Pub Date : 2025-11-03DOI: 10.1016/j.pediatrneurol.2025.10.028
Ria Pal MD, Chrisoula Cheronis MD, Elizabeth Mayne MD, PhD, Gary K. Steinberg MD, PhD, Sarah Lee MD
Background
To assess variability in postoperative blood pressure management and its association with stroke incidence in pediatric Moyamoya disease (MMD) patients undergoing surgical revascularization.
Methods
This retrospective cohort study was conducted at Stanford University Medical Center from 1992 to 2023. It included 109 pediatric MMD patients (≤18 years) who underwent revascularization surgery. The study the study evaluated outpatient systolic blood pressures, inpatient mean arterial pressure targets, and the use of vasoactive medications.
Results
Postoperative blood pressure management varied significantly based on patient age, syndrome group, and preoperative stroke status. There was no correlation between preoperative systolic blood pressure and postoperative blood pressure targets. Vasoactive medications were used in 55% of patients intravenously and 53% orally, often for extended durations, but without a clear association with transient neurological symptoms. Major strokes occurred in 6.4% of patients, primarily within the first postoperative week. Stroke incidence was associated with longer durations of vasoactive therapy (IV: 3.0 vs 0.0 days, P = 0.026; oral: 53.0 vs 0.0 days, P = 0.035), but not with specific blood pressure targets.
Conclusions
There is significant variability in postoperative blood pressure management in pediatric MMD, reflecting the absence of standardized guidelines. The increased risk of stroke during the first postoperative week, particularly among patients receiving prolonged vasoactive therapy, underscores the need for prospective studies to establish individualized hemodynamic targets and reduce practice variability.
背景：评估接受外科血运重建术的儿童烟雾病（MMD）患者术后血压管理的变异性及其与卒中发生率的关系。方法：回顾性队列研究于1992年至2023年在斯坦福大学医学中心进行。纳入109例接受血运重建术的儿童烟雾病患者（≤18岁）。这项研究评估了门诊病人的收缩压，住院病人的平均动脉压目标，以及血管活性药物的使用。结果：术后血压管理根据患者年龄、综合征组和术前卒中状态有显著差异。术前收缩压与术后血压指标无相关性。55%的患者静脉注射血管活性药物，53%的患者口服血管活性药物，通常持续时间较长，但与短暂性神经症状无明显关联。6.4%的患者发生严重中风，主要发生在术后第一周。卒中发生率与较长的血管活性治疗持续时间相关（静脉注射：3.0 vs 0.0天，P = 0.026；口服：53.0 vs 0.0天，P = 0.035），但与特定的血压目标无关。结论：儿童烟雾病术后血压管理存在显著差异，反映了标准化指南的缺乏。术后第一周卒中风险的增加，特别是在接受长期血管活性治疗的患者中，强调了前瞻性研究的必要性，以建立个体化的血流动力学目标并减少实践的可变性。
{"title":"Blood Pressure Management and Postoperative Stroke Risk in Pediatric Moyamoya Disease","authors":"Ria Pal MD, Chrisoula Cheronis MD, Elizabeth Mayne MD, PhD, Gary K. Steinberg MD, PhD, Sarah Lee MD","doi":"10.1016/j.pediatrneurol.2025.10.028","DOIUrl":"10.1016/j.pediatrneurol.2025.10.028","url":null,"abstract":"<div><h3>Background</h3><div>To assess variability in postoperative blood pressure management and its association with stroke incidence in pediatric Moyamoya disease (MMD) patients undergoing surgical revascularization.</div></div><div><h3>Methods</h3><div>This retrospective cohort study was conducted at Stanford University Medical Center from 1992 to 2023. It included 109 pediatric MMD patients (≤18 years) who underwent revascularization surgery. The study the study evaluated outpatient systolic blood pressures, inpatient mean arterial pressure targets, and the use of vasoactive medications.</div></div><div><h3>Results</h3><div>Postoperative blood pressure management varied significantly based on patient age, syndrome group, and preoperative stroke status. There was no correlation between preoperative systolic blood pressure and postoperative blood pressure targets. Vasoactive medications were used in 55% of patients intravenously and 53% orally, often for extended durations, but without a clear association with transient neurological symptoms. Major strokes occurred in 6.4% of patients, primarily within the first postoperative week. Stroke incidence was associated with longer durations of vasoactive therapy (IV: 3.0 vs 0.0 days, <em>P</em> = 0.026; oral: 53.0 vs 0.0 days, <em>P</em> = 0.035), but not with specific blood pressure targets.</div></div><div><h3>Conclusions</h3><div>There is significant variability in postoperative blood pressure management in pediatric MMD, reflecting the absence of standardized guidelines. The increased risk of stroke during the first postoperative week, particularly among patients receiving prolonged vasoactive therapy, underscores the need for prospective studies to establish individualized hemodynamic targets and reduce practice variability.</div></div>","PeriodicalId":19956,"journal":{"name":"Pediatric neurology","volume":"175 ","pages":"Pages 19-26"},"PeriodicalIF":2.1,"publicationDate":"2025-11-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145564850","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-11-01DOI: 10.1016/j.pediatrneurol.2025.10.020
S.H. Subramony MD , David R. Lynch MD, PhD
With the introduction of potential new therapy for Friedreich ataxia, the disorder has taken on a new importance in the world of pediatric neurology. Originally described more than 150 years ago, large scale clinical studies have defined diagnostic criteria and the underlying mutation as a biallelic, unstable expansion of an intronic guanine adenine adenine repeat in chromosome 9. In this review, we summarize the clinical features, routine management, pathophysiology, and emerging therapies for this devastating disease. The recent approval of omaveloxolone makes recognition of Friedreich ataxia and its treatment essential for all pediatric neurologists.
{"title":"Friedreich Ataxia","authors":"S.H. Subramony MD , David R. Lynch MD, PhD","doi":"10.1016/j.pediatrneurol.2025.10.020","DOIUrl":"10.1016/j.pediatrneurol.2025.10.020","url":null,"abstract":"<div><div>With the introduction of potential new therapy for Friedreich ataxia, the disorder has taken on a new importance in the world of pediatric neurology. Originally described more than 150 years ago, large scale clinical studies have defined diagnostic criteria and the underlying mutation as a biallelic, unstable expansion of an intronic guanine adenine adenine repeat in chromosome 9. In this review, we summarize the clinical features, routine management, pathophysiology, and emerging therapies for this devastating disease. The recent approval of omaveloxolone makes recognition of Friedreich ataxia and its treatment essential for all pediatric neurologists.</div></div>","PeriodicalId":19956,"journal":{"name":"Pediatric neurology","volume":"174 ","pages":"Pages 148-154"},"PeriodicalIF":2.1,"publicationDate":"2025-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145549983","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-10-31DOI: 10.1016/j.pediatrneurol.2025.10.026
Marian Michael Bercu MD, MSc , Kathryn E. Spykman BSN , Velisa M. Johnson PhD , Angel W. Hernandez MD
Background
Drug-resistant epilepsy (DRE) in the pediatric population is a challenging disease, with limited surgical interventions available. We report the preliminary outcomes for pediatric patients with multifocal DRE epilepsy treated with centromedian responsive neurostimulation.
Methods
A retrospective chart review was conducted in 11 pediatric patients with multifocal epilepsy who were treated with bilateral responsive neurostimulation (RNS) of the centromedian nucleus. All patients were implanted with at least one RNS system; three patients had two RNS systems implanted, with additional leads targeting bilateral cortical or subcortical epileptogenic areas.
Results
Ten out of the 11 patients (90%) treated with RNS (ages 5-20 years old, average 13.73 years old, median 14 years old) were found to have at least a 50% reduction in seizures using at least two modalities of data analysis. The average follow-up time was 1.33 years. Upon reviewing the data from the electronic medical records, 6 patients (55%) experienced a reduction in seizures of 75% or higher, 4 patients (36%) experienced a reduction in seizures between 50 and 74% and 1 patient (9%) was nonresponsive. At least 8 patients (72%) experienced subjective improvements in behavior, interactions, and/or academic performance. This was most pronounced in the subgroup of 4 patients diagnosed with autism spectrum disorder. Ninety percent of patients and families reported improvements in quality of life secondary to neurostimulation. No surgical or stimulation-related complications or side effects were encountered.
Conclusions
The preliminary outcomes suggest a robust response to central neurostimulation in pediatric patients suffering from multifocal DRE, with an excellent safety profile.
{"title":"Preliminary Outcomes of Central Responsive Neurostimulation for Multifocal Epilepsy in Pediatric Patients","authors":"Marian Michael Bercu MD, MSc , Kathryn E. Spykman BSN , Velisa M. Johnson PhD , Angel W. Hernandez MD","doi":"10.1016/j.pediatrneurol.2025.10.026","DOIUrl":"10.1016/j.pediatrneurol.2025.10.026","url":null,"abstract":"<div><h3>Background</h3><div>Drug-resistant epilepsy (DRE) in the pediatric population is a challenging disease, with limited surgical interventions available. We report the preliminary outcomes for pediatric patients with multifocal DRE epilepsy treated with centromedian responsive neurostimulation.</div></div><div><h3>Methods</h3><div>A retrospective chart review was conducted in 11 pediatric patients with multifocal epilepsy who were treated with bilateral responsive neurostimulation (RNS) of the centromedian nucleus. All patients were implanted with at least one RNS system; three patients had two RNS systems implanted, with additional leads targeting bilateral cortical or subcortical epileptogenic areas.</div></div><div><h3>Results</h3><div>Ten out of the 11 patients (90%) treated with RNS (ages 5-20 years old, average 13.73 years old, median 14 years old) were found to have at least a 50% reduction in seizures using at least two modalities of data analysis. The average follow-up time was 1.33 years. Upon reviewing the data from the electronic medical records, 6 patients (55%) experienced a reduction in seizures of 75% or higher, 4 patients (36%) experienced a reduction in seizures between 50 and 74% and 1 patient (9%) was nonresponsive. At least 8 patients (72%) experienced subjective improvements in behavior, interactions, and/or academic performance. This was most pronounced in the subgroup of 4 patients diagnosed with autism spectrum disorder. Ninety percent of patients and families reported improvements in quality of life secondary to neurostimulation. No surgical or stimulation-related complications or side effects were encountered.</div></div><div><h3>Conclusions</h3><div>The preliminary outcomes suggest a robust response to central neurostimulation in pediatric patients suffering from multifocal DRE, with an excellent safety profile.</div></div>","PeriodicalId":19956,"journal":{"name":"Pediatric neurology","volume":"175 ","pages":"Pages 40-49"},"PeriodicalIF":2.1,"publicationDate":"2025-10-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145571168","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-10-31DOI: 10.1016/j.pediatrneurol.2025.10.021
Sasha Taluri BS , Alan K. Percy MD , Amitha L. Ananth MD
There has been remarkable progress made in the understanding of Rett syndrome, with only 57 years between the description of a phenotype and its first FDA-approved treatment. In this review, we will discuss this history and the genetics of Rett syndrome and examine the clinical features of the condition, with particular attention to the progression of symptoms over time. Genotype-phenotype correlations will be reviewed. Clinical management strategies, including specific symptom management and disease modifying therapy, will be discussed. The review will end with a brief discussion of current and future research directions.
{"title":"Living With Rett Syndrome: From Discovery to Clinical Advancements and Emerging Therapies","authors":"Sasha Taluri BS , Alan K. Percy MD , Amitha L. Ananth MD","doi":"10.1016/j.pediatrneurol.2025.10.021","DOIUrl":"10.1016/j.pediatrneurol.2025.10.021","url":null,"abstract":"<div><div>There has been remarkable progress made in the understanding of Rett syndrome, with only 57 years between the description of a phenotype and its first FDA-approved treatment. In this review, we will discuss this history and the genetics of Rett syndrome and examine the clinical features of the condition, with particular attention to the progression of symptoms over time. Genotype-phenotype correlations will be reviewed. Clinical management strategies, including specific symptom management and disease modifying therapy, will be discussed. The review will end with a brief discussion of current and future research directions.</div></div>","PeriodicalId":19956,"journal":{"name":"Pediatric neurology","volume":"175 ","pages":"Pages 67-71"},"PeriodicalIF":2.1,"publicationDate":"2025-10-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145571170","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-10-30DOI: 10.1016/j.pediatrneurol.2025.10.023
Judith S. Miller PhD , Cristan Farmer PhD , Susan Blair MS, PT , Simona Bianconi MD , Natacha Akshoomoff PhD , Irina Anselm MD , Bruce A. Barshop MD, PhD , Lindsey Becker BS , Amanda E. Bennett MD, MPH , Leandra N. Berry PhD , Elizabeth M. Berry-Kravis MD, PhD , Aleksandra Bruchey PhD , Anna W. Byars PhD , Tricia Cimms MPH , Kim M. Cecil PhD , Maxine Covello BS , Laura S. Cubit PhD , Tanvi Das PhD , Robert J. Davis PharmD , Madison Drye BS , Audrey Thurm PhD
Background
The purpose of the Vigilan observational study (ClinicalTrials.gov, NCT02931682) was to prospectively assess the natural history and developmental course of creatine transporter deficiency (CTD).
Methods
Males with CTD aged 6 months to 65 years were evaluated at 6-month intervals for up to 4 years. Evaluations included neurodevelopmental assessments of intellectual functioning, adaptive functioning, and challenging behaviors and the onset and progression of medical comorbidities.
Results
Fifty participants (median age, 7.6 years) were enrolled. The predominant CTD phenotype consisted of significant intellectual disabilities and limited skill development over time. Most participants had a history of febrile or nonfebrile seizures, gastrointestinal symptoms, and growth failure. All participants learned how to walk, 78% developed at least some verbal speech, and 34% communicated using phrases or sentences. Norm-referenced neurodevelopment assessments indicated declining standardized scores over time; however, absolute scores (i.e., age equivalent person ability scores) indicated that developmental gains were slower than average, particularly among older participants. Between-person differences in neurodevelopmental skills as a function of age did not match within-person change, suggesting a cohort effect.
Conclusions
In this cohort, CTD was associated with significant and persistent intellectual disability. The use of absolute metrics from neurodevelopmental tests (e.g., person ability scores) allowed for the quantification of slow, but present, skill development.
{"title":"Longitudinal Characterization of Males With X-Linked Creatine Transporter Deficiency: Final Results of a Multiyear Observational Study","authors":"Judith S. Miller PhD , Cristan Farmer PhD , Susan Blair MS, PT , Simona Bianconi MD , Natacha Akshoomoff PhD , Irina Anselm MD , Bruce A. Barshop MD, PhD , Lindsey Becker BS , Amanda E. Bennett MD, MPH , Leandra N. Berry PhD , Elizabeth M. Berry-Kravis MD, PhD , Aleksandra Bruchey PhD , Anna W. Byars PhD , Tricia Cimms MPH , Kim M. Cecil PhD , Maxine Covello BS , Laura S. Cubit PhD , Tanvi Das PhD , Robert J. Davis PharmD , Madison Drye BS , Audrey Thurm PhD","doi":"10.1016/j.pediatrneurol.2025.10.023","DOIUrl":"10.1016/j.pediatrneurol.2025.10.023","url":null,"abstract":"<div><h3>Background</h3><div>The purpose of the Vigilan observational study (ClinicalTrials.gov, NCT02931682) was to prospectively assess the natural history and developmental course of creatine transporter deficiency (CTD).</div></div><div><h3>Methods</h3><div>Males with CTD aged 6 months to 65 years were evaluated at 6-month intervals for up to 4 years. Evaluations included neurodevelopmental assessments of intellectual functioning, adaptive functioning, and challenging behaviors and the onset and progression of medical comorbidities.</div></div><div><h3>Results</h3><div>Fifty participants (median age, 7.6 years) were enrolled. The predominant CTD phenotype consisted of significant intellectual disabilities and limited skill development over time. Most participants had a history of febrile or nonfebrile seizures, gastrointestinal symptoms, and growth failure. All participants learned how to walk, 78% developed at least some verbal speech, and 34% communicated using phrases or sentences. Norm-referenced neurodevelopment assessments indicated declining standardized scores over time; however, absolute scores (i.e., age equivalent person ability scores) indicated that developmental gains were slower than average, particularly among older participants. Between-person differences in neurodevelopmental skills as a function of age did not match within-person change, suggesting a cohort effect.</div></div><div><h3>Conclusions</h3><div>In this cohort, CTD was associated with significant and persistent intellectual disability. The use of absolute metrics from neurodevelopmental tests (e.g., person ability scores) allowed for the quantification of slow, but present, skill development.</div></div>","PeriodicalId":19956,"journal":{"name":"Pediatric neurology","volume":"175 ","pages":"Pages 8-18"},"PeriodicalIF":2.1,"publicationDate":"2025-10-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145537057","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-10-30DOI: 10.1016/j.pediatrneurol.2025.10.022
Christian Surí-Báez MD, MPH , Han Jun Kim MD , Janice Lester MLS , Robin T. Varughese MD , Sanjeev V. Kothare MD
New onset refractory status epilepticus and febrile infection-related epilepsy syndrome are rare and devastating entities in the pediatric population. While no known therapies have formally been established as the “gold standard” for management of the acute phase, consensus guidelines do establish interleukin therapies such as anakinra and tocilizumab as safe and effective second-line immunotherapeutic options. Despite the use of interleukin therapies, many patients continue to have super refractory status epilepticus. A number of publications (mainly case reports and case series) have described various adjunctive therapies in the management of new onset refractory status epilepticus/febrile infection-related epilepsy syndrome, including neuromodulatory therapies (such as vagal nerve stimulation, deep brain stimulation, and electroconvulsive therapy), surgical resection, noninterleukin immunotherapies (such as intrathecal dexamethasone, intravenous rituximab, and cyclophosphamide), infusions (such as lidocaine and magnesium), and anesthetic agents (such as sevoflurane). Utilizing a modified Preferred Reporting Items for Systematic reviews and Meta-Analyses approach, this narrative review summarizes the effectiveness and safety of second-line immunotherapies such as tocilizumab and anakinra, as well as the various adjunctive third-line therapies that aim to abort seizures and mitigate comorbidities within an intensive care setting, such as prolonged sedation and secondary systemic complications.
{"title":"Beyond Anakinra and Tocilizumab: Additional Adjunctive Therapies in Pediatric New Onset Refractory Status Epilepticus and Febrile Infection-Related Epilepsy Syndrome – A Narrative Review","authors":"Christian Surí-Báez MD, MPH , Han Jun Kim MD , Janice Lester MLS , Robin T. Varughese MD , Sanjeev V. Kothare MD","doi":"10.1016/j.pediatrneurol.2025.10.022","DOIUrl":"10.1016/j.pediatrneurol.2025.10.022","url":null,"abstract":"<div><div>New onset refractory status epilepticus and febrile infection-related epilepsy syndrome are rare and devastating entities in the pediatric population. While no known therapies have formally been established as the “gold standard” for management of the acute phase, consensus guidelines do establish interleukin therapies such as anakinra and tocilizumab as safe and effective second-line immunotherapeutic options. Despite the use of interleukin therapies, many patients continue to have super refractory status epilepticus. A number of publications (mainly case reports and case series) have described various adjunctive therapies in the management of new onset refractory status epilepticus/febrile infection-related epilepsy syndrome, including neuromodulatory therapies (such as vagal nerve stimulation, deep brain stimulation, and electroconvulsive therapy), surgical resection, noninterleukin immunotherapies (such as intrathecal dexamethasone, intravenous rituximab, and cyclophosphamide), infusions (such as lidocaine and magnesium), and anesthetic agents (such as sevoflurane). Utilizing a modified Preferred Reporting Items for Systematic reviews and Meta-Analyses approach, this narrative review summarizes the effectiveness and safety of second-line immunotherapies such as tocilizumab and anakinra, as well as the various adjunctive third-line therapies that aim to abort seizures and mitigate comorbidities within an intensive care setting, such as prolonged sedation and secondary systemic complications.</div></div>","PeriodicalId":19956,"journal":{"name":"Pediatric neurology","volume":"175 ","pages":"Pages 27-39"},"PeriodicalIF":2.1,"publicationDate":"2025-10-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145564700","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-10-25DOI: 10.1016/j.pediatrneurol.2025.10.014
Rachna Vipparla , Barbara Storch PhD , Vitória Penido de Paula MD , Naila Makhani MD, MPH , Areti Vassilopoulos PhD
Background
Children and adolescents with neurological diagnoses are more susceptible to experiencing depressive symptoms than their healthy peers. Research to date has found an increase in depressive symptoms among youth during the COVID-19 pandemic, but the impact on youth with neurological conditions has not been directly evaluated.
Methods
This study utilized retrospective data from individuals (n = 1,312) aged 12-21 years to evaluate changes in self-reported depressive symptoms on the Patient Health Questionnaire (Patient Health Questionnaire 2-item and Patient Health Questionnaire 9-item [PHQ-9]) before and after the onset of the COVID-19 pandemic (between March 2018 and March 2022).
Results
The total sample did not exhibit significant differences in depression scores across Patient Health Questionnaire 2-item and PHQ-9 prepandemic and postpandemic onset. However, a significant difference in nonzero PHQ-9 scores was observed in pediatric patients with epilepsy, indicating a shift from minimal to mild depressive symptoms from prepandemic (Mean = 1.69) to postpandemic (Mean = 6.09) onset (P = 0.040). Additional subgroups, including those with neurodevelopmental, psychological, and chronic pain conditions, displayed notable increases in symptom severity based on nonzero scores.
Conclusions
The findings in this study emphasize the need for increased mental health support within pediatric neurology, with a focus on patients with epilepsy. This future research will help attend to and prevent challenges faced by this group of patients when presented with future environmental and social disruptions analogous to the pandemics.
{"title":"Prepandemic and Postpandemic: How COVID-19 Pandemic Affected Depression Symptoms in Youth With Epilepsy and Other Neurological Conditions","authors":"Rachna Vipparla , Barbara Storch PhD , Vitória Penido de Paula MD , Naila Makhani MD, MPH , Areti Vassilopoulos PhD","doi":"10.1016/j.pediatrneurol.2025.10.014","DOIUrl":"10.1016/j.pediatrneurol.2025.10.014","url":null,"abstract":"<div><h3>Background</h3><div>Children and adolescents with neurological diagnoses are more susceptible to experiencing depressive symptoms than their healthy peers. Research to date has found an increase in depressive symptoms among youth during the COVID-19 pandemic, but the impact on youth with neurological conditions has not been directly evaluated.</div></div><div><h3>Methods</h3><div>This study utilized retrospective data from individuals (<em>n</em> = 1,312) aged 12-21 years to evaluate changes in self-reported depressive symptoms on the Patient Health Questionnaire (Patient Health Questionnaire 2-item and Patient Health Questionnaire 9-item [PHQ-9]) before and after the onset of the COVID-19 pandemic (between March 2018 and March 2022).</div></div><div><h3>Results</h3><div>The total sample did not exhibit significant differences in depression scores across Patient Health Questionnaire 2-item and PHQ-9 prepandemic and postpandemic onset. However, a significant difference in nonzero PHQ-9 scores was observed in pediatric patients with epilepsy, indicating a shift from minimal to mild depressive symptoms from prepandemic (<em>Mean</em> = 1.69) to postpandemic (<em>Mean</em> = 6.09) onset (<em>P</em> = 0.040). Additional subgroups, including those with neurodevelopmental, psychological, and chronic pain conditions, displayed notable increases in symptom severity based on nonzero scores.</div></div><div><h3>Conclusions</h3><div>The findings in this study emphasize the need for increased mental health support within pediatric neurology, with a focus on patients with epilepsy. This future research will help attend to and prevent challenges faced by this group of patients when presented with future environmental and social disruptions analogous to the pandemics.</div></div>","PeriodicalId":19956,"journal":{"name":"Pediatric neurology","volume":"174 ","pages":"Pages 135-139"},"PeriodicalIF":2.1,"publicationDate":"2025-10-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145513432","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Neurological conditions account for more than 20% of disease-related morbidity worldwide. Many of those affected by neurological conditions live in Africa, but there are limited descriptions of their prevalence.
Methods
We conducted a descriptive study of all patients aged 3 months to 18 years who were evaluated in the pediatric neurology clinic between January 2020 and December 2023. The data, collected by review of the patients' medical records by the principal investigator, included age, gender, medical history, examination findings, diagnosis, and treatment. Data were analyzed with the Statistical Package for Social Sciences (version 21) software using descriptive statistics.
Results
During the study period, 4,130 patients had pediatric consultations at Douala Gyneco-Obstetric and Pediatric Hospital, of which 1,111 (26.9%) were seen in the pediatric neurology clinic for evaluation of pediatric neurological disorders. A total of 854 patients met our inclusion criteria and were included in the study. The most common conditions were epilepsy (43.4%), cerebral palsy (31.3%), and autism spectrum disorders (13.9%). Regarding the therapeutic itinerary, most patients opted first for traditional practitioners (49.6%), while a smaller proportion (34.2%) chose conventional medicine as the first treatment option.
Conclusions
Pediatric neurological disorders account for a quarter of pediatric consultations at the Douala Gyneco-Obstetric and Pediatric Hospital, with the most common diagnoses being epilepsy and cerebral palsy. This may help in defining priorities, especially as far as rehabilitation, training and resources allocation are concerned.
{"title":"Clinical Profile of Pediatric Neurological Disorders at the Douala Gyneco-Obstetric and Pediatric Hospital in Cameroon","authors":"Dominique Enyama MD , Diomède Noukeu Njinkui MD , Maturin Florian Kengni Tefeu MD , Jeanne Mayouego Kouam MD , Daniel Armand Kago Tague MD , Arielle Annick Sime Tchouamo MD , Séraphin Nguefack MD","doi":"10.1016/j.pediatrneurol.2025.10.017","DOIUrl":"10.1016/j.pediatrneurol.2025.10.017","url":null,"abstract":"<div><h3>Background</h3><div>Neurological conditions account for more than 20% of disease-related morbidity worldwide. Many of those affected by neurological conditions live in Africa, but there are limited descriptions of their prevalence.</div></div><div><h3>Methods</h3><div>We conducted a descriptive study of all patients aged 3 months to 18 years who were evaluated in the pediatric neurology clinic between January 2020 and December 2023. The data, collected by review of the patients' medical records by the principal investigator, included age, gender, medical history, examination findings, diagnosis, and treatment. Data were analyzed with the Statistical Package for Social Sciences (version 21) software using descriptive statistics.</div></div><div><h3>Results</h3><div>During the study period, 4,130 patients had pediatric consultations at Douala Gyneco-Obstetric and Pediatric Hospital, of which 1,111 (26.9%) were seen in the pediatric neurology clinic for evaluation of pediatric neurological disorders. A total of 854 patients met our inclusion criteria and were included in the study. The most common conditions were epilepsy (43.4%), cerebral palsy (31.3%), and autism spectrum disorders (13.9%). Regarding the therapeutic itinerary, most patients opted first for traditional practitioners (49.6%), while a smaller proportion (34.2%) chose conventional medicine as the first treatment option.</div></div><div><h3>Conclusions</h3><div>Pediatric neurological disorders account for a quarter of pediatric consultations at the Douala Gyneco-Obstetric and Pediatric Hospital, with the most common diagnoses being epilepsy and cerebral palsy. This may help in defining priorities, especially as far as rehabilitation, training and resources allocation are concerned.</div></div>","PeriodicalId":19956,"journal":{"name":"Pediatric neurology","volume":"174 ","pages":"Pages 119-125"},"PeriodicalIF":2.1,"publicationDate":"2025-10-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145513450","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Pub Date : 2025-10-24DOI: 10.1016/j.pediatrneurol.2025.10.013
Elena González-Alguacil MD, J. Jose García Peñas MD, Nuria Lamagrande Casanova MD, Elsa Maria Santana Cabrera MD, Anna Duat Rodríguez PhD, Víctor Soto Insuga PhD
Background
To assess the efficacy and safety of adjunctive cannabidiol (CBD) in children with drug-resistant epileptic spasms (ES) beyond age 2 years.
Methods
We conducted a retrospective longitudinal study of patients with childhood epileptic spasms (CESs) treated with purified CBD (Epidyolex) at Niño Jesus Hospital in Madrid, Spain, from 2020 to 2024. All patients were older than 2 years and had drug-resistant epilepsy and ES as the primary seizure type. Efficacy was assessed by comparing ES frequency before and after CBD treatment, with a good response defined as a ≥50% reduction in ES. Adverse effects (AEs) were recorded to assess safety and tolerability.
Results
Of the 53 patients included, 58.5% achieved a ≥50% reduction in ES frequency, 15% of whom attained complete freedom from ES. Patients with malformations of cortical development and Down syndrome showed the highest response rates. Clobazam was the most frequently associated medication (77.3%), indicating a potential synergistic effect. AEs were reported in 62.2% of patients, with drowsiness, hyperammonemia, and behavioral disturbances being the most common. CBD was discontinued in 17% due to lack of efficacy and in 11.3% due to AEs.
Conclusions
CBD appears to be an effective and well-tolerated adjunctive treatment for drug-resistant ES in children older than 2 years. Significant response rates were found, particularly in patients with Down syndrome and malformations of cortical development. Future studies with larger cohorts and longer follow-up are needed to validate these findings and explore the potential for earlier use of CBD in treatment-resistant epilepsy.
{"title":"Cannabidiol as Adjunctive Treatment in Drug-Resistant Epilepsy With Epileptic Spasms Beyond Two Years of Age","authors":"Elena González-Alguacil MD, J. Jose García Peñas MD, Nuria Lamagrande Casanova MD, Elsa Maria Santana Cabrera MD, Anna Duat Rodríguez PhD, Víctor Soto Insuga PhD","doi":"10.1016/j.pediatrneurol.2025.10.013","DOIUrl":"10.1016/j.pediatrneurol.2025.10.013","url":null,"abstract":"<div><h3>Background</h3><div>To assess the efficacy and safety of adjunctive cannabidiol (CBD) in children with drug-resistant epileptic spasms (ES) beyond age 2 years.</div></div><div><h3>Methods</h3><div>We conducted a retrospective longitudinal study of patients with childhood epileptic spasms (CESs) treated with purified CBD (Epidyolex) at Niño Jesus Hospital in Madrid, Spain, from 2020 to 2024. All patients were older than 2 years and had drug-resistant epilepsy and ES as the primary seizure type. Efficacy was assessed by comparing ES frequency before and after CBD treatment, with a good response defined as a ≥50% reduction in ES. Adverse effects (AEs) were recorded to assess safety and tolerability.</div></div><div><h3>Results</h3><div>Of the 53 patients included, 58.5% achieved a ≥50% reduction in ES frequency, 15% of whom attained complete freedom from ES. Patients with malformations of cortical development and Down syndrome showed the highest response rates. Clobazam was the most frequently associated medication (77.3%), indicating a potential synergistic effect. AEs were reported in 62.2% of patients, with drowsiness, hyperammonemia, and behavioral disturbances being the most common. CBD was discontinued in 17% due to lack of efficacy and in 11.3% due to AEs.</div></div><div><h3>Conclusions</h3><div>CBD appears to be an effective and well-tolerated adjunctive treatment for drug-resistant ES in children older than 2 years. Significant response rates were found, particularly in patients with Down syndrome and malformations of cortical development. Future studies with larger cohorts and longer follow-up are needed to validate these findings and explore the potential for earlier use of CBD in treatment-resistant epilepsy.</div></div>","PeriodicalId":19956,"journal":{"name":"Pediatric neurology","volume":"174 ","pages":"Pages 81-85"},"PeriodicalIF":2.1,"publicationDate":"2025-10-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145459190","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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