Effects of explainable artificial intelligence in neurology decision support.

OBJECTIVE: Artificial intelligence (AI)-based decision support systems (DSS) are utilized in medicine but underlying decision-making processes are usually unknown. Explainable AI (xAI) techniques provide insight into DSS, but little is known on how to design xAI for clinicians. Here we investigate the impact of various xAI techniques on a clinician's interaction with an AI-based DSS in decision-making tasks as compared to a general population. METHODS: We conducted a randomized, blinded study in which members of the Child Neurology Society and American Academy of Neurology were compared to a general population. Participants received recommendations from a DSS via a random assignment of an xAI intervention (decision tree, crowd sourced agreement, case-based reasoning, probability scores, counterfactual reasoning, feature importance, templated language, and no explanations). Primary outcomes included test performance and perceived explainability, trust, and social competence of the DSS. Secondary outcomes included compliance, understandability, and agreement per question. RESULTS: We had 81 neurology participants with 284 in the general population. Decision trees were perceived as the more explainable by the medical versus general population (P < 0.01) and as more explainable than probability scores within the medical population (P < 0.001). Increasing neurology experience and perceived explainability degraded performance (P = 0.0214). Performance was not predicted by xAI method but by perceived explainability. INTERPRETATION: xAI methods have different impacts on a medical versus general population; thus, xAI is not uniformly beneficial, and there is no one-size-fits-all approach. Further user-centered xAI research targeting clinicians and to develop personalized DSS for clinicians is needed.

Converting LEA to Pelli-Robson contrast sensitivity score in neuro-ophthalmology patients.

PURPOSE: The Pelli-Robson and LEA contrast sensitivity charts are commonly used in clinical settings to measure contrast sensitivity. Although the Pelli-Robson chart is considered the gold standard, it is limited by its bulky size. The LEA chart, on the contrary, offers a more practical and portable option that is still reliable. This has led to questions about whether we can predict Pelli-Robson scores based on LEA scores. This study developed a conversion method to help transition from the LEA chart to the Pelli-Robson chart and validate the conversion score. METHODS: In this retrospective study, we analyzed the relationship between LEA and the Pelli-Robson contrast sensitivity test. Our study examined a total of 120 eyes. We developed a conversion table through the equipercentile equating method. Subsequently, we assessed the reliability and accuracy of this algorithm for converting LEA results into Pelli-Robson contrast sensitivity scores. RESULTS: The study used a conversion table to convert LEA scores to Pelli-Robson scores. The conversion table achieved a reliability of 0.91 based on intraclass correlation, and the algorithm had an accuracy of 81.6% within a 1-point difference from the raw score. CONCLUSIONS: This study reported a reliable and comparable conversion algorithm for transforming LEA scores into converted estimated Pelli-Robson scores, thereby improving the usefulness of existing data in both clinical and research contexts.

Artificial intelligence performance in clinical neurology queries: the ChatGPT model.

INTRODUCTION: The use of artificial intelligence technology is progressively expanding and advancing in the health and biomedical literature. Since its launch, ChatGPT has rapidly gained popularity and become one of the fastest-growing artificial intelligence applications in history. This study evaluated the accuracy and comprehensiveness of ChatGPT-generated responses to medical queries in clinical neurology. METHODS: We directed 216 questions from different subspecialties to ChatGPT. The questions were classified into three categories: multiple-choice, descriptive, and binary (yes/no answers). Each question in all categories was subjectively rated as easy, medium, or hard according to its difficulty level. Questions that also tested for intuitive clinical thinking and reasoning ability were evaluated in a separate category. RESULTS: ChatGPT correctly answered 141 questions (65.3%). No significant difference was detected in the accuracy and comprehensiveness scale scores or correct answer rates in comparisons made according to the question style or difficulty level. However, a comparative analysis assessing question characteristics revealed significantly lower accuracy and comprehensiveness scale scores and correct answer rates for questions based on interpretations that required critical thinking (p = 0.007, 0.007, and 0.001, respectively). CONCLUSION: ChatGPT had a moderate overall performance in clinical neurology and demonstrated inadequate performance in answering questions that required interpretation and critical thinking. It also displayed limited performance in specific subspecialties. It is essential to acknowledge the limitations of artificial intelligence and diligently verify medical information produced by such models using reliable sources.

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.

Artificial intelligence in neurology: opportunities, challenges, and policy implications.

Neurological conditions are the leading cause of disability and mortality combined, demanding innovative, scalable, and sustainable solutions. Brain health has become a global priority with adoption of the World Health Organization's Intersectoral Global Action Plan in 2022. Simultaneously, rapid advancements in artificial intelligence (AI) are revolutionizing neurological research and practice. This scoping review of 66 original articles explores the value of AI in neurology and brain health, systematizing the landscape for emergent clinical opportunities and future trends across the care trajectory: prevention, risk stratification, early detection, diagnosis, management, and rehabilitation. AI's potential to advance personalized precision neurology and global brain health directives hinges on resolving core challenges across four pillars-models, data, feasibility/equity, and regulation/innovation-through concerted pursuit of targeted recommendations. Paramount actions include swift, ethical, equity-focused integration of novel technologies into clinical workflows, mitigating data-related issues, counteracting digital inequity gaps, and establishing robust governance frameworks balancing safety and innovation.

A Comparison of Patients' and Neurologists' Assessments of their Teleneurology Encounter: A Cross-Sectional Analysis.

Background and Objectives: To better understand patients' and neurologists' assessments of their experiences regarding effectiveness of teleneurology encounters. Methods: Following an audio-video telehealth visit, neurologists asked patients to participate in a survey-based research study about the encounter, and then, the neurologists also recorded their own evaluations. Data were analyzed using standard quantitative and qualitative techniques for dichotomous and ordered-category survey responses in this cross-sectional analysis. Results: The study included unique encounters between 187 patients and 11 general neurologists. The mean patient age was 49 ± 17.5 years. Two thirds of the patients (66.8%, 125/187) were female. One third (33.2%; 62) were patients new to the NYU Langone Health neurology practices. The most common patient chief complaints were headache (69/187, 36.9%), focal and generalized numbness or tingling (21, 11.2%), memory difficulty (15, 8%), spine-related symptoms (12, 6.4%), and vertigo (11, 5.9%). Most patients (94.7%, 177/187) reported that the teleneurology encounter satisfied their needs. Patients and their neurologists agreed that the experience was effective in 91% (162/178) of encounters, regardless of whether the visit was for a new or established patient visit. Discussion: More than 90% of new and established patients and their neurologists agreed that teleneurology encounters were effective despite some limitations of the examination, the occasional need for patient assistance, and technical difficulties. Our results provide further evidence to justify and to expand the clinical use of teleneurology.

Pediatric Neuro-oncology.

OBJECTIVE: This article reviews the most common pediatric brain tumors, neurocutaneous syndromes, treatment-related neurotoxicities, and the long-term outcomes of survivors. LATEST DEVELOPMENTS: In the era of molecular diagnostics, the classification, management, and prognostication of pediatric brain tumors and neurocutaneous syndromes has been refined, resulting in advancements in patient management. Molecular diagnostics have been incorporated into the most recent World Health Organization 2021 classification. This knowledge has allowed for novel therapeutic approaches targeting the biology of these tumors with the intent to improve overall survival, decrease treatment-related morbidity, and improve quality of life. Advances in management have led to better survival, but mortality remains high and significant morbidity persists. Current clinical trials focus on tumor biology targeted therapy, deescalation of therapy, and multimodal intensified approaches with targeted therapy in more high-risk tumors. ESSENTIAL POINTS: Molecular diagnostics for pediatric brain tumors and neurocutaneous syndromes have led to novel therapeutic approaches targeting the biology of these tumors with the goals of improving overall survival and decreasing treatment-related morbidity. Further understanding will lead to continued refinement and improvement of tumor classification, management, and prognostication.

Neurologists' Experiences and Attitudes Towards Teleneurology - Has Covid-19 Pandemic Made a Difference?

Teleneurology is an adaption of telemedicine used in neurological practices. Due to the widespread availability of the Internet and the development of information and communication technology (ICT), the use of teleneurology has increased in healthcare systems. This study aimed to determine how the Covid-19 pandemic has affected neurologists' attitudes towards teleneurology as well as their experiences of remote health care before and after the pandemic. The study was conducted as a web-based questionnaire sent to all Finnish neurologists. Two identical surveys were sent via e-mail from the National Neurology Society. The first survey was conducted in spring 2021 and second in spring 2023. The results show that the pandemic moderately increased the use of teleneurology, which enhanced neurologists' technical skills. Neurologists estimated that the use of teleneurology will continue to increase in the future.

Global synergistic actions to improve brain health for human development.

The global burden of neurological disorders is substantial and increasing, especially in low-resource settings. The current increased global interest in brain health and its impact on population wellbeing and economic growth, highlighted in the World Health Organization's new Intersectoral Global Action Plan on Epilepsy and other Neurological Disorders 2022-2031, presents an opportunity to rethink the delivery of neurological services. In this Perspective, we highlight the global burden of neurological disorders and propose pragmatic solutions to enhance neurological health, with an emphasis on building global synergies and fostering a 'neurological revolution' across four key pillars - surveillance, prevention, acute care and rehabilitation - termed the neurological quadrangle. Innovative strategies for achieving this transformation include the recognition and promotion of holistic, spiritual and planetary health. These strategies can be deployed through co-design and co-implementation to create equitable and inclusive access to services for the promotion, protection and recovery of neurological health in all human populations across the life course.

Providers' Perceptions of Neurology Care Delivered Through Telemedicine Technology.

Background: The COVID-19 pandemic and subsequent acceleration of telemedicine usage allowed many neurologists to trial telemedicine for neurological care. The purpose of this study is to explore neurology providers' experiences with delivering telemedicine care during the COVID-19 pandemic. Methods: Semistructured video interviews were conducted with 27 neurology providers who practice at a single, urban academic center. Interviews were transcribed and analyzed for content and themes. Results: Five major themes were identified: virtual examination subspecialty differences, tips and tricks for the virtual examination, improved infrastructure needs, future technologies that could support the virtual examination, and preferences for the postpandemic telemedicine protocol. Subspecialists who described their visits as more focused on behavioral examination and obtaining patient history reported fewer limitations with delivering neurological care through telemedicine platforms. Conclusions: The implementation of a telemedicine system should reflect the needs of each neurology subspecialty. Funding is needed to improve logistical infrastructure for health providers' telemedicine visits, such as technical and administrative assistance, as well as creation and testing of technologies to support physical examination in the virtual environment.

Academic Neurology and the COVID-19 Pandemic: Resilience, Hope, and Solutions.

The SARS-CoV-2 (COVID-19) viral pandemic dramatically affected human health, health care delivery, health care workers, and health care research worldwide. The field of academic neurology was no exception. In this 2022 Presidential Plenary, we discuss the challenges faced by neurologists and neuroscientists professionally and personally. We review the threats posed by the pandemic to neuroscience research activities, materials, productivity, and funding. We then discuss the impact of the pandemic on clinical trials for neurologic diseases. Restrictions to patient enrolment due to limited in-person access to laboratory testing, imaging, and study visits led to delay in both clinical trial enrolment and study completion but also to innovative new means to engage clinical trial participants remotely and to strategies to critically appraise the frequency and design of trial-related patient evaluations. Clinical care was also challenged by initial pandemic prioritization of urgent visit and inpatient care and the rapid pivot to telehealth for most other neurology care encounters. Front-line neurology care teams faced their fears of infection, with the first few months of the pandemic being characterized by uncertainty, inconsistent national health care strategies, limited personal protective equipment, and an alarming rate of human illness and death caused by COVID-19. The personal and societal toll of the pandemic is incalculable. Across research and clinical neurology providers, women and particularly those with young families juggled the impossible balance of career and family care as schools closed and children required home-based education. Shining through this dark time are lessons that should shape a brighter future for our field. We are resilient, and the advances in neuroscience and neurology care continue to advance improved neurologic outcomes. The National Institutes of Health devised multiple support strategies for researchers to help bridge the pandemic. Telehealth, clinical trial designs that are more participant-centric with remote monitoring, and flexible work schedules are strategies to rebalance overworked lives and improve our engagement with our patients. As we re-emerge, we have the chance to reframe our field.

Neurologists' Evaluations of Experience and Effectiveness of Teleneurology Encounters.

Background and Objectives: To better understand neurologists' assessments of the experiences and effectiveness of teleneurology encounters. Methods: After completing an audio-video telehealth visit with verbally consenting patients, neurologists recorded their evaluations of the encounter. Data were analyzed using standard quantitative and qualitative techniques. Results: The study included unique encounters between 187 patients and 11 neurologists. The mean patient age was 49 ± 17.5 years. Two thirds of patients (66.8%, 125/187) were female. One third of patients (33.2%; 62) were new patients. The most common patient complaints were headache (69/187, 36.9%), focal and generalized numbness or tingling (21, 11.2%), memory difficulty (15, 8%), spine-related symptoms (12, 6.4%), and vertigo (11, 5.9%). Neurologists reported that they completed a virtual examination that provided enough information for medical decision-making in 94.9% of encounters (169/178, 9 missing responses). Fourteen of 25 examination elements important for medical decision-making could be performed sufficiently during virtual encounters. Examination assistance was needed for 16.4% (30/183) of patients, who were, on average, 17.3 years older than those who did not require assistance (62.9 years vs. 45.6 years, p = 0.0002). In 19.1% (34/178) of encounters, neurologists learned clinically relevant information from seeing patients in their homes. Neurologists' assessments of the effectiveness of encounters were not related to the presence (97.2%, 35/36 effective) or absence (95%, 134/141 effective) of technical difficulties (p = 0.5729) in 177 encounters (10 missing responses). Discussion: Neurologists reported that nearly 95% of teleneurology encounters were effective despite limitations of the virtual examination, occasional need for patient assistance, and technical difficulties.

Diseño de un protocolo con validez ecológica para la detección temprana de riesgo neurológico en población pediátrica de la primera infancia / Design of a protocol with ecological validity for the early detection of neurological risk in the pediatric population of early childhood

Introducción. El desarrollo del ser humano está determinado por factores biológicos, ambientales y contextuales que determinan la adquisición de habilidades neurológicas, y que bajo situaciones patológicas aumentan el riesgo de alteraciones en el neurodesarrollo desde etapas tempranas. Objetivo. Diseñar un protocolo con validez ecológica para la detección temprana de riesgo neurológico en la primera infancia por parte de equipos interdisciplinarios de rehabilitación. Métodos. Se realizó un estudio mixto, retrospectivo, transversal y descriptivo con un diseño exploratorio secuencial (DESPLOX). En la fase cualitativa se conformaron dos grupos focales: uno con padres (n=8) y otro con profesionales (n=6), de los cuales se obtuvieron las categorías del protocolo. En la fase cuantitativa se revisaron sistemáticamente artículos científicos (n=30) para la construcción de las orientaciones de acción. Finalmente, el protocolo se validó mediante un panel de expertos empleando el coeficiente de V de Aiken. Resultados. En la fase cualitativa emergieron cuatro categorías: 1) detección temprana, 2) contextos del desarrollo, 3) plan de intervención y 4) calidad y humanización en la atención. En la fase cuantitativa se seleccionaron las orientaciones de acción ubicadas en los Q2 y Q3. Posteriormente se evidenció una validación del protocolo igual a X ̅ =0,98. Conclusiones. Un protocolo de neurorrehabilitación válido ecológicamente se caracteriza por reconocer las percepciones, vivencias y experiencias de familiares y profesionales; recoger evidencia científica confiable; aportar orientaciones y recomendaciones sistémicas para la atención de niñas y niños, y contener criterios de validación de contenido.

Introduction. The human being's development is determined by biological, environmental, and contextual factors that determine the acquisition of neurological skills and that, under pathological situations, increase the risk of alterations in neurodevelopment from early stages. Objective. Design a protocol with ecological validity for the early detection of neurological risk in early childhood by interdisciplinary rehabilitation teams. Methods. A mixed, retrospective, cross-sectional, and descriptive study was performed with Exploratory Sequential Designs (DEXPLOS). In the qualitative phase, two focus groups were formed: one with parents (n=8) and another with professionals (n=6), from which the protocol categories were obtained. In the quantitative phase, scientific articles (n=30) were systematically reviewed to construct the action guidelines. Finally, the protocol was validated by a panel of experts using Aiken's V coefficient. Results. In the qualitative phase, four categories emerged: 1) early detection, 2) development contexts, 3) intervention plan, and 4) quality and humanization of care. In the quantitative phase, the action orientations located in Q2 and Q2 were selected. Subsequently, a validation of the protocol equal to X ̅=0.98 was evidenced. Conclusions. An ecologically valid neuro-rehabilitation protocol is characterized by recognizing the perceptions, expe-riences, and experiences of relatives and professionals; collecting reliable scientific evidence; providing systemic guide-lines and recommendations for the care of girls and boys, and containing content validation criteria.

A reassessment of Jackson's checklist and identification of two Down syndrome sub-phenotypes.

Down syndrome (DS) is characterised by several clinical features including intellectual disability (ID) and craniofacial dysmorphisms. In 1976, Jackson and coll. identified a checklist of signs for clinical diagnosis of DS; the utility of these checklists in improving the accuracy of clinical diagnosis has been recently reaffirmed, but they have rarely been revised. The purpose of this work is to reassess the characteristic phenotypic signs and their frequencies in 233 DS subjects, following Jackson's checklist. 63.77% of the subjects showed more than 12 signs while none showed less than 5, confirming the effectiveness of Jackson's checklist for the clinical diagnosis of DS. An association between three phenotypic signs emerged, allowing us to distinguish two sub-phenotypes: Brachycephaly, short and broad Hands, short Neck (BHN), which is more frequent, and "non-BHN". The strong association of these signs might be interpreted in the context of the growth defects observed in DS children suggesting decreased cell proliferation. Lastly, cognitive assessments were investigated for 114 subjects. The lack of association between the presence of a physical sign or the number of signs present in a subject and cognitive skills disproves the stereotype that physical characteristics are predictive of degree of ID.

[Adaptation of headache units in Andalusia to the COVID-19 pandemic. Analysis of the Andalusian Neurology Society's Headache Study Group]. / Adaptación de las unidades de cefalea de Andalucía a la pandemia por COVID-19. Análisis del Grupo de Estudio de Cefaleas de la Sociedad Andaluza de Neurología.

INTRODUCTION: The SARS-CoV-2 pandemic has given rise to a major change in healthcare and brought teleconsultation to the forefront. In neurology, headaches are the most frequent reason for visits. AIM: To assess the impact of the COVID-19 pandemic on the structure of headache units in Andalusia and the adaptations made to healthcare that are potentially useful innovations that can continue to be developed when the pandemic is over. MATERIALS AND METHODS: Cross-sectional observational study using an online survey of neurologists responsible for headache units and specialised consultations in Andalusia. RESULTS: During the state of alarm, all respondents used teleconsultation. The vast majority (92.8%) maintained some face-to-face activity, mostly for invasive techniques and new patients, using individual protection measures and as a way to avoid crowds. Half of them (50%) maintained botulinum toxin administrations at the scheduled times and 78.6% continued to prescribe monoclonal antibodies against calcitonin gene-related peptide. Altogether 78.5% are generally satisfied with the use of teleconsultation and 57.1% think it could be quite useful in the future. The main advantages reported were avoiding the need for the patient to travel and time savings; the disadvantages were the absence of physical examinations and difficulties in communicating. The most frequently expressed need for improvement was the use of video-calls. CONCLUSIONS: Some of the changes adopted during this time could continue to be useful in the future and, in the case of headaches, teleconsultation could be used as an option for following up patients who have already been diagnosed and do not require any invasive techniques.

TITLE: Adaptación de las unidades de cefalea de Andalucía a la pandemia por COVID-19. Análisis del Grupo de Estudio de Cefaleas de la Sociedad Andaluza de Neurología.Introducción. La pandemia por SARS-CoV-2 ha supuesto un gran cambio en la atención sanitaria y ha dado protagonismo a la teleconsulta. En neurología, las cefaleas constituyen el motivo más frecuente de consulta. Objetivo. Evaluar el impacto de la pandemia por COVID-19 en la estructura de las unidades de cefaleas de Andalucía y las adaptaciones asistenciales potencialmente útiles tras ella. Materiales y métodos. Estudio observacional transversal mediante encuesta en línea a los neurólogos responsables de las unidades y consultas monográficas de cefaleas de Andalucía. Resultados. Durante el estado de alarma, todos los encuestados usaron teleconsulta. El 92,8% mantuvo alguna actividad presencial, fundamentalmente para técnicas invasivas y pacientes nuevos, utilizando medidas de protección individual y para evitar aglomeraciones. El 50% mantuvo las administraciones de toxina botulínica en los tiempos adecuados y el 78,6% siguió prescribiendo anticuerpos monoclonales frente al péptido relacionado con el gen de la calcitonina. El 78,5% se encuentra globalmente satisfecho con el uso de la teleconsulta y el 57,1% considera que podría ser bastante útil de cara al futuro. Las principales ventajas expresadas fueron evitar el desplazamiento de los pacientes y el ahorro de tiempo; los inconvenientes, la ausencia de exploración física y la dificultad de comunicación. La necesidad de mejora más expresada fue el uso de videollamada. Conclusiones. Algunos de los cambios adoptados en este tiempo podrían seguir siendo útiles en el futuro y, en el caso de las cefaleas, la teleconsulta se podría emplear como opción para el seguimiento de pacientes ya diagnosticados y que no requieran técnicas invasivas.

Low online search interest in teleneurology before and during COVID-19 pandemic: an infodemiological study.

BACKGROUND: The conduct of patient consults greatly changed during the time of the COVID-19 pandemic. There was a decrease in face-to-face patient consults and enhanced utilization of virtual consults. Infodemiological studies, using Google Trends, focus on internet search trends that may reflect public interest and awareness in diseases and as a proxy of public health risk perception. OBJECTIVES: We aimed to investigate the online behavior of internet users on teleneurology, telehealth, and telemedicine during the time of the pandemic, as reflected in search volume indices (SVI) projected in Google Trends. METHODS: We used the data from Google Trends to quantify the interest of internet users in teleneurology, telehealth, and telemedicine. These keywords were entered in Google Trends as search terms. Data included were searches conducted from 2016 to 2020 to depict the transition into the pandemic. RESULTS: The SVI of teleneurology was unchanged during the 5-year period search relative to the SVIs of telehealth and telemedicine. Contrary to the noted worldwide increase in the SVI of telehealth and telemedicine during the year of pandemic, teleneurology's SVI remained stable despite the increase of its utilization. Focusing on teleneurology, the highest SVI was observed in 2018. CONCLUSION: There was an increase in the SVI of telehealth and telemedicine, possibly implying an increase in the general awareness of these virtual methods of health care, as catalyzed by the pandemic. However, the stable SVI of teleneurology may signify that the public awareness regarding it remained unchanged despite the increase in application in clinics and hospitals.

Patient perspectives in pediatric neurology: a critical shift in the paradigm of outcome measurement.

This review explores children's self-reported outcome measurements in pediatric neurology. We examine the following questions: (1) What is meant by patient-reported health, functioning, and quality of life outcomes? (2) How can patients express whether the interventions they receive do more good than harm? (3) Why and how should pediatric neurology patients help determine the outcomes of interest? (4) What tools and recommendations are available to evaluate the outcomes of interest? Applying patients' perspectives across the processes of evaluation of medical interventions has become an important expectation. These developments, consistent with current healthcare goals, coincide with the evolution of pediatric neurology into a sophisticated diagnostic-interventional field that aims to prolong survival, decrease impairments and symptoms, and improve patients' well-being - the recognized essential endpoints of interest in all medicine.

Secondary Immunodeficiency and Risk of Infection Following Immune Therapies in Neurology.

Secondary immunodeficiencies (SIDs) are acquired conditions that may occur as sequelae of immune therapy. In recent years a number of disease-modifying therapies (DMTs) has been approved for multiple sclerosis and related disorders such as neuromyelitis optica spectrum disorders, some of which are frequently also used in- or off-label to treat conditions such as chronic inflammatory demyelinating polyneuropathy (CIDP), myasthenia gravis, myositis, and encephalitis. In this review, we focus on currently available immune therapeutics in neurology to explore their specific modes of action that might contribute to SID, with particular emphasis on their potential to induce secondary antibody deficiency. Considering evidence from clinical trials as well as long-term observational studies related to the patients' immune status and risks of severe infections, we delineate long-term anti-CD20 therapy, with the greatest data availability for rituximab, as a major risk factor for the development of SID, particularly through secondary antibody deficiency. Alemtuzumab and cladribine have relevant effects on circulating B-cell counts; however, evidence for SID mediated by antibody deficiency appears limited and urgently warrants further systematic evaluation. To date, there has been no evidence suggesting that treatment with fingolimod, dimethyl fumarate, or natalizumab leads to antibody deficiency. Risk factors predisposing to development of SID include duration of therapy, increasing age, and pre-existing low immunoglobulin (Ig) levels. Prevention strategies of SID comprise awareness of risk factors, individualized treatment protocols, and vaccination concepts. Immune supplementation employing Ig replacement therapy might reduce morbidity and mortality associated with SIDs in neurological conditions. In light of the broad range of existing and emerging therapies, the potential for SID warrants urgent consideration among neurologists and other healthcare professionals.