{"title":"First-Drug Efficacy and Drug-Resistant Epilepsy Rates in Children With New-Onset Epilepsies: A Multicenter Large Cohort Study.","authors":"Pinar Gencpinar, Pinar Arican, Nihal Olgac Dündar, Betül Kilic, Esra Sarigecili, Cetin Okuyaz, Kursad Aydin, Hasan Tekgul","doi":"10.1177/08830738241283711","DOIUrl":"https://doi.org/10.1177/08830738241283711","url":null,"abstract":"<p><p><b>Objective:</b> This study aimed to assess the first-drug efficacy rate in newly diagnosed children with epilepsies treated with antiseizure medications. <b>Methods:</b> This retrospective study was conducted on 1003 children (age range: 3-10 years, and the mean duration of follow-up: 22 ± 13 months) with newly diagnosed epilepsy. The following parameters were evaluated: first-drug efficacy rate, first-drug-failure rate, and drug resistance rate in the cohort. <b>Results:</b> The first-drug-failure rate was defined in 335/1003 (33%) of the patients, no seizure control in 315 (31%), and drug withdrawal in 20 (2%). There was no significant difference between the group with focal-onset seizures and the group with generalized onset seizures. The first-drug efficacy rate was 67% in children with focal-onset seizures and 66% in children with generalized-onset seizures. Adjunctive antiseizure medication therapy was initiated in 335 patients-dual therapy with 180 patients (18%) and polytherapy with 155 (15%). Drug-resistant epilepsy was defined as 15% in the follow-up period. Etiology-specific diagnoses of the cohort were structural (n = 165, 17%), genetic (n = 25, 3%), metabolic (n = 15%), immune-infectious (n = 17 (2%), and unknown (n = 781, 77%). With a comparison of the 2 most common etiology subgroups (structural versus unknown), a first-drug efficacy rate of 53% and a higher prevalence of drug-resistant epilepsy at 30% were observed in children with structural etiology. First-drug efficacy was statistically lower in children without well-defined epilepsy syndromes (65%) compared with the rate of those with well-defined epilepsy syndrome (79%). <b>Conclusion:</b> This study revealed a first-drug failure rate (33%) in the presented cohort with a drug-resistance epilepsy rate (15%).</p>","PeriodicalId":15319,"journal":{"name":"Journal of Child Neurology","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2024-09-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142347554","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"We Don't Talk About X(Twitter): A Cross-Sectional Analysis of Social Media Utilization Among Neurologists.","authors":"Justin Rosati, Jaclyn M Martindale, Kathryn Xixis, Rachel Gottlieb-Smith, Gregory Russell, Nancy Bass, Jessica Goldstein","doi":"10.1177/08830738241273371","DOIUrl":"https://doi.org/10.1177/08830738241273371","url":null,"abstract":"<p><strong>Background and objectives: </strong>Medical professionals use social media for career development, education, clinical outreach, or advocacy. Prior studies estimate that 25% to 65% of health care providers use social media professionally; however, the number of users and platforms are rapidly changing. Therefore, as part of a broader study, we set out to assess platform preferences and social media usage among neurologists.</p><p><strong>Methods: </strong>This was a multisite cross-sectional analysis consisting of a REDCap survey of clinicians, residents, and medical students. Faculty, trainees, or clinical year medical students interested in child neurology or adult neurology residency or fellowship programs within the United States were eligible to participate. Recruitment methods were broad to encompass as diverse and extensive participation as possible. Results were analyzed using descriptive statistics. Data are presented according to the STROBE guidelines.</p><p><strong>Results: </strong>Of the 226 neurology respondents, 55% (n = 124) were child neurology and 45% (n = 102) were adult neurology across all career stages, including students. Of the 70% who reported using social media in a professional capacity, the most commonly reported reasons were for networking and collaboration (n = 95, 60%), self-directed medical learning (n = 90, 57%), and brand building and reputation (n = 62, 39%). Twitter and Facebook were the most common and versatile platforms used by neurologists. Medical students had the highest documentation of social media scholarships on their curriculum vitae (37%, <i>P</i> = .016) and the most interest (33%, <i>P</i> = .016) in learning how to document social media scholarships if they were not already. Early faculty shared this interest more than residents, fellows, or mid-late career faculty. In all groups except for mid-late career faculty, a majority of respondents (>75%) showed interest in learning how to leverage social media for career development.</p><p><strong>Discussion: </strong>Social media is used professionally by a majority of neurologists, most commonly for networking, self-directed learning, and building individual brands. Opportunities exist to better understand platform preferences and ways to optimize their use for various professional activities as well as to provide education on effective professional use of social media including documentation for promotion.</p>","PeriodicalId":15319,"journal":{"name":"Journal of Child Neurology","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2024-09-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142347556","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Julie C Skorup, Samuel R Pierce, Noor Ruwaih, Sara B DeMauro, Michelle J Johnson, Laura A Prosser
{"title":"Hammersmith Neonatal and Infant Neurological Examinations Scores in Typically Developing Infants Aged 1-6 Months.","authors":"Julie C Skorup, Samuel R Pierce, Noor Ruwaih, Sara B DeMauro, Michelle J Johnson, Laura A Prosser","doi":"10.1177/08830738241282722","DOIUrl":"https://doi.org/10.1177/08830738241282722","url":null,"abstract":"<p><p>The Hammersmith Neonatal (HNNE) and Infant (HINE) Neurological Examinations are increasingly used to evaluate developing neuromotor control in infants at risk for physical disability, but there is no global consensus on score interpretation across the first 6 months after birth. We report scores for typically developing, full-term infants aged 1 month for the HNNE and aged 2-6 months for the HINE. The median HNNE and HINE scores are consistent with previously published data. These normative data can be used to aid in the interpretation of HNNE and HINE scores from infants at risk for neuromotor impairment.</p>","PeriodicalId":15319,"journal":{"name":"Journal of Child Neurology","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2024-09-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142347555","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Lindsay Johnson-Bishop, Cemal Karakas, Stephen F Kralik, Clay T Cohen, Mark Zobeck, Nick Park, Karen Rabin, Davut Pehlivan, Saleh Bhar
{"title":"Cerebral Sinus Venous Thrombosis in Pediatric Acute Lymphoblastic Leukemia: Incidence, Clinical Characteristics, and Long-term Neurologic Outcomes.","authors":"Lindsay Johnson-Bishop, Cemal Karakas, Stephen F Kralik, Clay T Cohen, Mark Zobeck, Nick Park, Karen Rabin, Davut Pehlivan, Saleh Bhar","doi":"10.1177/08830738241282910","DOIUrl":"https://doi.org/10.1177/08830738241282910","url":null,"abstract":"<p><strong>Objective: </strong>To describe the incidence, clinical characteristics, and long-term outcomes of cerebral sinus venous thrombosis in children with acute lymphoblastic leukemia.</p><p><strong>Methods: </strong>This was a retrospective cohort study comprising pediatric patients with newly diagnosed or first-relapse acute lymphoblastic leukemia who developed cerebral sinus venous thrombosis at Texas Children's Hospital from 2002 to 2019.</p><p><strong>Results: </strong>Nineteen cases (1.7%) with cerebral sinus venous thrombosis were identified in all pediatric patients with acute lymphoblastic leukemia (n = 1129). Increased risk of cerebral sinus venous thrombosis was observed with age >10 years (<i>P </i>= .006). Twelve cases (63%) occurred during the induction, 4 (21%) during maintenance, and 3 (16%) during the consolidation phases of leukemia therapy. Seizures (10/19) and headaches (9/19) were the most common presenting symptoms. After treatment with anticoagulation therapy, we observed full resolution of thrombosis in 10 (53%) and partial resolution in 8 patients (42%). Long-term neurologic outcomes at follow-up in the 14 patients who survived included normal neurologic examinations (n = 10), epilepsy (n = 3), and focal neurologic deficits (n = 2). The death occurred in 5 individuals.</p><p><strong>Conclusion: </strong>Cerebral sinus venous thrombosis is a notable complication of pediatric acute lymphoblastic leukemia therapy. Older age (>10 years) was a risk factor for developing cerebral sinus venous thrombosis. Despite variable patient presentations and treatment durations, favorable clinical outcomes were observed in most patients after the treatment with anticoagulation for a minimum of 3 months.</p>","PeriodicalId":15319,"journal":{"name":"Journal of Child Neurology","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2024-09-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142347553","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Richard B Carozza, Kristen Bolte, Elton B Greene, Shilpa B Reddy, NgocHanh H Vu
{"title":"Cerebral Cortical Encephalitis and Other Meningocortical Manifestations of Myelin Oligodendrocyte Glycoprotein Antibody-Associated Disease in Children: Case Series and Review of the Literature.","authors":"Richard B Carozza, Kristen Bolte, Elton B Greene, Shilpa B Reddy, NgocHanh H Vu","doi":"10.1177/08830738241282354","DOIUrl":"https://doi.org/10.1177/08830738241282354","url":null,"abstract":"<p><p>Myelin oligodendrocyte glycoprotein (MOG) antibody-associated disease is a neuroinflammatory disorder (MOGAD) with heterogeneous phenotype including paroxysms of optic neuritis, transverse myelitis, acute disseminated encephalomyelitis, brainstem demyelination, and encephalitis. Fluid-attenuated inversion recovery hyperintense cortical lesions in MOG-associated encephalitis with seizures, or FLAMES, is a manifestation of cerebral cortical encephalitis seen less frequently than other typical MOG antibody-associated disease presentations. Cases of FLAMES are rarer in children, and frequently initially misdiagnosed with infectious meningoencephalitis. Other meningocortical manifestations of MOG antibody-associated disease have been described and likely exist along a continuum. In this retrospective single-center case series, we describe the demographic, clinical, radiographic, laboratory, and electroencephalographic features of 5 children with clinicoradiographic features consistent with the spectrum of MOG-IgG-positive meningocortical syndromes.</p>","PeriodicalId":15319,"journal":{"name":"Journal of Child Neurology","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2024-09-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142288195","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"Corrigendum to “Genetic Microcephaly in a Saudi Population: Unique Spectrum of Affected Genes Including a Novel One”","authors":"","doi":"10.1177/08830738241287145","DOIUrl":"https://doi.org/10.1177/08830738241287145","url":null,"abstract":"","PeriodicalId":15319,"journal":{"name":"Journal of Child Neurology","volume":null,"pages":null},"PeriodicalIF":1.9,"publicationDate":"2024-09-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142262184","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Stacey Dornette, Stephen Deptola, Brianna Hemmann, Charu Venkatesan, DonnaMaria E. Cortezzo
{"title":"Recognition and Management of Delirium in the Neonatal Intensive Care Unit: Case Series From a Single-Center Level 4 Intensive Care Unit","authors":"Stacey Dornette, Stephen Deptola, Brianna Hemmann, Charu Venkatesan, DonnaMaria E. Cortezzo","doi":"10.1177/08830738241282099","DOIUrl":"https://doi.org/10.1177/08830738241282099","url":null,"abstract":"Delirium often goes unrecognized in neonates and children because of lack of experience in evaluating behavior and cognition, insufficient awareness of the prevalence, and nondistinctive symptoms in this population. Although there are increasing reports of the presence of delirium in neonates, there are few data to guide the pharmacologic treatment in this population. In this retrospective single-center case series, we present our experience using quetiapine to treat delirium in 9 medically complex neonates. Based on an extensive literature review, expert opinion, and institutional experience, we propose an approach for monitoring and treating delirium in neonates and infants.","PeriodicalId":15319,"journal":{"name":"Journal of Child Neurology","volume":null,"pages":null},"PeriodicalIF":1.9,"publicationDate":"2024-09-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142262187","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Akshat Katyayan, Anuranjita Nayak, Gloria Diaz-Medina, Maureen Handoko, James John Riviello
{"title":"Acute Changes in Liver Function Tests During Initiation of Ketogenic Diet","authors":"Akshat Katyayan, Anuranjita Nayak, Gloria Diaz-Medina, Maureen Handoko, James John Riviello","doi":"10.1177/08830738241272063","DOIUrl":"https://doi.org/10.1177/08830738241272063","url":null,"abstract":"BackgroundKetogenic diet is an effective therapy for patients with medically refractory epilepsy. It is generally well tolerated, with the most common side effects being gastrointestinal. Hepatic toxicity has been described as an uncommon side effect of ketogenic diet, usually with long-term use. However, there are limited data to implicate ketogenic diet in acute liver toxicity.Methods and resultsWe analyzed all patients who underwent elective inpatient ketogenic diet initiation at our institution from June 2019 to June 2022. Of the 25 patients reviewed, we found 6 patients who showed acute, asymptomatic changes in liver function tests during initiation, in both hepatocellular and cholestatic patterns. Two patients stopped the ketogenic diet acutely and 3 patients continued ketogenic diet with changes in medications and/or addition of choline—all patients had improvement and normalization of liver function tests in the short term. One patient had acute normalization of chronically elevated liver function tests on ketogenic diet initiation.ConclusionKetogenic diet can cause acute changes in liver function tests during initiation of ketogenic diet, with both hepatocellular and cholestatic patterns, with and without the concurrent use of hepatotoxic medications. In most patients, ketogenic diet can be continued successfully by making changes to medications or addition of choline.","PeriodicalId":15319,"journal":{"name":"Journal of Child Neurology","volume":null,"pages":null},"PeriodicalIF":1.9,"publicationDate":"2024-09-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142262188","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Coral J. Sepulveda, Elaine Walsh, Kristen Carlin, Russell P. Saneto
{"title":"Using a Previsit Questionnaire for Initial Visits in a Pediatric Mitochondrial Clinic: Perspectives of Parents, a Specialty Physician, and a Clinical Coordinator","authors":"Coral J. Sepulveda, Elaine Walsh, Kristen Carlin, Russell P. Saneto","doi":"10.1177/08830738241278388","DOIUrl":"https://doi.org/10.1177/08830738241278388","url":null,"abstract":"Objective: In this study, we assessed the usefulness of a previsit questionnaire for children who were referred for an initial evaluation in a mitochondrial subspecialty clinic. We explored the themes regarding parent's questions, concerns, and goals. We aimed to add to existing knowledge about the usefulness of previsit questionnaires in a pediatric specialty setting from the perspective of parents, the specialist, and the clinical coordinator. Method: We enrolled 25 patients and their parent(s) over 25 months. Questionnaires were completed by the parent(s), the clinical coordinator, and the mitochondrial specialist. Descriptive statistics and thematic analysis were used to summarize results. Results: Parental responses suggested that they are most concerned about their child's clinical problems, communication, language and developmental delays, disease progression and prognosis, understanding mitochondrial disease, quality of life, and physical challenges including muscle and energy problems. Parents felt the previsit questionnaire was very helpful for both the doctor and for themselves to be prepared for their visit. The specialist and the clinical coordinator also found it to be helpful. Parental comments suggested that they felt that writing down the story of their child's life was helpful for the provider, allowed time for reflection, and improved the appointment experience. Some felt it was a difficult or redundant activity. Conclusion: Parents were often pleased to complete the previsit questionnaire. This allowed them to highlight concerns and share information that they wanted the care team to know about their child. We revised the tool based on feedback from parents and the specialist and will continue to use it in our clinic.","PeriodicalId":15319,"journal":{"name":"Journal of Child Neurology","volume":null,"pages":null},"PeriodicalIF":1.9,"publicationDate":"2024-09-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142262185","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Ellen N. Sutter, Janet M. Legare, Melissa A. Villegas, Kellie M. Collins, Jens Eickhoff, Bernadette T. Gillick
{"title":"Evidence-Based Infant Assessment for Cerebral Palsy: Diagnosis Timelines and Intervention Access in a Newborn Follow-up Setting","authors":"Ellen N. Sutter, Janet M. Legare, Melissa A. Villegas, Kellie M. Collins, Jens Eickhoff, Bernadette T. Gillick","doi":"10.1177/08830738241279690","DOIUrl":"https://doi.org/10.1177/08830738241279690","url":null,"abstract":"Evidence-based assessment pathways inform early detection of cerebral palsy and access to intervention. This study investigated the relationships between early evidence-based assessments, diagnosis timeline, and rehabilitation intervention access in a population of children with cerebral palsy who were seen between 2010 and 2022 at the University of Wisconsin Waisman Center Newborn Follow Up Clinic. Cerebral palsy–specific assessments were increasingly integrated after the publication of early detection guidelines by Novak et al. in 2017. Age at cerebral palsy first mention (high risk for cerebral palsy) decreased over time, although age at diagnosis remained similar. Infants who received multiple evidence-based assessments were diagnosed at a younger age. Ninety-nine percent of children were referred to rehabilitation therapies before diagnosis. Infant age at referral to outpatient therapies decreased over time. This study provides novel clinical data on diagnosis timelines and identifies remaining gaps related to implementation feasibility toward improved early diagnosis and intervention access.","PeriodicalId":15319,"journal":{"name":"Journal of Child Neurology","volume":null,"pages":null},"PeriodicalIF":1.9,"publicationDate":"2024-09-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142178929","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}