Child Neurology Research Articles

Addressing Early Education and Child Care Expulsion.

An important goal of early childhood education is teaching emotional self-regulation within the context of a safe, stable, nurturing environment. Expulsion of young children ignores underlying emotional and behavioral concerns, disproportionately affects children of color (Black or Hispanic), males, children with disabilities, and socioeconomically disadvantaged populations, and has long-term consequences on educational and life success. Addressing implicit bias and providing child mental health consultation (psychologists, social workers, developmental behavioral pediatricians, child psychiatrists, and child neurologists) to child care providers can prevent expulsion. Pediatricians and other providers within the medical home play an important part in preventing expulsion. However, pediatricians need more training in early childhood mental health and in understanding how systemic racism and implicit bias lead to preschool expulsion in children of color. By identifying children at risk for expulsion because of poverty, racial discrimination, toxic stress, insecure attachment, or history of trauma, the pediatrician can connect families with community resources that may ameliorate these effects. Pediatricians can provide information on social-emotional development in early childhood, promote positive parent-child relationships, and model and discuss age-appropriate and developmentally appropriate behavior management. Pediatricians can also guide parents toward high-quality child care programs that use mental health consultation and developmentally appropriate activities, both of which lessen the chance of child expulsion. Furthermore, behavioral health providers integrated into the medical home can provide consultation to child care providers on managing patients. These recommendations are consistent with our knowledge of early child brain development and support the current tenets of the American Academy of Pediatrics regarding the pediatrician's role in building resilience and buffering toxic stress to promote optimal child development.

Neuroplasticity as a driver of beneficial effects in music interventions in children with developmental disorders

The potential to make and to enjoy music is genetically enrooted in humans and is an important resource of joy and quality of live for children and adolescents. Music making induces short-term and long-term neuroplastic adaptations in cortical and subcortical structures. Normally developing children who learn to play a musical instrument show better auditory pattern recognition and auditory memory. Furthermore sensorimotor, intellectual, and emotional maturation are accelerated. These effects can also be used beneficially through music interventions in children and adolescents suffering from neurodevelopmental disorders, from hearing disorders, or from cerebral palsy. Children with autism spectrum disorders learn better self-control and attention management through musical interventions and are supported in contact and communication skills. For children with cochlear implants, musical training can improve long-term outcomes not only in music perception but also in speech perception. Finally, learning a musical instrument and the associated sensorimotor-auditory and emotional integration may help children with infantile cerebral palsy improve fine motor skills but also emotional stability. Due to the numerous positive results (albeit frequently in studies with small numbers and lower quality), we advocate the increased use of qualified music intervention in child neurology and its accompanying scientific evaluation.

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.

The 2023 AAN/AAP/CNS/SCCM Pediatric and Adult Brain Death/Death by Neurologic Criteria Consensus Practice Guideline: A Comparison With the 2010 and 2011 Guidelines.

In collaboration with the American Academy of Pediatrics, Child Neurology Society, and Society for Critical Care Medicine, the American Academy of Neurology formulated an updated, evidence-informed consensus-based guideline for pediatric and adult brain death/death by neurologic criteria (BD/DNC) determination. In comparison with the prior guidelines, the revisions and additions in this guideline, which are summarized in this review, are intended to (1) ensure recommendations are conservative, yet practical, and emphasize circumstances in which BD/DNC determination should be delayed or deferred, so as to minimize the risk of a false-positive BD/DNC determination; and (2) provide guidance about aspects of BD/DNC determination that clinicians find challenging and/or controversial. We hope that clinicians throughout the United States will use this information to revise their hospital BD/DNC determination policies to conform to the standardized process for BD/DNC determination described in the new guideline, to ensure that every BD/DNC evaluation is consistent and accurate.

Pediatric and Adult Brain Death/Death by Neurologic Criteria Consensus Guideline.

The purpose of this guideline is to update the 2010 American Academy of Neurology (AAN) brain death/death by neurologic criteria (BD/DNC) guideline for adults and the 2011 American Academy of Pediatrics, Child Neurology Society, and Society of Critical Care Medicine guideline for infants and children and to clarify the BD/DNC determination process by integrating guidance for adults and children into a single guideline. Updates in this guideline include guidance related to conducting the BD/DNC evaluation in the context of extracorporeal membrane oxygenation, targeted temperature management, and primary infratentorial injury. A panel of experts from multiple medical societies developed BD/DNC recommendations. Because of the lack of high-quality evidence on the subject, a novel, evidence-informed formal consensus process was used. This process relied on the panel experts' review and detailed knowledge of the literature surrounding BD/DNC to guide the development of preliminary recommendations. Recommendations were formulated and voted on, using a modified Delphi process, according to the 2017 AAN Clinical Practice Guideline Process Manual. Eighty-five recommendations were developed on the following: (1) general principles for the BD/DNC evaluation, (2) qualifications to perform BD/DNC evaluations, (3) prerequisites for BD/DNC determination, (4) components of the BD/DNC neurologic examination, (5) apnea testing as part of the BD/DNC evaluation, (6) ancillary testing as part of the BD/DNC evaluation, and (7) special considerations for BD/DNC determination.

Moyamoya Angiopathy: An Underdiagnosed Cause of Ischemic Stroke in a Tunisian Pediatric Cohort

BackgroundMoyamoya angiopathy is a rare cerebral vasculopathy and an underdiagnosed cause of arterial ischemic stroke in children. We aim to report the clinical and radiological presentations in a Tunisian pediatric cohort. MethodsWe identified moyamoya angiopathy in pediatric patients managed at the Child Neurology Department of Hedi Chaker Sfax University Hospital between 2008 and 2020 and reviewed their clinical and radiological data as well as their evolutionary profile. ResultsWe collected 14 patients with median age 40.6 months and a female predominance (sex ratio of 0.75). An arterial ischemic stroke (AIS) revealed the disease in all patients, with the major symptom being a motor deficit. Symptoms related to a transient ischemic attack before the diagnostic consultation were reported in four patients. Carotid territory was, clinically and radiologically, the most frequently involved. Brain magnetic resonance imaging with angiography was performed in 12 patients confirming the diagnosis by revealing the development of collateral vessels. All the investigations concluded to moyamoya disease in 57.2% and moyamoya syndrome in 42.8%. The latter was related to Down syndrome in five patients and neurofibromatosis type 1 in one patient. With a mean follow-up of 2.35 years, two patients had at least two more AISs during the first two years following diagnosis and 42.8% of patients were diagnosed with vascular or poststroke epilepsy. Full recovery was noted in 14.3% of cases. ConclusionsMoyamoya angiopathy in children is a serious condition that needs to be recognized due to the high risk of recurrent ischemic strokes.

Lennox–Gastaut Syndrome: Challenges in Diagnosis – Interviews with Two Key Opinion Leaders

Lennox–Gastaut syndrome (LGS) is a severe childhood-onset epilepsy syndrome that can be challenging to identify due to its heterogeneous and evolving presentation. During interviews conducted by EMJ in July and August 2023, Karen Keough, a child neurologist specialising in paediatric refractory epilepsy at Texas A&M University School of Medicine, Round Rock, USA, and Rhys Thomas, an adult neurologist specialising in epilepsy (leading on learning disability and genetic testing) at the Royal Victoria Infirmary, Newcastle, UK, and Newcastle University, UK, gave their informed opinions on the challenges of diagnosing LGS. The experts spoke about the characteristic features of LGS, and how its gradual evolution from childhood onset can impede diagnosis. In particular, the issues around diagnosing LGS in adulthood, after considerable disease evolution and against a background of uncertain/incomplete medical history, were discussed. The experts also considered the 2022 International League Against Epilepsy (ILAE) position paper for the definition of epilepsy syndromes in childhood, and its potential impact for directing the diagnosis of LGS. Keough and Thomas concluded by describing how raised awareness, vigilance, and support in diagnosis could help to optimise treatment and readjust the goals for the management of adults and children with LGS.

Current Practices in the Evaluation of Global Developmental Delay/Intellectual Disability: A Nationwide Survey of Child Neurologists.

Global developmental delay/intellectual disability (GDD/ID) are among the most common neurologic conditions evaluated by child neurologists in the United States. No recent neurology-specific guidelines for GDD/ID diagnostic evaluation exist, which could lead to practice variability. We assessed current practices in GDD/ID diagnostic evaluation among US child neurologists, including drivers of exome sequencing (ES). A 19-item online anonymous survey was distributed between April 2021 and September 2021 to 953 eligible child neurologists by email and/or online platforms through the American Academy of Neurology and Child Neurology Society. Multinomial logistic regression was used to determine the predictors of sending ES as a part of GDD/ID diagnostic evaluation. Of 172 unique respondents, 69.2% reported almost always obtaining a chromosomal microarray while 10.5% reported almost always pursuing ES. However, 65.1% identified ES as a first-tier diagnostic test for GDD/ID. Clinical practice demographics independently associated with a higher likelihood of pursuit of ES were more years of experience (p = 0.002) and more people with GDD/ID in one's practice (p < 0.001). Inclusion of brain MRI, EEG, and metabolic laboratory values as part of GDD/ID diagnostic evaluation varied widely. Modalities to screen for treatable disorders (ES or metabolic laboratory values) were reported to be consistently used by only 24.8% of respondents. Respondents identified key barriers to the pursuit of ES including the need for genetics referral/genetic counseling and insurance coverage/out-of-pocket cost. Among US child neurologists, there is marked practice variability in GDD/ID diagnostic evaluation across multiple types of testing, raising concern for disparities in care. There is a widespread lack of screening for treatable causes of ID, which may lead to missed diagnoses and avoidable morbidity. Despite most respondents' support for ES as a first-tier diagnostic test for GDD/ID, only a small minority routinely pursue ES as a part of their evaluation. Provider-level factors (years of experience, percent of patients with GDD/ID) and system-level barriers (access to genetics expertise, lack of insurance coverage) were determinants of the frequency of use of ES. These findings suggest the need for updated consensus guidelines and advocacy/education to improve child neurologists' ability to pursue ES for GDD/ID.

Clinical Evaluation and Management of Narcolepsy in Children and Adolescents

While sleepiness is common among children, and particularly adolescents, profound sleepiness in the setting of apparently adequate sleep should prompt consideration of a central disorder of hypersomnolence. These disorders, which include narcolepsy, idiopathic hypersomnia, Kleine-Levin syndrome, and others, are likely underrecognized in the pediatric population. Narcolepsy in particular should be of interest to child neurologists as the unique signs and symptoms of this disease often prompt evaluation in pediatric neurology clinics. While sleepiness may appear to be a straightforward complaint, its evaluation requires a nuanced approach. Cataplexy, a hallmark of narcolepsy, can be confused for other neurologic conditions, though understanding its various manifestations makes it readily identifiable. Clinicians should be aware of these symptoms, as delay in diagnosis and misdiagnosis are common in childhood narcolepsy. While treatment options have been limited in the past, many new therapeutic options have become available and can result in significant improvement in symptoms. Given the age at presentation, paroxysmal and chronic features, diagnostic modalities, and available treatment options, the field of child neurology is well equipped to see patients with narcolepsy. In this review, I will focus on the presentation, evaluation, and management of pediatric patients with narcolepsy.

52nd Annual Meeting of the Child Neurology Society Scientific Program.

52nd Annual Meeting of the Child Neurology Society Scientific Program.

Diagnostic Algorithm for Pediatric Headaches: A Clinical Improvement Initiative

BackgroundThe widespread variation in diagnosing primary headache disorders in children and adolescents results in reduced quality and high costs. Defining an algorithm for primary headache diagnoses in children and adolescents is part of a larger initiative to standardize and improve care. The aim of this algorithm was to increase the accuracy of headache diagnosis by formal criteria to more than 80% of patient encounters. MethodsA team of headache specialists, nurse practitioners, nurses, data analysts, and business specialists developed an algorithm based on available scientific evidence. This algorithm was vetted and adapted by the neurology faculty and headache specialists until final consensus was reached. Following three months of testing and validation, the algorithm was disseminated to general pediatric neurology clinics. The following information was gathered: percent of encounters utilizing the algorithm, percentage of encounters with appropriate diagnosis by formal criteria, percentage of encounters with appropriate testing ordered, and average cost per headache visit. ResultsCorrect diagnosis of primary headache by International Classification of Headache Disorders-3 criteria improved from 72% to 90% and appropriate testing improved from 80% to 94%. By the end of analysis, 94% of encounters were correctly implementing the algorithm. A year-long tracking revealed decreased cost of headache evaluation by 6% compared with the year prior. ConclusionsA standardized algorithm improved the diagnostic accuracy in general child neurology clinics. Expanding the algorithm to primary care and pediatric emergency rooms could have a greater impact on headache evaluation and diagnosis; this should result in improved care and outcomes with reduced cost.

Mandatory child neurology training for adult neurology residents in Pakistan

Mandatory child neurology training for adult neurology residents in Pakistan

Local Culture and Community Through a Digital Lens: Viewpoint on Designing and Implementing a Virtual Second Look Event for Residency Applicants.

The COVID-19 pandemic altered how residency interviews occur. Despite 2 years of web-based interviews, these are still perceived as inferior to in-person experiences. Showcasing a program and location is critical for recruitment; however, it is difficult to highlight the program's location and community digitally. This article presents the authors' viewpoints on designing and implementing a virtual second look for residency applicants. Our objective was to host a web-based event to feature the benefits of living in Winston-Salem, North Carolina, for residency applicants, enhance recruitment efforts, and ensure a successful residency match. The goal was to cover topics that interested all applicants, highlight how Winston-Salem is a special place to live, involve current residents, and engage community members. Three programs-child neurology, neurology, and family medicine were chosen for a pilot virtual second look. All residency program directors' were asked to recommend community contacts and help identify residents and faculty who may serve as content experts on one of the topics in the panel discussions. A total of 24 community leaders from restaurants, venues, schools, and businesses were contacted, and 18 agreed to participate. The panel discussions included living in and raising a family in Winston-Salem, experiencing Winston-Salem arts and music, where to eat and drink like a local, and enjoying sports and outdoors in the area. The 2-hour event was hosted on Zoom. Postevent feedback assessments were automatically sent to each registrant through Research Electronic Data Capture (REDCap). This study was deemed exempt from Wake Forest University Health Sciences institutional review board review (IRB00088703). There were 51 registrants for the event, and 28 of 48 registrants provided postevent feedback, which was positive. The authors found in the MATCH residency results that 2 of 2 child neurology positions, 4 of 6 adult neurology positions, and 1 of 10 family medicine positions attended our second look event. One adult neurology resident who did not participate was an internal candidate. All respondents agreed or strongly agreed that the session was valuable, well organized, and met their expectations or goals. Furthermore, all respondents gained new information during this web-based event not obtained during their interview day. The virtual second look event for residency attendees featured the benefits of living in Winston-Salem, and the perspectives of current residents. Feedback from the session was overall positive; however, a top desire would be devoting more time for the applicants to ask questions directly to the community leaders and our resident trainees. This program could be reproducible by other institutions. It could be broadened to a graduate medical education-wide virtual second look event where all medical and surgical programs could opt to participate, facilitating an equitable opportunity for prospective applicants.

Curriculum Innovations: A Comprehensive Teleneurology Curriculum for Neurology Trainees.

As the role of teleneurology expands, it is important to prepare trainees to perform virtual encounters proficiently. We created a comprehensive multimodality teleneurology curriculum for residents to teach key aspects of telehealth encounters including the virtual examination and skill development across several environments. We developed and implemented a teleneurology curriculum focused on teaching the virtual neurologic examination, measuring teleneurology competency, and providing opportunities for trainees to perform telehealth encounters in multiple settings. Residents (N = 22) were first surveyed on what methods would be most helpful to learn teleneurology. Trainees observed a faculty member conducting a teleneurology visit with another faculty member playing a patient. Residents then practiced a teleneurology encounter during a 10-minute objective structured clinical examination (OSCE) at a simulation center. After positive feedback from the fall of 2020, we adapted the OSCE to be completely remote in the spring of 2021 for senior residents. Trainees then performed teleneurology visits during their continuity clinics and subspecialty clinic rotations. All neurology residents from adult and child neurology and neuropsychiatry programs at New York University Grossman School of Medicine participated in the curriculum. Residents identified a variety of teaching modalities that would help them learn teleneurology: didactics with slides (25%), live demonstration (25%), simulated experience (23%), starting with live patients (23%), and articles/reading material (4%). To date, 68 trainees participated in the OSCE. Trainees who completed on-site and remote simulations reported increased comfort (p < 0.05) and interest in teleneurology (p < 0.05) and requested more access to simulations during training. Sensorimotor assessment and adequate visualization of the affected limb were identified as areas for improvement. Our multimodal 3-year teleneurology curriculum provides opportunities for residents to learn and apply teleneurology. Survey tools helped strengthen the curriculum to optimize educational potential. We implemented a teleneurology simulation with and without the use of a simulation center. We plan to expand our teleneurology clinical and simulation experiences to trainees based on our data and further developments in teleneurology and to track the progress of teleneurology skills as residents advance through training.

Brazilian experts' consensus on the treatment of infantile epileptic spasm syndrome in infants.

Infantile epileptic spasms syndrome (IESS) is a rare but severe condition affecting children early and is usually secondary to an identifiable brain disorder. It is related to psychomotor deterioration in childhood and epilepsy in adult life. Treatment is challenging as infantile spasms may not respond to most antiseizure medication, and relapse is frequent. To evaluate the literature regarding treatment of IESS and provide a practical guidance to a healthcare system with limited resources. An expert committee from the Brazilian Society of Child Neurology reviewed and discussed relevant scientific evidence in the treatment of IESS regarding the drugs available in Brazil. Oral prednisolone and vigabatrin are the most common drugs used as first-line therapy; they are efficient and affordable therapy as both are available in the Brazilian unified health system (SUS, in the Portuguese acronym). Intramuscular adrenocorticotropic hormone (ACTH) presents similar efficacy as oral prednisolone but has a higher cost and is not available in Brazil. Other antiseizure medications such as topiramate, levetiracetam, or benzodiazepines have limited response and are prescribed as adjuvant therapy. If the health service has nutritionists, a ketogenic diet should be implemented for those not responding to hormonal and vigabatrin treatment. Epilepsy surgery is mainly indicated for patients with focal lesions that do not respond to pharmacological therapy. Early treatment of IESS with efficient drugs is feasible in our country. Using standard protocols increases the odds of achieving complete cessation in a shorter time and decreases relapse.

Celebrating the 30th Anniversary of the Korean Child Neurology Society

Celebrating the 30th Anniversary of the Korean Child Neurology Society

High-Yield, Case-Based, Interactive Workshop on Telehealth and Teleneurology With Pediatric Resident Physicians.

Increasing prevalence of neurologic disorders with an aging global population and limited availability of neurologists may lead to worse patient outcomes. As a result of the COVID-19 pandemic, telehealth services surged, and despite easing public health measures, the demand has remained. Telehealth technology has the potential to close the physical gaps in expanding the reach of care. This academic half-day workshop sought to provide a learning opportunity in response to these concerns. The workshop consisted of small- and large-group case discussions among pediatric resident physicians (PGY 1-PGY 3) moderated by two child neurology faculty physicians over Zoom. Participants received a learner document with prereading articles and questions for each case. PowerPoint presentations with video demonstrations were used to introduce the cases and guide discussions. Of the 25 attendees, 14 (56% response rate) answered a nonmandatory postsession survey. Eighty-six percent of the respondents were very or extremely satisfied with the content covered and were similarly satisfied with the effectiveness of content delivery. Seventy-nine percent of the respondents found the content helpful or very helpful in preparation for the board, and 93% anticipated applying the content covered occasionally or frequently in their clinical practice. Small-group discussions with video demonstrations are helpful in increasing proficiency with telehealth technology and in examining board-relevant cases on pediatric patients. There is strong interest in subsequent telehealth half-day workshops that incorporate teaching through group discussions on relevant patient case scenarios.

Telemedicine in Developing Countries: Knowledge, Attitudes, and Practices of Caregivers of Children with Epilepsy Regarding Telemedicine at the Philippine Children’s Medical Center

Purpose: The coronavirus disease 2019 pandemic has prompted the use of telemedicine as an alternative method for providing continuous epilepsy care. This study was conducted to validate and administer a web-based questionnaire to assess the knowledge, attitudes, and practices (KAP) regarding telemedicine among caregivers of children with epilepsy at the Philippine Children’s Medical Center (PCMC). Methods: This cross-sectional study was conducted by a primary investigator from the child neurology section of PCMC between July 2022 and October 2022 and consisted of two phases. In phase 1, content validation and pre-testing of the Filipino version of the questionnaire were conducted with 29 caregivers. Phase 2 involved the web-based administration of the final version of the questionnaire to 89 caregivers. Results: The resulting questionnaire comprised four main sections: demographics, KAP. Regarding caregivers’ knowledge, 70.8% had not heard of telemedicine before participating in teleconsultations at PCMC. However, most participants were able to correctly identify its purposes (86.4%), benefits (87.6%), and barriers (78.7%). All aspects of the caregivers’ attitudes demonstrated positive agreement with the Likert scale of attitudes, with &lt;i&gt;P&lt;/i&gt; values of &lt;0.01, which indicated statistical significance. The most common device used was a cellular phone, and most caregivers identified Facebook Messenger, Viber, and Zoom as the most useful platforms. Conclusion: Our study revealed low awareness of the availability of telemedicine services but good knowledge of its purposes, benefits, and barriers. Caregivers exhibited positive attitudes toward telemedicine, with Facebook Messenger identified as the most useful platform.

Empowering differences: Disability in child neurology training

Empowering differences: Disability in child neurology training

Pediatric Neurology Workforce in Saudi Arabia: A 5-Year Update.

The medical workforce plays a pivotal role in advancing human health, particularly within the healthcare system of Saudi Arabia. While government-employed healthcare providers form the central structure of the system and offer free healthcare services, the private healthcare sector is also witnessing significant growth. In parallel, the field of child neurology has experienced notable transformations in recent years, with continued expansion. This expansion brings forth a range of challenges for both current and future pediatric neurologists, necessitating careful consideration and proactive measures to address them. To investigate and analyze the current characteristics of the workforce, with a specific focus on their employment status and related data. This is a cross-sectional analysis, using a survey to assess the distribution of pediatric neurologists in Saudi Arabia (SA). The final analytical sample included 82 subjects, working in 13 regions in SA. A descriptive analysis was used to address the study question. The survey received responses from a total of 82 pediatric neurologists in Saudi Arabia (response rate 55%), with 38 (46%) being men and 44 (54%) being women. The mean age was 33 ± 1.225 years. The majority of participants practiced in major cities such as Riyadh and Jeddah. Nearly 50% of pediatric neurologists experienced some form of delay in obtaining their first job, ranging from 1 to 36 months. The landscape of the pediatric neurology workforce is currently witnessing noteworthy demographic shifts. With the majority of practitioners concentrated in major cities, there is an ongoing demand for qualified professionals in peripheral areas. This study describes the real-life challenges faced by pediatric neurologists, particularly the delay in securing employment after graduation, and underscores the critical importance of addressing these persistent issues along the journey of pediatric neurology.