Twenty five percent of children who sustain a mild traumatic brain injury (mTBI) or concussion experience persisting post-concussion symptoms (PPCS). The symptoms of PPCS significantly overlap with postural orthostatic tachycardia syndrome (POTS). We aimed to review the literature and investigate the prevalence of POTS in children and adolescents recovering from mTBI.
Methods
This single centre controlled cross-sectional cohort study recruited children (aged 8-18 years inclusive) diagnosed with mTBI in the emergency department. At 4-6 weeks postinjury, children completed the Active Stand Test. The primary outcome was change in heart rate alongside clinical symptoms consistent with POTS.
Results
A total of 113 participants (64 PPCS, 24 mTBI with no clinical symptoms [asymptomatic], and 25 healthy controls) of similar age (mean 13.9 years) and sex (n = 55 [48.7%] male) underwent orthostatic testing. There were no significant group differences in heart rate or blood pressure at baseline, or within 10 minutes of standing. Three (4.8%) symptomatic participants satisfied POTS diagnostic criteria, compared to 0 asymptomatic and one (4.2%) control participant.
Conclusions
Using the Active Stand Test, a small proportion of children with PPCS satisfied clinical POTS criteria. Although it should be considered in the differential diagnosis, POTS is not common nor overrepresented in PPCS.
{"title":"Orthostatic Tachycardia in Children With and Without Persisting Post-concussion Symptoms Following Mild Traumatic Brain Injury: A Prospective Controlled Study","authors":"Athena Stein PhD, MPH , Karen M. Barlow PhD, MSc, MBChB, MRCPCH","doi":"10.1016/j.pediatrneurol.2025.11.001","DOIUrl":"10.1016/j.pediatrneurol.2025.11.001","url":null,"abstract":"<div><h3>Background</h3><div>Twenty five percent of children who sustain a mild traumatic brain injury (mTBI) or concussion experience persisting post-concussion symptoms (PPCS). The symptoms of PPCS significantly overlap with postural orthostatic tachycardia syndrome (POTS). We aimed to review the literature and investigate the prevalence of POTS in children and adolescents recovering from mTBI.</div></div><div><h3>Methods</h3><div>This single centre controlled cross-sectional cohort study recruited children (aged 8-18 years inclusive) diagnosed with mTBI in the emergency department. At 4-6 weeks postinjury, children completed the Active Stand Test. The primary outcome was change in heart rate alongside clinical symptoms consistent with POTS.</div></div><div><h3>Results</h3><div>A total of 113 participants (64 PPCS, 24 mTBI with no clinical symptoms [asymptomatic], and 25 healthy controls) of similar age (mean 13.9 years) and sex (n = 55 [48.7%] male) underwent orthostatic testing. There were no significant group differences in heart rate or blood pressure at baseline, or within 10 minutes of standing. Three (4.8%) symptomatic participants satisfied POTS diagnostic criteria, compared to 0 asymptomatic and one (4.2%) control participant.</div></div><div><h3>Conclusions</h3><div>Using the Active Stand Test, a small proportion of children with PPCS satisfied clinical POTS criteria. Although it should be considered in the differential diagnosis, POTS is not common nor overrepresented in PPCS.</div></div>","PeriodicalId":19956,"journal":{"name":"Pediatric neurology","volume":"175 ","pages":"Pages 72-80"},"PeriodicalIF":2.1,"publicationDate":"2025-11-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145605627","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 : 2025-11-07DOI: 10.1016/j.pediatrneurol.2025.11.002
Marie E. Clements MD , Christina Briscoe MD, EdM , Angela M. Curcio MD , Laurie M. Douglass MD , Janani Kassiri MD, PhD , Lynn C. Liu MD, MS , Sally V. Mathias MD , Debra T. Moore-Hill MD, MPH , Deepthi Nalluri MD , Audrey Nath MD, PhD , Amanda M. Romeu DO , Tahniat Syed MD, MPH , Victoria Vinarsky DO , Susan Wiener MD, MPH , Qian-Zhou JoJo Yang MD , Sarah J. Betstadt MD, MPH , Elizabeth I. Harrison MD, MS , Neil Kulkarni MD , Preeti Puntambekar MD , Debopam Samanta MD, MS , Laura Kirkpatrick MD
Epilepsy in the Child-Bearing Ages through Menopause is an international consortium of clinicians dedicated to improving the health of women with epilepsy across the lifespan. Epilepsy in the Child-Bearing Ages through Menopause's Adolescent and Young Adult Committee addresses quality of care for female youth with epilepsy. The Committee developed a literature review and expert opinion guidance for child neurologists on performing optimal counseling about epilepsy and reproductive health for female youth with epilepsy. To do so, we identified and voted on key topics essential for this counseling, then conducted comprehensive literature reviews for each topic, iteratively developed key statements about counseling content and style for each topic and voted on final content for inclusion. The included topics were teratogenesis, folic acid supplementation, pregnancy and fertility, contraception, heritability of epilepsy, menstrual and hormonal disorders, catamenial epilepsy, and taking a sexual history. This review provides a clear, novel framework for pediatric neurologists to counsel adolescent and young adult women with epilepsy about their reproductive health, supporting improvement in practices recommended by professional organization such as the American Academy of Neurology and Child Neurology Foundation.
{"title":"Counseling Reproductive-Age Youth With Epilepsy: Literature Review and Expert Opinion From the Epilepsy in the Child-Bearing Ages Through Menopause Consortium","authors":"Marie E. Clements MD , Christina Briscoe MD, EdM , Angela M. Curcio MD , Laurie M. Douglass MD , Janani Kassiri MD, PhD , Lynn C. Liu MD, MS , Sally V. Mathias MD , Debra T. Moore-Hill MD, MPH , Deepthi Nalluri MD , Audrey Nath MD, PhD , Amanda M. Romeu DO , Tahniat Syed MD, MPH , Victoria Vinarsky DO , Susan Wiener MD, MPH , Qian-Zhou JoJo Yang MD , Sarah J. Betstadt MD, MPH , Elizabeth I. Harrison MD, MS , Neil Kulkarni MD , Preeti Puntambekar MD , Debopam Samanta MD, MS , Laura Kirkpatrick MD","doi":"10.1016/j.pediatrneurol.2025.11.002","DOIUrl":"10.1016/j.pediatrneurol.2025.11.002","url":null,"abstract":"<div><div>Epilepsy in the Child-Bearing Ages through Menopause is an international consortium of clinicians dedicated to improving the health of women with epilepsy across the lifespan. Epilepsy in the Child-Bearing Ages through Menopause's Adolescent and Young Adult Committee addresses quality of care for female youth with epilepsy. The Committee developed a literature review and expert opinion guidance for child neurologists on performing optimal counseling about epilepsy and reproductive health for female youth with epilepsy. To do so, we identified and voted on key topics essential for this counseling, then conducted comprehensive literature reviews for each topic, iteratively developed key statements about counseling content and style for each topic and voted on final content for inclusion. The included topics were teratogenesis, folic acid supplementation, pregnancy and fertility, contraception, heritability of epilepsy, menstrual and hormonal disorders, catamenial epilepsy, and taking a sexual history. This review provides a clear, novel framework for pediatric neurologists to counsel adolescent and young adult women with epilepsy about their reproductive health, supporting improvement in practices recommended by professional organization such as the American Academy of Neurology and Child Neurology Foundation.</div></div>","PeriodicalId":19956,"journal":{"name":"Pediatric neurology","volume":"175 ","pages":"Pages 90-101"},"PeriodicalIF":2.1,"publicationDate":"2025-11-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145616289","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 : 2025-11-05DOI: 10.1016/j.pediatrneurol.2025.10.027
Anna Sosa BS , Kimberly A. O'Neill MD , Thibo Billiet PhD , Jingyun Chen PhD , Laura Couvreur MD , Stijn Denissen PhD , Matthew Lustberg MA , Shayna Pehel BS , Annemie Ribbens PhD , Lauren Krupp MD
Background
While cognitive processing and brain volume assessments are commonly used in multiple sclerosis (MS) research, they are not routinely incorporated into clinical care. Advances in neuroimaging software now allow for brain volume measurement to be incorporated into standard practice and guide clinical decision-making.
Methods
In this single-center, outpatient, cohort study, pediatric-onset multiple sclerosis (POMS) patients underwent at least two volumetric brain magnetic resonance imagings (MRIs) and two Symbol Digit Modalities Tests (SDMT) at least 6 months apart. Associations were analyzed using Pearson's correlation coefficient and linear regressions.
Results
Forty patients with POMS were included. The first volumetric brain MRI occurred at a median of 2.7 years after symptom onset. At the first volumetric MRI, 12% of patients had white matter brain volumes within 1% of normative values (n = 2000 healthy controls). Whole brain volume percentile (r = 0.4, P = 0.01), white matter percentile (r = 0.4, P = 0.01), and white matter lesion volume (r = −0.4, P = 0.005) were associated with SDMT score. Annualized percent brain volume change of the hippocampus (measured a median of 1.6 years apart) significantly correlated with follow-up SDMT (r = 0.4, P = 0.02), and annualized percent brain volume change of the thalamus correlated with annual change in SDMT z-score (r = 0.4, P = 0.009).
Conclusions
Application of new software allows for volumetric assessment to be incorporated into clinical scans and provides clinicians with added data for patient management. This is critical for patients with POMS as the neurological examination often shows few to no abnormalities. Furthermore, a subset of POMS patients have smaller than expected brain volume which links to subsequent poorer cognitive performance.
虽然认知处理和脑容量评估通常用于多发性硬化症（MS）研究，但它们并未常规纳入临床护理。神经成像软件的进步现在允许脑容量测量纳入标准实践并指导临床决策。方法在这项单中心、门诊、队列研究中，儿科发病多发性硬化症（POMS）患者至少间隔6个月接受了两次脑容量磁共振成像（mri）和两次符号数字模式测试（SDMT）。使用Pearson相关系数和线性回归分析相关性。结果共纳入40例POMS患者。第一次脑容量MRI在症状出现后的中位时间为2.7年。在第一次体积MRI中，12%的患者的白质脑体积在正常值的1%以内（n = 2000名健康对照）。全脑体积百分位数（r = 0.4, P = 0.01）、白质百分位数（r = 0.4, P = 0.01）、白质病变体积（r = - 0.4, P = 0.005）与SDMT评分相关。海马年化脑容量变化百分比（测量中位数间隔1.6年）与随访SDMT显著相关（r = 0.4, P = 0.02），丘脑年化脑容量变化百分比与SDMT z-score年化变化相关（r = 0.4, P = 0.009）。新软件的应用允许容积评估纳入临床扫描，并为临床医生提供患者管理的额外数据。这对POMS患者至关重要，因为神经学检查通常显示很少或没有异常。此外，一部分POMS患者的脑容量小于预期，这与随后较差的认知表现有关。
{"title":"Incorporating Measures of Cognitive Processing Speed and Brain Volume in Clinical Management of Pediatric Onset Multiple Sclerosis","authors":"Anna Sosa BS , Kimberly A. O'Neill MD , Thibo Billiet PhD , Jingyun Chen PhD , Laura Couvreur MD , Stijn Denissen PhD , Matthew Lustberg MA , Shayna Pehel BS , Annemie Ribbens PhD , Lauren Krupp MD","doi":"10.1016/j.pediatrneurol.2025.10.027","DOIUrl":"10.1016/j.pediatrneurol.2025.10.027","url":null,"abstract":"<div><h3>Background</h3><div>While cognitive processing and brain volume assessments are commonly used in multiple sclerosis (MS) research, they are not routinely incorporated into clinical care. Advances in neuroimaging software now allow for brain volume measurement to be incorporated into standard practice and guide clinical decision-making.</div></div><div><h3>Methods</h3><div>In this single-center, outpatient, cohort study, pediatric-onset multiple sclerosis (POMS) patients underwent at least two volumetric brain magnetic resonance imagings (MRIs) and two Symbol Digit Modalities Tests (SDMT) at least 6 months apart. Associations were analyzed using Pearson's correlation coefficient and linear regressions.</div></div><div><h3>Results</h3><div>Forty patients with POMS were included. The first volumetric brain MRI occurred at a median of 2.7 years after symptom onset. At the first volumetric MRI, 12% of patients had white matter brain volumes within 1% of normative values (n = 2000 healthy controls). Whole brain volume percentile (r = 0.4, <em>P</em> = 0.01), white matter percentile (r = 0.4, <em>P</em> = 0.01), and white matter lesion volume (r = −0.4, <em>P</em> = 0.005) were associated with SDMT score. Annualized percent brain volume change of the hippocampus (measured a median of 1.6 years apart) significantly correlated with follow-up SDMT (r = 0.4, <em>P</em> = 0.02), and annualized percent brain volume change of the thalamus correlated with annual change in SDMT z-score (r = 0.4, <em>P</em> = 0.009).</div></div><div><h3>Conclusions</h3><div>Application of new software allows for volumetric assessment to be incorporated into clinical scans and provides clinicians with added data for patient management. This is critical for patients with POMS as the neurological examination often shows few to no abnormalities. Furthermore, a subset of POMS patients have smaller than expected brain volume which links to subsequent poorer cognitive performance.</div></div>","PeriodicalId":19956,"journal":{"name":"Pediatric neurology","volume":"175 ","pages":"Pages 50-57"},"PeriodicalIF":2.1,"publicationDate":"2025-11-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145571169","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 : 2025-11-04DOI: 10.1016/j.pediatrneurol.2025.10.030
Miles Fisher DO , Kristen Bolte DO , Shilpa B. Reddy MD , Mark Rodeghier PhD , Lori C. Jordan MD, PhD
Background
To identify predictors of drug-resistant epilepsy (DRE) in children with a history of perinatal ischemic stroke (PIS).
Methods
This single-center retrospective observational study analyzed children with PIS using international classification of diseases, ninth revision (ICD-9) codes, institutional databases, medical records, and neuroimaging from 2012 to 2023. DRE was defined as seizures unresponsive to ≥2 antiseizure medications. The Pediatric Stroke Outcome Measure (PSOM) was retrospectively scored.
Results
Of 96 children with PIS, 56% developed epilepsy and 20 (21%) had DRE. Median age at the last visit was 7.9 years (interquartile range, 3.1-11.7 years.) Among those with DRE, 70% had presumed perinatal stroke and 30% had symptomatic neonatal stroke. PSOM scores differed by epilepsy status: median PSOM was 2.5 for DRE, 1.8 for non-DRE epilepsy and 1.0 for children without epilepsy; paired comparisons for neurological outcome found a difference between those with DRE compared to those without epilepsy (P < 0.001). Hippocampal volume reduction was the only predictor of DRE (odds ratio 6.45, 95% confidence interval 1.80-23.16, P = 0.004) in a multivariable model including sex, neonatal seizures, and total PSOM. Children with DRE tried a median of four antiseizure medications after the newborn period, and 13 (65%) underwent epilepsy surgery. Favorable outcomes (seizure-free or >90% reduction) were seen in 62% postsurgery, including three focal resections, four functional hemispherectomies, and one posterior quadrant disconnection.
Conclusions
Hippocampal volume reduction is a strong predictor of DRE following PIS. Epilepsy and DRE were more common in older children. Hemispherectomy and focal resections were associated with favorable seizure outcomes.
{"title":"Predictors of Drug-Resistant Epilepsy After Perinatal Stroke","authors":"Miles Fisher DO , Kristen Bolte DO , Shilpa B. Reddy MD , Mark Rodeghier PhD , Lori C. Jordan MD, PhD","doi":"10.1016/j.pediatrneurol.2025.10.030","DOIUrl":"10.1016/j.pediatrneurol.2025.10.030","url":null,"abstract":"<div><h3>Background</h3><div>To identify predictors of drug-resistant epilepsy (DRE) in children with a history of perinatal ischemic stroke (PIS).</div></div><div><h3>Methods</h3><div>This single-center retrospective observational study analyzed children with PIS using international classification of diseases, ninth revision (ICD-9) codes, institutional databases, medical records, and neuroimaging from 2012 to 2023. DRE was defined as seizures unresponsive to ≥2 antiseizure medications. The Pediatric Stroke Outcome Measure (PSOM) was retrospectively scored.</div></div><div><h3>Results</h3><div>Of 96 children with PIS, 56% developed epilepsy and 20 (21%) had DRE. Median age at the last visit was 7.9 years (interquartile range, 3.1-11.7 years.) Among those with DRE, 70% had presumed perinatal stroke and 30% had symptomatic neonatal stroke. PSOM scores differed by epilepsy status: median PSOM was 2.5 for DRE, 1.8 for non-DRE epilepsy and 1.0 for children without epilepsy; paired comparisons for neurological outcome found a difference between those with DRE compared to those without epilepsy (<em>P</em> < 0.001). Hippocampal volume reduction was the only predictor of DRE (odds ratio 6.45, 95% confidence interval 1.80-23.16, <em>P</em> = 0.004) in a multivariable model including sex, neonatal seizures, and total PSOM. Children with DRE tried a median of four antiseizure medications after the newborn period, and 13 (65%) underwent epilepsy surgery. Favorable outcomes (seizure-free or >90% reduction) were seen in 62% postsurgery, including three focal resections, four functional hemispherectomies, and one posterior quadrant disconnection.</div></div><div><h3>Conclusions</h3><div>Hippocampal volume reduction is a strong predictor of DRE following PIS. Epilepsy and DRE were more common in older children. Hemispherectomy and focal resections were associated with favorable seizure outcomes.</div></div>","PeriodicalId":19956,"journal":{"name":"Pediatric neurology","volume":"175 ","pages":"Pages 1-7"},"PeriodicalIF":2.1,"publicationDate":"2025-11-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145537056","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 : 2025-11-04DOI: 10.1016/j.pediatrneurol.2025.10.029
Lakshmi Balaji DNB , Didu S. Kariyawasam PhD , Karen Herbert DPT , Hugo A. Sampaio MBBS , Esther Tantsis PhD , Michelle A. Farrar PhD
Background
The use of disease modifying therapies has altered the natural history of spinal muscular atrophy (SMA) leading to changing needs and recognition of multisystem involvement, including cognitive processes. We aimed to investigate the prevalence of, and characterize the behavioral and emotional profiles of children/young people with symptomatic SMA.
Methods
This single-centre, cross-sectional study of children 4-17 years with symptomatic SMA, assessed emotional and behavioral problems using Strengths and Difficulties Questionnaire (SDQ). Clinical characteristics and parent- and child/young person-reported outcomes (Pediatric Quality of Life neuromuscular module) specific to a neuromuscular disorder were also collated. Fisher's exact, Kruskal-Wallis, and regression tests were used for analyses.
Results
Forty-eight children were enrolled (median age [interquartile range]: 7.8 years [5.4-11.4]). Total SDQ scores identified difficulties in 17/48 (35.4%) children with SMA, compared to population frequency of 10%; 16/4 8 (33.3%) parents perceived that their child's emotional/behavioral difficulties were burden on the family, which were chronic for 9/16 (56.3%) and substantial for 10/16 (62.5%), interfering with child's everyday life. Difficulties within at least one domain of the SDQ were identified in 29/48 (60.4%). Of the cohort, 12/48(25%) had difficulties in the domains of hyperactivity, emotional regulation and conduct. For those with abnormal SDQ scores, there was significant association with lower total Pediatric Quality of Life neuromuscular module scores (odds ratio: 1.09, 95% confidence intervals: 1.02, 1.16, P = 0.009).
Conclusions
The study found clinically significant level of emotional and behavioral dysregulation in children/young people with SMA in all categories of motor function, and with negative impact on everyday life. In the context of changing phenotypes and function with treatment, these were evident across varying severities of motor function. These findings support routine mental health surveillance as a means of early identification and intervention, alongside the provision of psychological support to optimize health outcomes.
{"title":"Behavioral and Emotional Challenges in Children With Spinal Muscular Atrophy","authors":"Lakshmi Balaji DNB , Didu S. Kariyawasam PhD , Karen Herbert DPT , Hugo A. Sampaio MBBS , Esther Tantsis PhD , Michelle A. Farrar PhD","doi":"10.1016/j.pediatrneurol.2025.10.029","DOIUrl":"10.1016/j.pediatrneurol.2025.10.029","url":null,"abstract":"<div><h3>Background</h3><div>The use of disease modifying therapies has altered the natural history of spinal muscular atrophy (SMA) leading to changing needs and recognition of multisystem involvement, including cognitive processes. We aimed to investigate the prevalence of, and characterize the behavioral and emotional profiles of children/young people with symptomatic SMA.</div></div><div><h3>Methods</h3><div>This single-centre, cross-sectional study of children 4-17 years with symptomatic SMA, assessed emotional and behavioral problems using Strengths and Difficulties Questionnaire (SDQ). Clinical characteristics and parent- and child/young person-reported outcomes (Pediatric Quality of Life neuromuscular module) specific to a neuromuscular disorder were also collated. Fisher's exact, Kruskal-Wallis, and regression tests were used for analyses.</div></div><div><h3>Results</h3><div>Forty-eight children were enrolled (median age [interquartile range]: 7.8 years [5.4-11.4]). Total SDQ scores identified difficulties in 17/48 (35.4%) children with SMA, compared to population frequency of 10%; 16/4 8 (33.3%) parents perceived that their child's emotional/behavioral difficulties were burden on the family, which were chronic for 9/16 (56.3%) and substantial for 10/16 (62.5%), interfering with child's everyday life. Difficulties within at least one domain of the SDQ were identified in 29/48 (60.4%). Of the cohort, 12/48(25%) had difficulties in the domains of hyperactivity, emotional regulation and conduct. For those with abnormal SDQ scores, there was significant association with lower total Pediatric Quality of Life neuromuscular module scores (odds ratio: 1.09, 95% confidence intervals: 1.02, 1.16, <em>P</em> = 0.009).</div></div><div><h3>Conclusions</h3><div>The study found clinically significant level of emotional and behavioral dysregulation in children/young people with SMA in all categories of motor function, and with negative impact on everyday life. In the context of changing phenotypes and function with treatment, these were evident across varying severities of motor function. These findings support routine mental health surveillance as a means of early identification and intervention, alongside the provision of psychological support to optimize health outcomes.</div></div>","PeriodicalId":19956,"journal":{"name":"Pediatric neurology","volume":"175 ","pages":"Pages 81-87"},"PeriodicalIF":2.1,"publicationDate":"2025-11-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145605636","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 : 2025-11-04DOI: 10.1016/j.pediatrneurol.2025.10.025
Ann Biehl PharmD, MS, Lionel Van Holle MS, Nayla Chaijale PhD, Rebecca Zhang Roper MD, PhD
{"title":"Reply to Chen et al. “A Postmarketing Pharmacovigilance Study of Fenfluramine: Adverse Event Data Mining and Analysis Based on the US Food and Drug Administration Public Data Open Project (openFDA)”","authors":"Ann Biehl PharmD, MS, Lionel Van Holle MS, Nayla Chaijale PhD, Rebecca Zhang Roper MD, PhD","doi":"10.1016/j.pediatrneurol.2025.10.025","DOIUrl":"10.1016/j.pediatrneurol.2025.10.025","url":null,"abstract":"","PeriodicalId":19956,"journal":{"name":"Pediatric neurology","volume":"175 ","pages":"Pages 88-89"},"PeriodicalIF":2.1,"publicationDate":"2025-11-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145616288","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 : 2025-11-03DOI: 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}
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