On the Necessity of Ethical Guidelines for Novel Neurotechnologies.

Because novel neurotechnologies may alter human identity and society in profound ways, we advocate for the early integration of ethics into neurotechnology. We recommend developing and adopting a set of guidelines, like the Belmont Report on human subject research, as a framework for development and use of brain-related technologies.

Seven Strategies to Integrate Equity within Translational Research in Neurology.

The rapidly accelerating translation of biomedical advances is leading to revolutionary therapies that are often inaccessible to historically marginalized populations. We identified and synthesized recent guidelines and statements to propose 7 strategies to integrate equity within translational research in neurology: (1) learn history; (2) learn about upstream forces; (3) diversify and liberate; (4) change narratives and adopt best communication practices; (5) study social drivers of health and lived experiences; (6) leverage health technologies; and (7) build, sustain, and lead culturally humble teams. We propose that equity should be a major goal of translational research, equally important as safety and efficacy. ANN NEUROL 2024;95:432-441.

Survey of service needs to embed genome sequencing for motor neuron disease in neurology in the English National Health Service.

All people with motor neuron disease (pwMND) in England are eligible for genome sequencing (GS), with panel-based testing. With the advent of genetically targeted MND treatments, and increasing demand for GS, it is important that clinicians have the knowledge and skills to support pwMND in making informed decisions around GS. We undertook an online survey of clinical genomic knowledge and genetic counselling skills in English clinicians who see pwMND. There were 245 respondents to the survey (160 neurology clinicians and 85 genetic clinicians). Neurology clinicians reported multiple, overlapping barriers to offering pwMND GS. Lack of time to discuss GS in clinic and lack of training in genetics were reported. Neurology clinicians scored significantly less well on self-rated genomic knowledge and genetic counselling skills than genetic clinicians. The majority of neurology clinicians reported that they do not have adequate educational or patient information resources to support GS discussions. We identify low levels of genomic knowledge and skills in the neurology workforce. This may impede access to GS and precision medicine for pwMND.

PET-based response assessment criteria for diffuse gliomas (PET RANO 1.0): a report of the RANO group.

Response Assessment in Neuro-Oncology (RANO) response criteria have been established and were updated in 2023 for MRI-based response evaluation of diffuse gliomas in clinical trials. In addition, PET-based imaging with amino acid tracers is increasingly considered for disease monitoring in both clinical practice and clinical trials. So far, a standardised framework defining timepoints for baseline and follow-up investigations and response evaluation criteria for PET imaging of diffuse gliomas has not been established. Therefore, in this Policy Review, we propose a set of criteria for response assessment based on amino acid PET imaging in clinical trials enrolling participants with diffuse gliomas as defined in the 2021 WHO classification of tumours of the central nervous system. These proposed PET RANO criteria provide a conceptual framework that facilitates the structured implementation of PET imaging into clinical research and, ultimately, clinical routine. To this end, the PET RANO 1.0 criteria are intended to encourage specific investigations of amino acid PET imaging of gliomas.

The 2023 American Academy of Neurology, American Academy of Pediatrics, Child Neurology Society, and Society of Critical Care Medicine Pediatric and Adult Brain Death/Death by Neurologic Criteria Determination Consensus Guidelines: What the Critical Care Team Needs to Know.

Guidelines for brain death/death by neurologic criteria (BD/DNC) determination were revised to provide a consistent and updated approach to BD/DNC evaluation across all ages by the American Academy of Neurology, American Academy of Pediatrics, Child Neurology Society, and Society of Critical Care Medicine. This article is intended to complement the guidelines and highlight aspects relevant to the critical care community; the actual guidelines should be used to update hospital protocols and dictate clinical practice. Because BD/DNC evaluations are conducted in the ICU, it is essential for members of the critical care community to familiarize themselves with these guidelines. The fundamental concept of BD/DNC has not changed; BD/DNC is permanent loss of function of the brain as a whole, including the brain stem, resulting in coma, brainstem areflexia, and apnea in the setting of an adequate stimulus. The BD/DNC evaluation requires a sufficient observation period to ensure there is no chance of recovery, followed by exclusion of potentially confounding conditions like hypothermia, hypotension, severe metabolic disturbances, or medication effects. Specific guidance is provided for patients who were treated with therapeutic hypothermia or medical or surgical interventions to manage intracranial hypertension. The guidelines outline a structured and meticulous neurologic examination and detail the responses consistent with BD/DNC. A protocol is provided for how to safely perform apnea testing, including modifications needed for patients on extracorporeal membrane oxygenation. Controversial issues such as consent, BD/DNC evaluation in pregnancy, preservation of neuroendocrine function, and primary posterior fossa injuries are addressed. The ultimate goal is to ensure a consistent and accurate approach to BD/DNC evaluation in patients of all ages, fostering public trust in the medical community's ability to determine death. By adhering to these guidelines, critical care clinicians can confidently navigate the challenging aspects of BD/DNC determination.

Demographics, Practice Analysis, and Geographic Distribution of Neuro-Ophthalmologists in the United States in 2023.

PURPOSE: To assess the demographic characteristics and geographic distribution of neuro-ophthalmologists practicing in the United States. DESIGN: A cross-sectional study. PARTICIPANTS: Neuro-ophthalmologists across the United States. METHODS: In this cross-sectional study, public databases from the American Academy of Ophthalmology, North American Neuro-ophthalmology Society, American Neurological Association, and American Academy of Neurology were used to identify neuro-ophthalmologists in the United States as of April 2023. Providers' office locations were geocoded using ArcGIS pro, version 2.9 (Esri). Data on age, sex, and residency and fellowship training were collected. Analysis was performed using SPSS 28.0 (IBM Corp.). MAIN OUTCOME MEASURES: Neuro-ophthalmologists' demographics, and information about their medical education, postgraduate education, residency training, fellowship training, years in practice, practice environment, and geographic distribution of neuro-ophthalmologists across the United States. RESULTS: A total of 635 neuro-ophthalmologists (436 male, 68.7%) were identified. The majority (599, 94.3%) graduated from an allopathic medical school. Most of the 85 physicians who held a secondary graduate degree had a PhD (54, 63.5%). Although approximately three-quarters (429, 67.6%) completed their residency in ophthalmology, 159 (25%) had residency positions in neurology and 47 (7.4%) had residency positions in both. Approximately one-third (191, 30.0%) were trained in more than 1 fellowship, including oculoplastics (78, 12.3%) or pediatric ophthalmology (53, 8.3%). The average post-fellowship years of experience was 23.7±13.7 years, with 134 (21.1%) in their early career (< 10 years), 120 (18.9%) in their mid-careers (10-19 years), and 381 (60.0%) in their late careers (> 20 years). Male neuro-ophthalmologists had 10.5±1.1 more years of experience than female neuro-ophthalmologists (P < 0.001). Three states (Maine, South Dakota, Wyoming) and 2897 counties (93.2%) had no neuro-ophthalmologists. Counties without a neuro-ophthalmologist had lower median income (P < 0.001), lower access to a vehicle (P = 0.024), and lower rates of health insurance (P = 0.012). CONCLUSIONS: Practicing neuro-ophthalmologists are mostly male and often are trained in more than 1 subspecialty. More than half of the practicing neuro-ophthalmologists are in their late careers, which may further exacerbate the existing geographic and socioeconomic disparities in access to neuro-ophthalmology. FINANCIAL DISCLOSURE(S): The author(s) have no proprietary or commercial interest in any materials discussed in this article.

Recognizing and Responding to the Needs of Future Child and Adult Neurology Care Through the Evolution of Residency Training.

Recent insights into the frequency of occurrence and the genetic and mechanistic basis of nervous system disease have demonstrated that neurologic disorders occur as a spectrum across all ages. To meet future needs of patients with neurologic disease of all ages and prepare for increasing implementaton of precision therapies, greater integration of child and adult neurology residency training is needed. ANN NEUROL 2023;94:1005-1007.

Methodological considerations for observational studies of treatment effectiveness in neurology: a clinician's guide.

Data from cohorts, registries, randomised trials, electronic medical records and administrative claims databases have increasingly been used to inform the use of therapies for neurological diseases. While novel sophisticated methods are enabling us to use existing data to guide treatment decisions, the complexity of statistical methodology is making appraisal of clinical evidence increasingly demanding. In this narrative review, we provide a brief overview of the most commonly used methods for evaluation of treatment effectiveness in neurology. This primer discusses complementarity of randomised and non-randomised study designs, sources of observational data, different forms of bias and the appropriate mitigation strategies, statistical significance, Bayesian approaches and provides an overview of multivariable regression models, propensity score-based models, causal inference, mediation analysis and Mendelian randomisation.

Genetic etiology of progressive pediatric neurological disorders.

BACKGROUND: The aim of the study was to characterize molecular diagnoses in patients with childhood-onset progressive neurological disorders of suspected genetic etiology. METHODS: We studied 48 probands (age range from newborn to 17 years old) with progressive neurological disorders of unknown etiology from the largest pediatric neurology clinic in Finland. Phenotypes included encephalopathy (54%), neuromuscular disorders (33%), movement disorders (11%), and one patient (2%) with hemiplegic migraine. All patients underwent whole-exome sequencing and disease-causing genes were analyzed. RESULTS: We found 20 (42%) of the patients to have variants in genes previously associated with disease. Of these, 12 were previously reported disease-causing variants, whereas eight patients had a novel variant on a disease-causing gene: ATP7A, CHD2, PURA, PYCR2, SLC1A4, SPAST, TRIT1, and UPF3B. Genetics also enabled us to define atypical clinical presentations of Rett syndrome (MECP2) and Menkes disease (ATP7A). Except for one deletion, all findings were single-nucleotide variants (missense 72%, truncating 22%, splice-site 6%). Nearly half of the variants were de novo. CONCLUSIONS: The most common cause of childhood encephalopathies are de novo variants. Whole-exome sequencing, even singleton, proved to be an efficient tool to gain specific diagnoses and in finding de novo variants in a clinically heterogeneous group of childhood encephalopathies. IMPACT: Whole-exome sequencing is useful in heterogeneous pediatric neurology cohorts. Our article provides further evidence for and novel variants in several genes. De novo variants are an important cause of childhood encephalopathies.

General neurology: Current challenges and future implications.

BACKGROUND AND PURPOSE: In the coming decades, the world will face an increasing burden of neurological disorders (ND) and an urgent need to promote brain health. These challenges contrast with an insufficient neurological workforce in most countries, as well as decreasing numbers of general neurologists and neurologists attracted to work in general neurology (GN). This white paper aims to review the current situation of GN and reflect on its future. METHODS: The European Academy of Neurology (EAN) task force (TF) met nine times between November 2021 and June 2023. During the 2023 EAN annual meeting, attendees were asked to answer five questions concerning the future of GN. The document was sent for suggestions and eventually approval to the board and the presidents of the 47 national societies of the EAN. RESULTS: The TF first identified four relevant current and future challenges related to GN: (i) definition, (ii) practice, (iii) education, and (iv) research. The TF then identified seven initiatives to further develop GN at both the academic and community level. Finally, the TF formulated 16 recommendations to promote GN in the future. CONCLUSIONS: GN will remain essential in the coming decades to provide rapid, accessible, and comprehensive management of patients with ND that is affordable and cost-effective. There is also a need for research, education, and other initiatives aiming to facilitate improved working conditions, recognition, and prestige for those pursuing a career in GN.

The 2022 European postgraduate (residency) programme in neurology in a historical and international perspective.

BACKGROUND AND PURPOSE: Neurology residency programmes, which were first established at the beginning of the 20th century, have become mandatory all over Europe in the last 40-50 years. The first European Training Requirements in Neurology (ETRN) were published in 2005 and first updated in 2016. This paper reports the most recent revisions of the ETRN. METHODS: Members of the EAN board performed an in depth revision of the ETNR 2016-version, which was reviewed by members of the European Board and Section of Neurology of the UEMS, the Education and Scientific Panels, the Resident and Research Fellow Section and the Board of the EAN, as well as the presidents of the 47 European National Societies. RESULTS: The new (2022) ETRN suggest a 5-year training subdivided in three phases: a first phase (2 years) of general neurology training, a second phase (2 years) of training in neurophysiology/neurological subspecialties and a third phase (1 year) to expand clinical training (e.g., in other neurodisciplines) or for research (path for clinical neuroscientist). The necessary theoretical and clinical competences as well as learning objectives in diagnostic tests have been updated, are newly organized in four levels and include 19 neurological subspecialties. Finally, the new ETRN require, in addition to a programme director, a team of clinician-educators who regularly review the resident's progress. The 2022 update of the ETRN reflects emerging requirements for the practice of neurology and contributes to the international standardization of training necessary for the increasing needs of residents and specialists across Europe.

European Academy of Neurology (EAN) guideline on the management of amyotrophic lateral sclerosis in collaboration with European Reference Network for Neuromuscular Diseases (ERN EURO-NMD).

BACKGROUND: This update of the guideline on the management of amyotrophic lateral sclerosis (ALS) was commissioned by the European Academy of Neurology (EAN) and prepared in collaboration with the European Reference Network for Neuromuscular Diseases (ERN EURO-NMD) and the support of the European Network for the Cure ALS (ENCALS) and the European Organization for Professionals and Patients with ALS (EUpALS). METHODS: Grading of Recommendations Assessment, Development, and Evaluation (GRADE) methodology was used to assess the effectiveness of interventions for ALS. Two systematic reviewers from Cochrane Response supported the guideline panel. The working group identified a total of 26 research questions, performed systematic reviews, assessed the quality of the available evidence, and made specific recommendations. Expert consensus statements were provided where insufficient evidence was available. RESULTS: A guideline mapping effort revealed only one other ALS guideline that used GRADE methodology (a National Institute for Health and Care Excellence [NICE] guideline). The available evidence was scarce for many research questions. Of the 26 research questions evaluated, the NICE recommendations could be adapted for 8 questions. Other recommendations required updates of existing systematic reviews or de novo reviews. Recommendations were made on currently available disease-modifying treatments, multidisciplinary care, nutritional and respiratory support, communication aids, psychological support, treatments for common ALS symptoms (e.g., muscle cramps, spasticity, pseudobulbar affect, thick mucus, sialorrhea, pain), and end-of-life management. CONCLUSIONS: This update of the guideline using GRADE methodology provides a framework for the management of ALS. The treatment landscape is changing rapidly, and further updates will be prepared when additional evidence becomes available.

The COVID-19 pandemic and neurology: A survey on previous and continued restrictions for clinical practice, curricular training, and health economics.

BACKGROUND AND PURPOSE: The COVID-19 pandemic has significantly impacted health systems worldwide. Here, we assessed the pandemic's impact on clinical service, curricular training, and financial burden from a neurological viewpoint during the enforced lockdown periods and the assumed recovery by 2023. METHODS: An online 18-item survey was conducted by the European Academy of Neurology (EAN) NeuroCOVID-19 Task Force among the EAN community. The survey was online between February and March 2023. Questions related to general, demographic, clinical, work, education, and economic aspects. RESULTS: We collected 430 responses from 79 countries. Most health care professionals were aged 35-44 years, with >15 years of work experience. The key findings of their observations were as follows. (i) Clinical services were cut back in all neurological subspecialties during the most restrictive COVID-19 lockdown period. The most affected neurological subspecialties were services for patients with dementia, and neuromuscular and movement disorders. The levels of reduction and the pace of recovery were distinct for acute emergencies and in- and outpatient care. Recovery was slow for sleep medicine, autonomic nervous system disorders, neurorehabilitation, and dementia care. (ii) Student and residency rotations and grand rounds were reorganized, and congresses were converted into a virtual format. Conferences are partly maintained in a hybrid format. (iii) Affordability of neurological care and medication shortage are emerging issues. CONCLUSIONS: Recovery of neurological services up to spring 2023 has been incomplete following substantial disruption of neurological care, medical education, and health economics in the wake of the COVID-19 pandemic. The continued limitations for the delivery of neurological care threaten brain health and call for action on a global scale.

Embracing the future-is artificial intelligence already better? A comparative study of artificial intelligence performance in diagnostic accuracy and decision-making.

BACKGROUND AND PURPOSE: The integration of artificial intelligence (AI) in healthcare has the potential to revolutionize patient care and clinical decision-making. This study aimed to explore the reliability of large language models in neurology by comparing the performance of an AI chatbot with neurologists in diagnostic accuracy and decision-making. METHODS: A cross-sectional observational study was conducted. A pool of clinical cases from the American Academy of Neurology's Question of the Day application was used as the basis for the study. The AI chatbot used was ChatGPT, based on GPT-3.5. The results were then compared to neurology peers who also answered the questions-a mean of 1500 neurologists/neurology residents. RESULTS: The study included 188 questions across 22 different categories. The AI chatbot demonstrated a mean success rate of 71.3% in providing correct answers, with varying levels of proficiency across different neurology categories. Compared to neurology peers, the AI chatbot performed at a similar level, with a mean success rate of 69.2% amongst peers. Additionally, the AI chatbot achieved a correct diagnosis in 85.0% of cases and it provided an adequate justification for its correct responses in 96.1%. CONCLUSIONS: The study highlights the potential of AI, particularly large language models, in assisting with clinical reasoning and decision-making in neurology and emphasizes the importance of AI as a complementary tool to human expertise. Future advancements and refinements are needed to enhance the AI chatbot's performance and broaden its application across various medical specialties.

A strategic neurological research agenda for Europe: Towards clinically relevant and patient-centred neurological research priorities.

BACKGROUND AND PURPOSE: Neurological disorders constitute a significant portion of the global disease burden, affecting >30% of the world's population. This prevalence poses a substantial threat to global health in the foreseeable future. A lack of awareness regarding this high burden of neurological diseases has led to their underrecognition, underappreciation, and insufficient funding. Establishing a strategic and comprehensive research agenda for brain-related studies is a crucial step towards aligning research objectives among all pertinent stakeholders and fostering greater societal awareness. METHODS: A scoping literature review was undertaken by a working group from the European Academy of Neurology (EAN) to identify any existing research agendas relevant to neurology. Additionally, a specialized survey was conducted among all EAN scientific panels, including neurologists and patients, inquiring about their perspectives on the current research priorities and gaps in neurology. RESULTS: The review revealed the absence of a unified, overarching brain research agenda. Existing research agendas predominantly focus on specialized topics within neurology, resulting in an imbalance in the number of agendas across subspecialties. The survey indicated a prioritization of neurological disorders and research gaps. CONCLUSIONS: Building upon the findings from the review and survey, key components for a strategic and comprehensive neurological research agenda in Europe were delineated. This research agenda serves as a valuable prioritization tool for neuroscientific researchers, as well as for clinicians, donors, and funding agencies in the field of neurology. It offers essential guidance for creating a roadmap for research and clinical advancement, ultimately leading to heightened awareness and reduced burden of neurological disorders.

Neurology Training and Medical Education in Resource-Limited Settings: Building and Growing the First Neurology Residency Program in East Africa.

The burden of neurological disease disproportionately affects low- and middle-income countries, where the lowest number of neurologists are located. Building local training opportunities in resource-limited settings is a foundational step to enhancing the neurological workforce and improving access to neurological care in these regions. In this article, we describe the development and growth of the first neurology residency program in East Africa, which was established in 2006 at Zewditu Memorial Hospital and the Tikur Anbessa Specialized Hospital, Addis Ababa University, Ethiopia. We highlight the impact of the program on clinical care, research, collaborations between neurologists across Ethiopia, and ways to build educational opportunities and mentorship while faced with limited resources. The main challenges in starting the residency program included lack of faculty with neurological expertise, lack of a precedent for subspecialty training in our setting, as well as limited resources and space. The formation of sustainable international collaborations with clinicians at established institutions in high-income countries and neurological societies has been a major source of support in developing the initial infrastructure, curriculum and educational content, knowledge assessments, and mentored research projects. Local partnerships with related medical specialties, including internal medicine, critical care, neurological surgery, and psychiatry, were also instrumental in creating training opportunities. As the program continues to evolve, many challenges remain, including limited diagnostics, lack of access to advanced treatment modalities, lack of fellowship training opportunities in various neurological subspecialties, and insufficient training and experience in scientific writing. Despite these challenges, the residency program has persevered and its creation resulted in many positive changes: since its inception in 2006, we graduated 80 neurologists and the number of practicing neurologists in Ethiopia has increased from 5 to 78, our institution has evolved into a national referral center for neurology, graduates have published 61 articles in the past 3 years and contributed to international neurology research, and alumni of the program have grown the Association of Ethiopian Neurologists. Future directions include development of fellowship opportunities, creation of international rotations, and implementation of teleneurology to further strengthen neurological care across Ethiopia.

Neurological Practice in the Time of War: Perspectives and Experiences from Ukraine.

The full-scale Russian invasion of Ukraine has significantly impacted the country's healthcare system. Insufficient infrastructure, destruction of medical facilities, and barriers to prevention and treatment efforts hinder the provision of timely, high-quality care to our patients. We aim to describe the impact of the war on neurological care across Ukraine. In this article, leading national experts in stroke, epilepsy, multiple sclerosis, and movement disorders describe their personal experience and efforts in organizing and providing care since the war started in February 2022. A neurologist who cared for patients in Mariupol recounts the first weeks of the war when the city was under constant attacks. An international stroke expert describes the role of Task Force for Ukraine, a European Stroke Organization initiative to support the Ukrainian stroke community. We discuss a series of critical challenges facing Ukraine's neurologists, patients, and healthcare delivery system, including shortages of personnel and medical supplies, disrupted logistics, and lack of funding. In addition, we highlight various interventions and strategies aimed at counteracting these challenges, including international support, collaborations within Ukraine, and initiatives enhancing the resilience of the Ukrainian neurology community. As the war is ongoing, this article emphasizes the pressing need for continuous support and investment in the Ukrainian healthcare system to preserve guaranteed access to high-quality healthcare for the Ukrainian people during the war and in its aftermath. Insights from the essays can inform the development and implementation of effective strategies and interventions tailored to such extraordinary circumstances.

Racial and Ethnic Disparities in Neurological Care in the United States.

The burden of neurological disease is increasing globally. In the United States, this burden is disproportionally greater for Black and Latino communities who have limited access to neurological care. Health services researchers have attempted to identify racial and ethnic disparities in neurological care and possible solutions. This article reviews the most current literature on racial and ethnic disparities in commonly encountered neurological conditions, including Stroke, Alzheimer's Disease, Multiple Sclerosis, Epilepsy, Parkinson's Disease, and Migraine. Disparities exist in disease incidence, diagnosis, access to care, treatment, outcomes, and representation in epidemiologic studies and clinical trials. Many of the disparities observed in neurological care in the United States are a consequence of longstanding racist and discriminatory policies and legislation that increase risk factors for the development of neurological disease or lead to disparities in accessing quality neurological care. Therefore, additional efforts on the legislative, community health, and healthcare system levels are necessary to prevent the onset of neurological disease and achieve equity in neurological care.

Neurological Care within the Indian Health Service.

American Indians and Alaska Natives (AI/AN) are the Indigenous peoples of the United States. According to the U.S. Census Bureau, approximately 9.7 million people self-identified as AI/AN (alone or in combination with other races), representing 2.9% of the total U.S. population. These people represent diverse groups of discrete Tribes, each with their language, culture, and geographic home. As part of the conquest and settlement of North America, some Indigenous peoples signed treaties with the U.S. government, surrendering their lands in return for various government commitments, including health care. The Indian Health Service (IHS) was born out of these agreements. The IHS is an agency in the U.S. Department of Health and Human Services under the U.S. Public Health Service. The IHS provides a comprehensive health service delivery system for approximately 2.7 million AI/AN who belong to 574 federally recognized Tribes/nations in 37 states. The aim of this paper is to make recommendations regarding the initiation of sustainable neurology care in marginalized or underserved populations by reviewing 40 years of neurology care provision within the IHS. We will discuss (1) the IHS, (2) neurological care provided within the IHS, including midlevel provider extension of neurology care and traditional medical care, and (3) select neurological diagnoses within AI/AN populations. Marginalized populations, including those in the United States that are rural, remote, or low socioeconomic status, lack access to specialty neurology care. This includes many AI/AN. The IHS has developed novel solutions to promote specialty care, including neurology. Notably, initial IHS investments in full-time neurology providers have led to more robust neurology care, often receiving attention from university programs. This suggests that an initial investment in stable on-site full-time neurology services provides a path to potential sustainable care for marginalized populations.