Neuromuscular Emergencies.

OBJECTIVE: This article aims to familiarize the reader with the clinical approach, diagnostic considerations, and treatment strategies for patients presenting with abrupt-onset or acutely worsening weakness due to neuromuscular disorders. LATEST DEVELOPMENTS: Neuromuscular weakness is often the result of an inflammatory process. In recent years, there has been growing recognition of pathologic antibodies that cause neuromuscular injury. This has allowed clinicians to make a more accurate diagnosis. Additionally, neuromuscular junction disorders and myopathies are increasingly identified as the adverse effects of novel anticancer therapies, namely immune checkpoint inhibitors. More data are being incorporated into frameworks for neuroprognostication after neuromuscular emergencies, especially for commonly encountered disorders such as Guillain-Barré syndrome. ESSENTIAL POINTS: Care of patients with neuromuscular emergencies requires prompt attention to respiratory status. Once supportive measures are in place to protect the airway and facilitate effective ventilation, diagnostic considerations should hinge on appropriate neurologic localization. Aggressive immunosuppression is often required for immune-mediated neuromuscular disorders, and clinicians must be thoughtful in selecting a strategy that best aligns with each patient's risk factors and comorbidities.

Using Neurology Trainees as Standardized Patients in a Neurological Emergency Simulation Curriculum for Medical Students.

Purpose: Simulation manikins have limited ability to mimic neurological exam findings, which has historically constrained their use in neurology education. We developed a cased-based simulation curriculum in which neurology trainees acted as standardized patients (SPs) and portrayed the neurologic exam for medical students. Materials/Methods: We ran monthly simulations of two cases (acute stroke and seizure) with resident/fellow SPs. Pre-/post-session surveys assessed students' self-rated confidence in neurological clinical skills (gathering a history, performing an exam, presenting a case) and knowledge domains. Questions about students' attitudes about neurology were adapted from a validated assessment tool. Paired t-tests were performed for quantitative items. Qualitative thematic analysis identified key themes. Results: Sixty-one students participated. Post-session, students reported significantly higher self-confidence in all neurological clinical skills and knowledge domains (p < 0.002). Greater than ninety-five percent agreed the session met the learning objectives; 95% recommended it to others. Resident/fellow SPs were cited as the most effective educational component. Students appreciated evaluating acute emergencies and reported an increased interest in neurology careers. Conclusions: A case-based simulation curriculum with neurology trainees portraying the SP increased students' self-reported knowledge, skills, and confidence in managing neurological emergencies. Our intervention may improve medical student neurology education and increase interest in the field. Future research should evaluate clinical skills objectively. Supplementary Information: The online version contains supplementary material available at 10.1007/s40670-024-02016-w.

A Modified Delphi Consensus Approach to Define Entrustable Professional Activities for Neurocritical Care Advanced Practice Providers.

OBJECTIVES: To define consensus entrustable professional activities (EPAs) for neurocritical care (NCC) advanced practice providers (APPs), establish validity evidence for the EPAs, and evaluate factors that inform entrustment expectations of NCC APP supervisors. DESIGN: A three-round modified Delphi consensus process followed by application of the EQual rubric and assessment of generalizability by clinicians not affiliated with academic medical centers. SETTING: Electronic surveys. SUBJECTS: NCC APPs ( n = 18) and physicians ( n = 12) in the United States with experience in education scholarship or APP program leadership. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: The steering committee generated an initial list of 61 possible EPAs. The panel proposed 30 additional EPAs. A total of 47 unique nested EPAs were retained by consensus opinion. The steering committee defined six core EPAs addressing medical knowledge, procedural competencies, and communication proficiency which encompassed the nested EPAs. All core EPAs were retained and subsequently met the previously described cut score for quality and structure using the EQual rubric. Most clinicians who were not affiliated with academic medical centers rated each of the six core EPAs as very important or mandatory. Entrustment expectations did not vary by prespecified groups. CONCLUSIONS: Expert consensus was used to create EPAs for NCC APPs that reached a predefined quality standard and were important to most clinicians in different practice settings. We did not identify variables that significantly predicted entrustment expectations. These EPAs may aid in curricular design for an EPA-based assessment of new NCC APPs and may inform the development of EPAs for APPs in other critical care subspecialties.

Patent Foramen Ovale Management for Secondary Stroke Prevention: State-of-the-Art Appraisal of Current Evidence.

Patent foramen ovale (PFO) is frequently identified in young patients with ischemic stroke. Randomized controlled trials provide robust evidence supporting PFO closure in selected patients with cryptogenic ischemic stroke; however, several questions remain unanswered. This report summarizes current knowledge on the epidemiology of PFO-associated stroke, the role of PFO as a cause of stroke, and anatomic high-risk features. We also comment on breakthrough developments in patient selection algorithms for PFO closure in relation to the PFO-associated stroke causal likelihood risk stratification system. We further highlight areas for future research in PFO-associated stroke including the efficacy and safety of PFO closure in the elderly population, incidence, and long-term consequences of atrial fibrillation post-PFO closure, generalizability of the results of clinical trials in the real world, and the need for assessing the effect of neurocardiology teams on adherence to international recommendations. Other important knowledge gaps such as sex, race/ethnicity, and regional disparities in access to diagnostic technologies, PFO closure devices, and clinical outcomes in the real world are also discussed as priority research topics.

Be in the Digital Room Where it Happens, Part II: Social Media for Neurology Educators.

Social media has changed the way we communicate and interact. Unsurprisingly, it has also changed how we teach and learn. Younger generations of learners have transitioned from traditional educational sources to digital ones. Medical educators need to adapt to trends in medical education and develop fluency in the digital methods used by medical learners today. This is part two of a two-part series on social media and digital education in neurology. This article provides an overview of how social media can be used as a teaching tool in medical education and provides an overview in which it is grounded. We offer practical strategies on how social media can promote lifelong learning, educator development, educator support, and foster educator identity with accompanying neurology-specific examples. We also review considerations for incorporating social media into teaching and learning practices and future directions for integrating these tools in neurology education.

Engaging teaching strategies in neurology: the flipped classroom and simulation training.

Just as neurology continues to expand its diagnostic and therapeutic modalities, so too does neurology education continue to expand in its pedagogical modalities. In this article, we describe two educational techniques-the flipped classroom and simulation-that we have incorporated in our teaching of neurology to students and doctors in training, with some practical tips for their successful implementation.

Characterization of human disease phenotypes associated with mutations in TREX1, RNASEH2A, RNASEH2B, RNASEH2C, SAMHD1, ADAR, and IFIH1.

Aicardi-Goutières syndrome is an inflammatory disease occurring due to mutations in any of TREX1, RNASEH2A, RNASEH2B, RNASEH2C, SAMHD1, ADAR or IFIH1. We report on 374 patients from 299 families with mutations in these seven genes. Most patients conformed to one of two fairly stereotyped clinical profiles; either exhibiting an in utero disease-onset (74 patients; 22.8% of all patients where data were available), or a post-natal presentation, usually within the first year of life (223 patients; 68.6%), characterized by a sub-acute encephalopathy and a loss of previously acquired skills. Other clinically distinct phenotypes were also observed; particularly, bilateral striatal necrosis (13 patients; 3.6%) and non-syndromic spastic paraparesis (12 patients; 3.4%). We recorded 69 deaths (19.3% of patients with follow-up data). Of 285 patients for whom data were available, 210 (73.7%) were profoundly disabled, with no useful motor, speech and intellectual function. Chilblains, glaucoma, hypothyroidism, cardiomyopathy, intracerebral vasculitis, peripheral neuropathy, bowel inflammation and systemic lupus erythematosus were seen frequently enough to be confirmed as real associations with the Aicardi-Goutieres syndrome phenotype. We observed a robust relationship between mutations in all seven genes with increased type I interferon activity in cerebrospinal fluid and serum, and the increased expression of interferon-stimulated gene transcripts in peripheral blood. We recorded a positive correlation between the level of cerebrospinal fluid interferon activity assayed within one year of disease presentation and the degree of subsequent disability. Interferon-stimulated gene transcripts remained high in most patients, indicating an ongoing disease process. On the basis of substantial morbidity and mortality, our data highlight the urgent need to define coherent treatment strategies for the phenotypes associated with mutations in the Aicardi-Goutières syndrome-related genes. Our findings also make it clear that a window of therapeutic opportunity exists relevant to the majority of affected patients and indicate that the assessment of type I interferon activity might serve as a useful biomarker in future clinical trials.

Temporal trends in early clinical manifestations of perinatal HIV infection in a population-based cohort.

CONTEXT: The effect of early antiretroviral therapy (ART) on the early progression of perinatal human immunodeficiency virus (HIV) infection is not well defined. OBJECTIVE: To examine early disease progression and survival in a population-based cohort with perinatal HIV infection in relation to year of birth and use of ART. DESIGN, SETTING, AND PATIENTS: Retrospective study of temporal trends in early progression of perinatal HIV infection among 205 HIV-infected children in Northern California born between January 1, 1988, and December 31, 2001, and followed up through age 3 years. MAIN OUTCOME MEASURES: Prevalence of and age at progression to a first US Centers for Disease Control and Prevention category C diagnosis relative to year of birth, type of ART, and age at initiation of therapy. RESULTS: Of 205 children, 134 (65%) received ART and/or Pneumocystis jiroveci pneumonia prophylaxis. By age 3 years, 81 (40%) progressed to a category C diagnosis, 41 (51%) of whom died. Untreated children were significantly more likely to progress to a category C diagnosis (62% [44/71] untreated vs 28% [37/134] treated children, P<.001); none of 23 infants who received triple ART progressed to category C. However, even without triple ART, very early mono/dual ART (by age 2 months vs 3-4 months) was associated with delayed and decreased progression to category C (P = .02). Of 33 children born between January 1, 1996, and December 31, 2001, only 7 (21%) progressed to category C (P = .02 compared with 1988-1995), 6 of 7 of whom received no therapy. More recent year of birth and more advanced therapy were associated with improved survival. CONCLUSIONS: This population-based cohort demonstrated decreased early HIV progression and improved survival at age 3 years, associated with more advanced therapy. Although limited by small sample size, the findings suggest that very early treatment, even without triple ART, was associated with improved outcome.