to visit another outside institution, where computed tomography (CT) angiography showed bilateral occlusion of the ICAs at their origins extending intracranially. The patient was then transferred to our hospital, where head CT revealed bilateral acute infarcts predominantly in the left centrum ovale/corona radiata and left temporoparietal region. CT perfusion showed a large area of hypoperfusion in the entire left hemisphere as well as part of the right hemisphere (mismatch volume of 438-526 mL). The patient had significant neurological deficits despite sustained high perfusion pressure, so the following morning, the patient was taken for angiography showing complete occlusion of the left ICA with support mostly from the left external carotid artery (ECA)/ophthalmic collateralization. The microcatheter was able to be advanced to the level of the ophthalmic segment of the left ICA, so the decision was made to proceed with stenting from the left ophthalmic ICA to the cervical ICA. Seven consecutive coronary-carotid stents were placed to essentially reconstruct the left ICA. Post-stenting, the patient was treated with an Integrilin drip and transitioned to Aspirin and Brilinta the following morning. The patient's symptoms markedly improved after the procedure. CT perfusion, as well as diffusion magnetic resonance imaging (MRI), revealed recovery of the patient's penumbra and stability of the existing infarcts despite the delayed nature of revascularization respectively. This is a rarely reported study in literature describing the successful deployment of multiple stents in recreating the ICA from its extracranial to intracranial portion.

INTRODUCTION:Although dementia prevalence differs by race, it remains unclear whether cognition and neuropsychiatric symptom severity differ between Black and White individuals with dementia. METHODS:Using National Alzheimer's Coordinating Center (NACC) data, we evaluated dementia prevalence in non-Hispanic Black and White participants and compared their clinicodemographic characteristics. We examined race differences in cognition, neuropsychiatric symptoms, and functional abilities in participants with dementia using multivariable linear and logistic regression models. RESULTS:We included 5,700 Black and 31,225 White participants across 39 Alzheimer's Disease Research Centers. Of these, 1,528 (27%) Black and 11,267 (36%) White participants had dementia diagnoses. Despite having lower dementia prevalence, risk factors were more prevalent among Black participants. Black participants with dementia showed greater cognitive deficits, neuropsychiatric symptoms/severity, and functional dependence. DISCUSSION:Despite lower dementia prevalence, Black participants with dementia had more dementia risk factors, as well as greater cognitive impairment and neuropsychiatric symptom severity than White participants.

The demand for intraoperative monitoring (IOM) of lumbar spine surgeries has escalated to accommodate more challenging surgical approaches to prevent perioperative neurologic deficits. Identifying impending injury of individual lumbar roots can be done by assessing free-running EMG and by monitoring the integrity of sensory and motor fibers within the roots by eliciting somatosensory (SEP), and motor evoked potentials. However, the common nerves for eliciting lower limb SEP do not monitor the entire lumbar plexus, excluding fibers from L1 to L4 roots. We aimed to technically optimize the methodology for saphenous nerve SEP (Sap-SEP) proposed for monitoring upper lumbar roots in the operating room. In the first group, the saphenous nerve was consecutively stimulated in two different locations: proximal in the thigh and distal close to the tibia. In the second group, three different recording derivations (10-20 International system) to distal saphenous stimulation were tested. Distal stimulation yielded a higher Sap-SEP amplitude (mean ± SD) than proximal: 1.36 ± 0.9 µV versus 0.62 ± 0.6 µV, (p < 0.0001). Distal stimulation evoked either higher (73%) or similar (12%) Sap-SEP amplitude compared to proximal in most of the nerves. The recording derivation CPz-cCP showed the highest amplitude in 65% of the nerves, followed by CPz-Fz (24%). Distal stimulation for Sap-SEP has advantages over proximal stimulation, including simplicity, lack of movement and higher amplitude responses. The use of two derivations (CPz-cCP, CPz-Fz) optimizes Sap-SEP recording.

BACKGROUND AND PURPOSE/OBJECTIVE:Skull base tumors are commonly supplied by dural branches of the meningohypophyseal and inferolateral trunks. Embolization through these arteries is often avoided due to technical challenges and inherent risks; however, successful embolization can be a valuable surgical adjunct. We aimed to review the success and complications in our series of tumor embolizations through the meningohypophyseal and inferolateral trunks. MATERIALS AND METHODS/METHODS:We performed a retrospective review of patients with tumor treated with preoperative embolization at our institution between 2010 and 2020. We reviewed the following data: patients' demographics, tumor characteristics, endovascular embolization variables, and surgical results including estimated blood loss, the need for transfusion, and operative time. RESULTS:= 4) trunk. In this group of patients, on average, 79% of tumors were embolized. No mortality or morbidity from the embolization procedure was observed in this subgroup of patients. The average estimated blood loss in the operation was 395 mL (range, 200-750 mL). None of the patients required a transfusion, and the average operative time was 7.3 hours. CONCLUSIONS:Some skull base tumors necessitate embolization through ICA branches such as the meningohypophyseal and inferolateral trunks. Our series demonstrates that an effective and safe embolization may be performed through these routes.

Epilepsy is associated with genetic risk factors and cortico-subcortical network alterations, but associations between neurobiological mechanisms and macroscale connectomics remain unclear. This multisite ENIGMA-Epilepsy study examined whole-brain structural covariance networks in patients with epilepsy and related findings to postmortem epilepsy risk gene expression patterns. Brain network analysis included 578 adults with temporal lobe epilepsy (TLE), 288 adults with idiopathic generalized epilepsy (IGE), and 1328 healthy controls from 18 centres worldwide. Graph theoretical analysis of structural covariance networks revealed increased clustering and path length in orbitofrontal and temporal regions in TLE, suggesting a shift towards network regularization. Conversely, people with IGE showed decreased clustering and path length in fronto-temporo-parietal cortices, indicating a random network configuration. Syndrome-specific topological alterations reflected expression patterns of risk genes for hippocampal sclerosis in TLE and for generalized epilepsy in IGE. These imaging-transcriptomic signatures could potentially guide diagnosis or tailor therapeutic approaches to specific epilepsy syndromes.

Encephalopathy, a common condition among patients hospitalized with COVID-19, can be a challenge to manage and negatively affect prognosis. While encephalopathy may present clinically as delirium, subsyndromal delirium, or coma and may be a result of systemic causes such as hypoxia, COVID-19 has also been associated with more prolonged encephalopathy due to less common but nevertheless severe complications, such as inflammation of the brain parenchyma (with or without cerebrovascular involvement), demyelination, or seizures, which may be disproportionate to COVID-19 severity and require specific management. Given the large number of patients hospitalized with severe acute respiratory syndrome coronavirus-2 infection, even these relatively unlikely complications are increasingly recognized and are particularly important because they require specific management. Therefore, the aim of this review is to provide pragmatic guidance on the management of COVID-19 encephalopathy through consensus agreement of the Global COVID-19 Neuro Research Coalition. A systematic literature search of MEDLINE, medRxiv, and bioRxiv was conducted between January 1, 2020, and June 21, 2021, with additional review of references cited within the identified bibliographies. A modified Delphi approach was then undertaken to develop recommendations, along with a parallel approach to score the strength of both the recommendations and the supporting evidence. This review presents analysis of contemporaneous evidence for the definition, epidemiology, and pathophysiology of COVID-19 encephalopathy and practical guidance for clinical assessment, investigation, and both acute and long-term management.