Child Neurology Society Research Articles

53rd Child Neurology Society Annual Meeting.

53rd Child Neurology Society Annual Meeting.

Analysis of Gender Discrepancies in Leadership Roles and Recognition Awards in the Child Neurology Society.

Gender disparities have been demonstrated across several medical specialties, including neurology. Although women have comprised most of the child neurology trainees since 2007, it is not apparent whether this demographic shift is reflected in the Child Neurology Society (CNS) awards and leadership. This study aimed to evaluate the differences in gender representation among leadership positions and award recipients within the CNS. The primary outcome measure was the total number of board of director (BOD) positions or awards given by gender each year. A retrospective review of publicly available data was conducted on CNS members, post-training award recipients, and BOD positions, including nomination records, from 1972 to 2023. Data abstracted were restricted to gender to preserve member and nominee anonymity. Gender identification and consensus were determined through a combination of strategies and study members. Data analysis included descriptive statistics, Pearson χ2 test, and the exact binomial test to compare gender proportions and the probability of being underrepresented in awards, leadership, and nominations over time. Data are presented according to the Strengthening the Reporting of Observational Studies in Epidemiology guidelines. From 1972 to 2023, women represented 29% (44/152) of the BOD positions and 26% (61/236) of post-training award recipients presented by the CNS. Despite the increase in the proportion of women in child neurology, the overall gap in gender representation in leadership positions remains broadly stable. Only 13% (4/32) of CNS presidents have been women, a significant underrepresentation (95% CI 2.3%-52%, p < 0.004), although the representation of women in nonpresidential positions increased from 2003 to 2023. Women are also underrepresented as overall awardees (95% CI 12%-38%, p < 0.00001) except for the Philip R. Dodge Young Investigator Award, which is an investigator-initiated application. Women remain underrepresented at the highest levels of recognition in child neurology despite representing most of the field. Reasons for disparities are known to be multifactorial and likely include gender bias and structural sexism. We present several discussion topics that seek to rationalize this disparity and provide suggestions for improving diversity, equity, and inclusion for leadership roles and awards.

Opioid neurotoxicity: A case series and review from members of the Child Neurology Society Neurocritical Care Special Interest Group

Opioid neurotoxicity: A case series and review from members of the Child Neurology Society Neurocritical Care Special Interest Group

Brazilian consensus recommendations on the diagnosis and treatment of autoimmune encephalitis in the adult and pediatric populations.

Autoimmune encephalitis (AIE) is a group of inflammatory diseases characterized by the presence of antibodies against neuronal and glial antigens, leading to subacute psychiatric symptoms, memory complaints, and movement disorders. The patients are predominantly young, and delays in treatment are associated with worse prognosis. With the support of the Brazilian Academy of Neurology (Academia Brasileira de Neurologia, ABN) and the Brazilian Society of Child Neurology (Sociedade Brasileira de Neurologia Infantil, SBNI), a consensus on the diagnosis and treatment of AIE in Brazil was developed using the Delphi method. A total of 25 panelists, including adult and child neurologists, participated in the study. The panelists agreed that patients fulfilling criteria for possible AIE should be screened for antineuronal antibodies in the serum and cerebrospinal fluid (CSF) using the tissue-based assay (TBA) and cell-based assay (CBA) techniques. Children should also be screened for anti-myelin oligodendrocyte glucoprotein antibodies (anti-MOG). Treatment should be started within the first 4 weeks of symptoms. The first-line option is methylprednisolone plus intravenous immunoglobulin (IVIG) or plasmapheresis, the second-line includes rituximab and/or cyclophosphamide, while third-line treatment options are bortezomib and tocilizumab. Most seizures in AIE are symptomatic, and antiseizure medications may be weaned after the acute stage. In anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis, the panelists have agreed that oral immunosuppressant agents should not be used. Patients should be evaluated at the acute and postacute stages using functional and cognitive scales, such as the Mini-Mental State Examination (MMSE), the Montreal Cognitive Assessment (MoCA), the Modified Rankin Scale (mRS), and the Clinical Assessment Scale in Autoimmune Encephalitis (CASE). The present study provides tangible evidence for the effective management of AIE patients within the Brazilian healthcare system.

Effects of explainable artificial intelligence in neurology decision support.

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. 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. 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. 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.

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.

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.

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.

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

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

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

The 65th Annual Meeting of the Japanese Society of Child Neurology

The 65th Annual Meeting of the Japanese Society of Child Neurology

Developmental milestones as ACNS turns one year old

Developmental milestones as ACNS turns one year old

SIGnature Libraries: A roadmap for the formation of special interest group libraries.

"SIGnature Libraries" channel the dynamism of academic society-based special interest groups (SIG) to systematically identify and provide user-oriented access to essential literature for a subspecialty field in a manner that keeps pace with the field's continuing evolution. The libraries include literature beyond clinical trial data to encompass historical context, diagnostic conceptualization, and community organization materials to foster a holistic understanding of how neurologic conditions affect individuals, their community, and their lived experience. Utilizing a modified-Delphi approach, Child Neurology Society's Cerebral Palsy (CP) SIG (n = 75) administered two rounds of literature submissions and ratings. A final review by an 11-member international advisory group determined threshold ratings for resource inclusion and the library's final structure. Seventy-nine articles were submitted for the first Delphi round and 22 articles for the second Delphi round. Survey response rates among SIG members were 29/75 for the first round and 24/75 for the second round. The advisory board added additional articles in the final review process in view of the overall project goal. A total of 60 articles were included in the final library, and articles were divided into seven sections and stratified by rating scores. A process for ongoing revisions of the library was determined. The library will be published on the Child Neurology Society website and made publicly accessible. The CP SIGnature Library offers learners an unprecedented resource that provides equitable access to latest consensus guidelines, existing seminal datasets, up-to-date review articles, and other patient care tools. A distinctive feature of the library is its intentional large scope and depth, presented in a stratified fashion relative to the consensus-determined importance of each article. Learners can efficiently navigate the library based on individual interests and goals, and the library can be used as core curriculum for CP education.

Fetal Neurology Practice Survey (P10-9.001)

<h3>Objective:</h3> To study the fetal neurology care delivery across centers in the US. <h3>Background:</h3> Fetal neurology is a rapidly evolving subspecialty that centers on the diagnosis and treatment of neurologic conditions of the developing brain. Innovations in fetal diagnostic tools and therapies have resulted in an increased need for specialized training. Prenatal diagnostic input with postnatal continuity of care can better address sequelae including developmental, behavioral, epileptic and mental health disorders. Despite the growing need for fetal neurology services, little is known about the current practice. <h3>Design/Methods:</h3> The Fetal Neurology Consortium (representatives from 9 sites across the US) distributed a survey characterizing fetal neurologic care delivery to practicing child neurologists using a Redcap database posted to the Child Neurology Society member website in November 2021 and March 2022. The results were summarized using descriptive statistics. <h3>Results:</h3> Representatives from 43 US institutions responded. Most centers had a fetal diagnostic center (n=38/43, 88%), with about half using a fetal neurology registry. Most performed fetal ultrasound and MRI on site. The annual fetal consultations ranged between &lt;20 for 40% to &gt;100 for 17%. About 50% of respondents were subspecialty trained. Earliest gestational age of fetal MRI was 16–19 weeks in 30% and 20–23 weeks in 30%. 25% of respondents participated in interdisciplinary consultations. Majority of the respondents followed neurodevelopment clinically. 91% were interested in participating in a collaborative national registry. The top-five priorities ranked by respondents were clinical guidelines, long-term follow-up data, imaging, practice improvement and prenatal counseling technique. <h3>Conclusions:</h3> This survey highlights heterogeneity in fetal neurology practice, lack of standardization, and high demand for practice guidelines and education. Interdisciplinary collaborations will be crucial for relevant curriculum and program development, including increasing attention to healthcare disparities. This synergy among different disciplines will improve outcomes and may reduce the burden of neurological diseases with fetal origins across the lifespan. <b>Disclosure:</b> Dr. Tarui has nothing to disclose. Dr. Venkatesan has nothing to disclose. Dr. Gano has received personal compensation in the range of $5,000-$9,999 for serving as an Expert Witness for Clifford Law Offices. The institution of Dr. Gano has received research support from Cerebral Palsy Alliance Research Foundation. Dr. Lemmon has received research support from NINDS. Dr. Lemmon has received research support from BAND foundation. Dr. Lemmon has received research support from Doris Duke Charitable Foundation. The institution of Dr. Mulkey has received research support from Thrasher Research Fund. The institution of Dr. Mulkey has received research support from Centers of Disease Control. The institution of Dr. Mulkey has received research support from National Institutes of Health. The institution of Dr. Pardo has received research support from UCB Biosciences. The institution of Dr. Pardo has received research support from The Patrick G. and Shirley W. Ryan Foundation . The institution of Dr. Pardo has received research support from NINDS- Stroke Trials Network. The institution of Dr. Pardo has received research support from NINDS-I-ACQUIRE. The institution of Dr. Pardo has received research support from NINDS- VIPS-II. Dr. Emrick has received personal compensation in the range of $5,000-$9,999 for serving as an Expert Witness for Arkema. The institution of Dr. Emrick has received research support from Lysogene. The institution of Dr. Emrick has received research support from PTC. The institution of Dr. Emrick has received research support from Roche. The institution of Dr. Emrick has received research support from NIH. Dr. Scher has nothing to disclose. Dr. Agarwal has nothing to disclose.

The Gomez-Lopez-Hernandez Syndrome: the contribution of two Hispanic giants of Pediatric Neurology. (P4-3.004)

<h3>Objective:</h3> Detail the history of the Gomez-Lopez-Hernandez syndrome (GLHS) which highlights the contribution of two giants of Pediatric Neurology of Hispanic Origin. <h3>Background:</h3> GLHS also known as cerebello-trigeminal dermal dysplasia, is a rare neurocutaneous condition of variable phenotype which was first described by two Hispanic renowned pediatric neurologists: Drs. Manuel Gomez and Arturo Lopez-Hernandez. GLHS is characterized by rhombencephalosynapsis, alopecia, and trigeminal anesthesia. Dr. Gomez of Spanish-Cuban origin is known for his long tenure as former head of Pediatric Neurology, Mayo Clinic, founder of the Child Neurology Society and his strong interest in the phakomatoses. His index GLHS case was of a 4.5-year-old girl with absent corneal reflexes, alopecia, delayed motor function, and bilateral facial analgesia [1979]. Gomez postulated that arrest of the neuroectoderm development resulted in the abnormal trigeminal innervation. Dr. Lopez-Hernandez is one of the fathers of Pediatric Neurology in Mexico having established the first pediatric neurology service at the National Institute of Pediatrics and later serving as its first head of the Neurology Department. In 1982 Lopez-Hernandez reported two unrelated girls with craniosynostosis, ataxia, trigeminal anesthesia and parietal alopecia associated with pons-vermis fusion. The GLHS eponym was first used in the literature in 1997. To date there are nearly 60 cases reported. The phenotype has been expanded to include short stature, craniofacial anomalies, growth hormone deficiency, hydrocephalus, and neuropsychiatric symptoms. No genetic cause has been identified. <h3>Design/Methods:</h3> Not applicable <h3>Results:</h3> Not applicable <h3>Conclusions:</h3> The specialty of Pediatric Neurology emerged during the 20^th century. During this period, many neurologists played significant roles in revolutionizing this field. The Gomez-Lopez-Hernandez syndrome serves to highlight the contributions of two notable giants of pediatric neurology of Hispanic origin, at a time when there was limited representation of minorities in the medical profession. <b>Disclosure:</b> Ms. Liang has nothing to disclose. Dr. Viereck has nothing to disclose. The institution of Dr. Liow has received research support from UCB. The institution of Dr. Liow has received research support from Livanova. The institution of Dr. Liow has received research support from Biogen. The institution of Dr. Liow has received research support from Novartis. The institution of Dr. Liow has received research support from Eisai. The institution of Dr. Liow has received research support from Engage Therapeutics. The institution of Dr. Liow has received research support from SK Lifescience. The institution of Dr. Liow has received research support from Cerevel. The institution of Dr. Liow has received research support from Xenon. The institution of Dr. Liow has received research support from NeuroDerm. The institution of Dr. Liow has received research support from Avanir. The institution of Dr. Liow has received research support from Annovis. The institution of Dr. Liow has received research support from Acadia. Enrique Carrazana has received personal compensation for serving as an employee of Neurelis. Enrique Carrazana has received personal compensation in the range of $100,000-$499,999 for serving as a Consultant for Neurelis, Alexza, Zogenix. Enrique Carrazana has received personal compensation in the range of $10,000-$49,999 for serving as an officer or member of the Board of Directors for Hawaii-Biotech, CND Life Sciences. Enrique Carrazana has stock in Neurelis, Marinus, .

The Role of Child Neurologists in the Management of Motor Disability in Cerebral Palsy: Establishing the Path Forward

BackgroundCerebral palsy (CP) is the most common motor disability of childhood, and yet the role of child neurologists and neurodevelopmentalists (CN/NDDs) in the management of children with CP is unclear. Although previous surveys showed that CN/NDDs believe they are uniquely expert in CP motor phenotyping and should be involved in CP management, others have demonstrated that training in CP management among CN/NDD residency programs is inadequate. MethodsIn this article, we surveyed a group of CN/NDDs at the Child Neurology Society Cerebral Palsy Special Interest Group meeting on January 27, 2022. Questions addressed provider comfort with CP tone management including motor phenotyping, pharmacologic and surgical management, barriers and solutions to improving practice, and the use of systems-based care. ResultsResponses from 42 participants demonstrated that CN/NDDs lack experience with CP tone management, with 48% and 58% of respondents reporting little to no experience in pharmacologic or surgical management, respectively. Primary barriers identified to improving comfort with CP tone management included lack of knowledge and ineffective treatment options, while most solutions centered on improving collaborations between CN/NDDs and other specialties. Only 50% of respondents reported currently using systems-based care in the management of patients with CP. ConclusionsAn interdisciplinary, systems-based care model would allow for collaboration and knowledge sharing between involved specialties and provide high-value goal-directed care to maximize the functional outcomes for every individual with CP.

Severe pediatric acute encephalopathy syndromes related to SARS-CoV-2.

To clarify whether severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection cause acute encephalopathy in children and which are the most common syndromes that cause them and what are the outcomes. A nationwide web-based survey among all members of the Japanese Society of Child Neurology to identify pediatric patients aged < 18 years who developed acute encephalopathy in Japan between 1 January 2020 and 31 May 2022 associated with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection confirmed by polymerase chain reaction or antigen tests using pharyngeal swabs. Acute encephalopathy was defined as acute onset of impaired consciousness lasting > 24 h or an altered mental state; neurological symptoms arising within 2 weeks of onset of COVID-19 or multisystem inflammatory syndrome in children (MIS-C)/pediatric inflammatory multisystem syndrome (PIMS); evidence of SARS-CoV-2 infection; and reasonable exclusion of other diseases. Patients were divided into the known clinico-radiological acute encephalopathy syndrome group and unexplained or unclassifiable acute encephalopathy group. Outcomes were assessed by pediatric cerebral performance category (PCPC) score at hospital discharge. Of the 3,802 society members, 217 representing institutions responded, and 39 patients with suspected acute encephalopathy were reported, of which 31 met inclusion criteria. Of these patients, 14 were diagnosed with known clinico-radiological acute encephalopathy syndromes, with acute encephalopathy with biphasic seizures and late reduced diffusion (five patients) being the most common. Five developed acute encephalopathy associated with MIS-C/PIMS. Among 31 patients, 9 (29.0%) had severe sequelae or died (PCPC ≥ 4). Two of three patients with encephalopathy with acute fulminant cerebral edema and two with hemorrhagic shock and encephalopathy syndrome died. The PCPC scores were higher in the known clinico-radiological acute encephalopathy syndrome group than in the unexplained or unclassifiable acute encephalopathy group (P < 0.01). Acute encephalopathy related to SARS-CoV-2 infection was demonstrated to be more severe than that caused by other viruses in Japan. Acute encephalopathy syndromes characterized by specific neuroradiological findings was associated with poor clinical outcomes.

A half‐century check‐up for child neurology training

A half‐century check‐up for child neurology training