Fatal Case of Chronic Jamestown Canyon Virus Encephalitis Diagnosed by Metagenomic Sequencing in Patient Receiving Rituximab.

A 56-year-old man receiving rituximab who had months of neurologic symptoms was found to have Jamestown Canyon virus in cerebrospinal fluid by clinical metagenomic sequencing. The patient died, and postmortem examination revealed extensive neuropathologic abnormalities. Deep sequencing enabled detailed characterization of viral genomes from the cerebrospinal fluid, cerebellum, and cerebral cortex.

Seizures in patients with cancer.

Seizures are common in patients with cancer and either result from brain lesions, paraneoplastic syndromes, and complications of cancer treatment or are provoked by systemic illness (metabolic derangements, infections). Evaluation should include a tailored history, neurologic examination, laboratory studies, neuroimaging, and electroencephalogram. In unprovoked seizures, antiepileptic drug (AED) treatment is required, and a nonenzyme-inducing AED is preferred. Treatment of the underlying cancer with surgery, chemotherapy, and radiation therapy also can help reduce seizures. Benzodiazepines are useful in the treatment of both provoked seizures and breakthrough epileptic seizures and as first-line treatment for status epilepticus. Counseling for safety is an important component in the care of a patient with cancer who has seizures. Good seizure management can be challenging but significantly improves the quality of life during all phases of care, including end-of-life care.

Teaching the Neurological Examination in a Rapidly Evolving Clinical Climate.

The neurological examination remains the essence of neurology. It allows symptoms to be assessed, diagnoses to be made, and dynamic functions to be followed. Skill in the neurological examination has faced increasing challenges from the encroachment of diagnostic imaging, but has maintained its clinical utility. It has also encountered the battle for the precious time within a medical curriculum. This review considers how the neurological examination can best be taught into the future. It does so by considering factors related to the examination, the learner, the teacher, and the modern clinical environment.

Interprofessional simulation to improve safety in the epilepsy monitoring unit.

OBJECTIVE: Patient safety is critical for epilepsy monitoring units (EMUs). Effective training is important for educating all personnel, including residents and nurses who frequently cover these units. We performed a needs assessment and developed a simulation-based team training curriculum employing actual EMU sentinel events to train neurology resident-nurse interprofessional teams to maximize effective responses to high-acuity events. METHODS: A mixed-methods design was used. This included the development of a safe-practice checklist to assess team response to acute events in the EMU using expert review with consensus (a modified Delphi process). All nineteen incoming first-year neurology residents and 2 nurses completed a questionnaire assessing baseline knowledge and attitudes regarding seizure management prior to and following a team training program employing simulation and postscenario debriefing. Four resident-nurse teams were recorded while participating in two simulated scenarios. Employing retrospective video review, four trained raters used the newly developed safe-practice checklist to assess team performance. We calculated the interobserver reliability of the checklist for consistency among the raters. We attempted to ascertain whether the training led to improvement in performance in the actual EMU by comparing 10 videos of resident-nurse team responses to seizures 4-8months into the academic year preceding the curricular training to 10 that included those who received the training within 4-8months of the captured video. RESULTS: Knowledge in seizure management was significantly improved following the program, but confidence in seizure management was not. Interrater agreement was moderate to high for consistency of raters for the majority of individual checklist items. We were unable to demonstrate that the training led to sustainable improvement in performance in the actual EMU by the method we used. CONCLUSIONS: A simulated team training curriculum using a safe-practice checklist to improve the management of acute events in an EMU may be an effective method of training neurology residents. However, translating the results into sustainable benefits and confidence in management in the EMU requires further study.

What's in Your Nomogram? Personalized Prognostication of Verbal Memory Decline after Temporal Lobe Resection in Adults With Epilepsy.

OBJECTIVE: This study aims to develop and externally validate models to predict the probability of postoperative verbal memory decline in adults following temporal lobe resection (TLR) for epilepsy using easily accessible preoperative clinical predictors. METHODS: Multivariable models were developed to predict delayed verbal memory outcome on 3 commonly used measures: Rey Auditory Verbal Learning Test (RAVLT), and Logical Memory (LM), and Verbal Paired Associates (VPA) subtests from Wechsler Memory Scale-Third Edition. Using Harrell's step-down procedure for variable selection, models were developed in 359 adults who underwent TLR at Cleveland Clinic and validated in 290 adults at 1 of 5 epilepsy surgery centers in the United States or Canada. RESULTS: Twenty-nine percent of the development cohort and 26% of the validation cohort demonstrated significant decline on at least 1 verbal memory measure. Initial models had good-to-excellent predictive accuracy (calibration (c) statistic range = .77-.80) in identifying patients with memory decline; however, models slightly underestimated decline in the validation cohort. Model coefficients were updated using data from both cohorts to improve stability. The model for RAVLT included surgery side, baseline memory score, and hippocampal resection. The models for LM and VPA included surgery side, baseline score, and education. Updated model performance was good to excellent (RAVLT c = .81, LM c = .76, VPA c = .78). Model calibration was very good, indicating no systematic over- or under-estimation of risk. CONCLUSIONS: Nomograms are provided in 2 easy-to-use formats to assist clinicians in estimating the probability of verbal memory decline in adults considering TLR for treatment of epilepsy.

A randomized trial for the treatment of refractory status epilepticus.

BACKGROUND: Refractory status epilepticus (RSE) has a mortality of 16-39%; coma induction is advocated for its management, but no comparative study has been performed. We aimed to assess the effectiveness (RSE control, adverse events) of the first course of propofol versus barbiturates in the treatment of RSE. METHODS: In this randomized, single blind, multi-center trial studying adults with RSE not due to cerebral anoxia, medications were titrated toward EEG burst-suppression for 36-48 h and then progressively weaned. The primary endpoint was the proportion of patients with RSE controlled after a first course of study medication; secondary endpoints included tolerability measures. RESULTS: The trial was terminated after 3 years, with only 24 patients recruited of the 150 needed; 14 subjects received propofol, 9 barbiturates. The primary endpoint was reached in 43% in the propofol versus 22% in the barbiturates arm (P = 0.40). Mortality (43 vs. 34%; P = 1.00) and return to baseline clinical conditions at 3 months (36 vs. 44%; P = 1.00) were similar. While infections and arterial hypotension did not differ between groups, barbiturate use was associated with a significantly longer mechanical ventilation (P = 0.03). A non-fatal propofol infusion syndrome was detected in one patient, while one subject died of bowel ischemia after barbiturates. DISCUSSION: Although undersampled, this trial shows significantly longer mechanical ventilation with barbiturates and the occurrence of severe treatment-related complications in both arms. We describe practical issues necessary for the success of future studies needed to improve the current unsatisfactory state of evidence.

Epilepsy: A Clinical Overview.

The diagnosis and treatment of seizures and epilepsy is a common task of the physician. Approximately 1 in 10 people will have a seizure during their lifetime. Epilepsy is the tendency to have unprovoked seizures. Epilepsy is the fourth most common neurological disorder and affects 1 in 26 people in the United States and 65 million people worldwide. Evaluation of a patient presenting with a seizure involves excluding an underlying neurologic or medical condition, classifying the seizure type and determining if the patient has epilepsy. Proper treatment requires accurate diagnosis of the epilepsy type and syndrome and use of a medication that is effective and without adverse effects. Most patients can achieve complete seizure control with medication, but if medication is unsuccessful, surgical treatment can be an option. Special situations in the care of people with epilepsy include status epilepticus, women with epilepsy, the older adult, and safety issues.

Randomized Study of Bedside vs Hallway Rounding: Neurology Rounding Study.

Over the last century, attending rounds have shifted away from the bedside. Despite evidence for greater patient satisfaction rates and improved nursing perception of teamwork with bedside presentations, residents and attending physicians are apprehensive of the bedside approach. There is lack of data to guide rounding practices within neurology, and therefore, optimal rounding methods remain unclear. The objective of this study was to compare bedside rounding with hallway rounding on an academic neurology inpatient service and assess efficiency, trainee education, and satisfaction among patients and staff. We conducted a single-center prospective randomized study of bedside vs hallway rounding on new inpatient neurology admissions over 1-week blocks. The bedside team presented patients at the bedside, whereas the hallway team presented patients outside of the patient's room. We evaluated the 2 approaches with time-motion analysis, which investigated the rounding style's effect on composition and timing of rounds (primary outcome) and surveys of patients, nurses, residents, and attending physicians on both teams (secondary outcomes). The mean rounding time per newly admitted patient in the bedside group (n = 38 patients) and hallway group (n = 41 patients) was 23 minutes and 23.2 minutes, respectively (p = 0.93). The bedside group spent on average 56.4% of patient rounding time in the patient's room, whereas the hallway group spent 39.5% of rounding time in the patient's room (p = 0.036). Residents perceived hallway rounding to be more efficient and associated it with a superior educational experience and more effective data review. Nurses had improved perception of their participation in bedside rounds. Although patients' views of bedside and hallway rounds were similar, patients who had experienced bedside rounds preferred it. In conclusion, bedside rounding was perceived less favorably by most residents but was as efficient as hallway rounding. Although bedside rounding limited the use of technology for data review, it promoted nursing participation and resulted in more time spent with the patient. CLINICAL TRIAL REGISTRATION NUMBER: Registered retrospectively per the editors' suggestion (NCT04754828).

Identifying and Analyzing Systems Failures: An Interactive, Experiential Learning Approach to Quality Improvement for Clerkship-Level Medical Students.

Introduction: Medical students are positioned to observe, document, and explore opportunities to improve patient safety and quality in their institutions. Medical schools are introducing quality improvement (QI) knowledge and skills in the preclinical classrooms, yet few provide opportunities to apply these tools in the clinical setting. Methods: Clerkship students participated in two 1-hour sessions, organized in groups of 12-15 students, led by faculty with QI expertise. The sessions in the module introduced core concepts in QI and patient safety, while drawing on students' own clinical experiences. Students identified a system failure they encountered in their own clinical setting/practice and analyzed contributing factors using the 5 Whys Tool. We evaluated the efficacy of the two-session module with a pre- and postsurvey of students' self-reported change in knowledge, skills, and attitudes. Surveys also assessed students' satisfaction with module content and format. Faculty perspectives were solicited by email. Results: In April-May 2019, 59 students at a large US medical school participated. Of students, 73% and 53% completed pre- and postsurveys, respectively. All students submitted a report of an identified systems failure and their analysis of contributing factors. Students' self-rated knowledge and skills increased significantly. Students preferred active engagement compared to passive learning. Students and faculty identified areas for future module improvement. Discussion: The educational program was well received and increased students' knowledge and confidence in core concepts of QI and safety. The module addressed the requirement for graduating students to identify safety incidents and contribute to a culture of QI.

Pediatric and adult neurologist perspectives on the challenges of sustaining a transfer clinic.

We present a novel epilepsy fellow-driven transfer clinic model and discuss the challenges experienced in finding sustainability; this is timely as many pioneering transition clinics are dissolving across North America. The goal of this clinic was to improve patient care and satisfaction, as measured by a post-visit telephone survey. Unfortunately, our transfer clinic model proved unsustainable due to several factors, broadly categorized as (1) cultural-societal differences between the pediatric and adult health care environments, (2) staffing issues, (3) lack of an established standardized process for transfer of care, and (4) financial and administrative barriers. We suggest potential solutions to these challenges, but the fate of transition and transfer of care clinics may ultimately depend on implementation of practice, policy, and/or financial guidelines.

Brain Death Determination: An Interprofessional Simulation to Determine Brain Death and Communicate with Families Focused on Neurology Residents.

Introduction: Significant variation exists in determining brain death despite an expectation of competence for all neurology residents. In addition, family discussions regarding brain death are challenging and may influence organ donation. Methods: We developed two simulations of increasing complexity for PGY 2 and PGY 3 neurology residents. High-fidelity mannequins were used to simulate patients; standardized actors portrayed family members. In the first simulation, residents determined brain death and shared this information with a grieving family. In the second simulation, residents determined brain death in a more complicated scenario, requiring ancillary testing and accurate result interpretation. Following the determination, residents met with a challenging family. The residents worked with an interdisciplinary team and responded to the family's emotions, used active listening skills, and supported the family through next steps. Results: Twelve residents completed the simulations. Prior to the simulation, three (25%) residents felt comfortable discussing a brain death diagnosis; following the simulation, eight (67%) residents felt comfortable/very comfortable discussing brain death. Prior to the simulation, eight (67%) residents stated they knew prerequisites for performing a brain death examination and seven (58%) agreed they knew indications for ancillary testing; these numbers increased to 100% following the simulation. The number of residents who felt comfortable performing the brain death exam increased from five (42%) to 10 (83%). Discussion: This simulation of determining brain death and leading difficult family meetings was well-received by neurology residents. Further work should focus on the effects of simulation-based education on practice variation and organ donation consent rates.

Training in Neurology: Rapid implementation of cross-institutional neurology resident education in the time of COVID-19.

In-person resident didactics are traditionally limited to the faculty within a single institution. Tele-education efforts have been implemented in neurology to various degrees historically, but the coronavirus disease 2019 (COVID-19) pandemic has necessitated a broad and immediate overhaul in neurology didactic training. To respond to the immediate need for resident didactics, we created a rapid onset, volunteer tele-education didactic series publicized on online forums to the American Academy of Neurology A.B. Baker Section via Synapse and the Women Neurologists Group via Facebook. We describe how, with just 1 week of lead time, we created an ongoing neurology lecture series featuring faculty from across the country lecturing on a diverse range of neurology topics. The series is ongoing and draws upwards of 120 residents per lecture. Tele-education offers unique benefits to enhance the education of all neurology trainees everywhere.

Frequency and patterns of MRI abnormalities due to status epilepticus.

BACKGROUND: MRI changes due to status epilepticus (SE) often suggest a combination of cytotoxic and vasogenic edema, but it is unclear why only certain patients have MRI changes. OBJECTIVES: To determine the frequency of MRI changes due to SE and the associated patient characteristics. METHODS: We reviewed records for demographics, medical history, and MRI changes attributable to seizures of all patients admitted to Brigham and Women's Hospital or Massachusetts General Hospital for SE from 1/1999 to 7/2003 and who had MRI during admission. RESULTS: Ten (11.6%) of the eighty-six patients identified had MRI abnormalities likely due to seizures. Four, two with pre-existing epilepsy and two with extratemporal structural lesions, had focally increased signal on T2 and diffusion-weighted imaging (DWI) in the hippocampus ipsilateral to the seizure focus. One, with elevated levels of clozapine, had increased signal on T2 weighted images and variably restricted diffusion in the splenium. Five had gyral distribution of restricted diffusion and increased signal on T2 weighted images; they had complex medical comorbidities and possible hypoperfusion or hypoxia associated with SE. CONCLUSIONS: Among patients with SE who had MRI changes, those with previous epilepsy or extratemporal structural lesions showed increased diffusion in the hippocampus and may have selective hippocampal vulnerability to seizure-induced hyperexcitability. Patients with hyperintense signal in the cortical gray matter had episodes of possible hypoperfusion or hypoxia.

Diagnosis in Neurologic Disease.

The diagnosis of neurologic disease is relevant to the non-neurologist because neurologic symptoms are a common reason patients present to their health care provider and most of these patients are never referred to a neurologist. The diagnosis of a neurologic disease is a rewarding endeavor because it requires intellectual rigor, skill, and is of paramount importance to patient care. A tailored history and examination lead to localization and differential diagnosis. Diagnostic testing often involves neuroimaging and serum testing and also may involve lumbar puncture, electroencephalogram, nerve conduction studies, and electromyography. In the modern era, all neurologic diagnoses lead to treatments.

Smartphone EEG and remote online interpretation for children with epilepsy in the Republic of Guinea: Quality, characteristics, and practice implications.

PURPOSE: Children with epilepsy in low-income countries often go undiagnosed and untreated. We examine a portable, low-cost smartphone-based EEG technology in a heterogeneous pediatric epilepsy cohort in the West African Republic of Guinea. METHODS: Children with epilepsy were recruited at the Ignace Deen Hospital in Conakry, 2017. Participants underwent sequential EEG recordings with an app-based EEG, the Smartphone Brain Scanner-2 (SBS2) and a standard Xltek EEG. Raw EEG data were transmitted via Bluetooth™ connection to an Android™ tablet and uploaded for remote EEG specialist review and reporting via a new, secure web-based reading platform, crowdEEG. The results were compared to same-visit Xltek 10-20 EEG recordings for identification of epileptiform and non-epileptiform abnormalities. RESULTS: 97 children meeting the International League Against Epilepsy's definition of epilepsy (49 male; mean age 10.3 years, 29 untreated with an antiepileptic drug; 0 with a prior EEG) were enrolled. Epileptiform discharges were detected on 21 (25.3%) SBS2 and 31 (37.3%) standard EEG recordings. The SBS2 had a sensitivity of 51.6% (95%CI 32.4%, 70.8%) and a specificity of 90.4% (95%CI 81.4%, 94.4%) for all types of epileptiform discharges, with positive and negative predictive values of 76.2% and 75.8% respectively. For generalized discharges, the SBS2 had a sensitivity of 43.5% with a specificity of 96.2%. CONCLUSIONS: The SBS2 has a moderate sensitivity and high specificity for the detection of epileptiform abnormalities in children with epilepsy in this low-income setting. Use of the SBS2+crowdEEG platform permits specialist input for patients with previously poor access to clinical neurophysiology expertise.

Status Epilepticus Severity Score (STESS): a tool to orient early treatment strategy.

BACKGROUND: Status epilepticus (SE) treatment ranges from small benzodiazepine doses to coma induction. For some SE subgroups, it is unclear how the risk of an aggressive therapeutic approach balances with outcome improvement. We recently developed a prognostic score (Status Epilepticus Severity Score, STESS), relying on four outcome predictors (age, history of seizures, seizure type and extent of consciousness impairment), determined before treatment institution. Our aim was to assess whether the score might have a role in the treatment strategy choice. METHODS: This cohort study involved adult patients in three centers. For each patient, the STESS was calculated before primary outcome assessment: survival vs. death at discharge. Its ability to predict survival was estimated through the negative predictive value for mortality (NPV). Stratified odds ratios (OR) for mortality were calculated considering coma induction as exposure; strata were defined by the STESS level. RESULTS: In the observed 154 patients, the STESS had an excellent negative predictive value (0.97). A favorable STESS was highly related to survival (P < 0.001), and to return to baseline clinical condition in survivors (P < 0.001). The combined Mantel-Haenszel OR for mortality in patients stratified after coma induction and their STESS was 1.5 (95 % CI: 0.59-3.83). CONCLUSION: The STESS reliably identifies SE patients who will survive. Early aggressive treatment could not be routinely warranted in patients with a favorable STESS, who will almost certainly survive their SE episode. A randomized trial using this score would be needed to confirm this hypothesis.