Pediatric neurology最新文献

{"title":"The Impact of Social Determinants of Health on Quality of Life in a Cohort of 1300 Children With Epilepsy","authors":"Christopher W. Beatty MD ,&nbsp;Stephanie M. Ahrens DO ,&nbsp;Kristen H. Arredondo MD ,&nbsp;Jaime D. Twanow MD ,&nbsp;Ammar Shaikhouni MD, PhD ,&nbsp;Mariah Eisner MS ,&nbsp;Nancy Auer FNP ,&nbsp;Shivani Bhatnagar DNP ,&nbsp;Adam P. Ostendorf MD","doi":"10.1016/j.pediatrneurol.2025.08.004","DOIUrl":"10.1016/j.pediatrneurol.2025.08.004","url":null,"abstract":"<div><h3>Background</h3><div>Health-related quality of life (HRQoL) in children with epilepsy is a measure of the impact of epilepsy on mental and physical health. Although epilepsy features, treatments, and developmental/learning issues impact HRQoL, less data exist on how social, non–disease-related factors play a role. This retrospective observational study examines the association of social determinants of health (SDOH) with HRQoL in children with epilepsy.</div></div><div><h3>Methods</h3><div>Pediatric Quality of Life Inventory Epilepsy Module (PedsQLEp) scores were collected from 1311 children with epilepsy over one-year. Clinical characteristics and SDOH were fit to multivariable linear regression models to identify associations.</div></div><div><h3>Results</h3><div>Cognition and Executive domains of the PedsQLEp had the lowest median scores of 50 and 54, respectively. Both had the largest association with developmental/learning issues (Cognition, β = −32; 95% confidence interval [CI], −35 to −30; Executive β = −19; 95% CI, −22 to −16). Impact had a strong association with seizure burden and antiseizure medication (ASM) side effects. Mood and Sleep were most associated with ASM side effects. SDOH, including public insurance and increased social needs, were associated with lower HRQoL for Cognition, Executive, Impact, and Mood domains but to a lesser degree than other factors.</div></div><div><h3>Conclusions</h3><div>In children with epilepsy, SDOH were associated with HRQoL, but to a lesser degree than epilepsy-related factors. Higher seizure burden, frequent ASM side effects, and developmental/learning issues had the largest association with lower HRQoL. This study highlights areas for intervention to improve HRQoL and indicates that HRQoL may not be a useful outcome measure to assess the impact of an intervention on SDOH.</div></div>","PeriodicalId":19956,"journal":{"name":"Pediatric neurology","volume":"172 ","pages":"Pages 63-70"},"PeriodicalIF":2.1,"publicationDate":"2025-08-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144921182","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}

{"title":"Preferences of Adolescents and Young Adults With Epilepsy and Caregivers on Reproductive Health Counseling by Neurologists: A Concept Mapping Study","authors":"Laura Kirkpatrick MD ,&nbsp;Erin Friel BPhil ,&nbsp;Jasmin Rivero-Guerra ,&nbsp;Amy Tao ,&nbsp;Janani Kassiri MD, PhD ,&nbsp;Marie Clements MD ,&nbsp;Christina Briscoe MD, MEd ,&nbsp;Page B. Pennell MD ,&nbsp;Jessica G. Burke PhD, MHS ,&nbsp;Sara Baumann PhD, MPH ,&nbsp;Traci M. Kazmerski MD, MS","doi":"10.1016/j.pediatrneurol.2025.08.002","DOIUrl":"10.1016/j.pediatrneurol.2025.08.002","url":null,"abstract":"<div><h3>Objective</h3><div>To ascertain reproductive health counseling priorities of adolescent and young adult women with epilepsy (AWWE) and caregivers during neurology visits.</div></div><div><h3>Methods</h3><div>We recruited AWWE aged 14-26 years and caregivers from institutional neurology clinics, a research registry, and epilepsy listservs for a Concept Mapping study. Participants (1) brainstormed topics important for counseling of AWWE about reproductive health, (2) sorted topics into categories and rated their importance (on a five-point Likert scale) for AWWE aged 14-17 and 18-26 years, and (3) met to interpret study findings. We included a small subset of the participants in the interpretation meeting to allow meaningful discussion.</div></div><div><h3>Results</h3><div>Thirty-four AWWE and 20 caregivers generated 37 topics, which were sorted/rated by 35 AWWE and 23 caregivers; seven AWWE and nine caregivers attended the interpretation meeting. Consensus categories included “Hormonal Changes,” “Contraception,” “Sex &amp; Epilepsy,” “Preparing for Pregnancy,” “Pregnancy with Epilepsy,” and “Parenthood &amp; Epilepsy.” For ages 14-17 years, categories rated at least 4.00 for importance included “Hormonal Changes,” “Contraception,” “Sex and Epilepsy,” and “Preparing for Pregnancy.” For ages 18-26 years, all categories were rated at least 4.00. In the interpretation meeting, participants proposed a previsit checklist tool to indicate topics of interest.</div></div><div><h3>Conclusions</h3><div>AWWE want counseling about reproductive health from neurologists that is tailored by age and more comprehensive than current American Academy of Neurology recommendations. Use of a previsit checklist tool may help identify individual patient and family counseling priorities.</div></div>","PeriodicalId":19956,"journal":{"name":"Pediatric neurology","volume":"172 ","pages":"Pages 35-45"},"PeriodicalIF":2.1,"publicationDate":"2025-08-11","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144912317","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}

{"title":"Baclofen and Botulinum Toxin A Use in Tone Management for Children With Cerebral Palsy: A Review of Current Literature and Research Gaps in Pre- and Perioperative Care","authors":"Michael Li BSc ,&nbsp;Naama Rozen MSc ,&nbsp;Kate Wortley MD ,&nbsp;Ram Mishaal MD","doi":"10.1016/j.pediatrneurol.2025.07.018","DOIUrl":"10.1016/j.pediatrneurol.2025.07.018","url":null,"abstract":"<div><div>Cerebral palsy (CP) is a leading cause of motor disability in children. Many children with CP have hypertonia, and some will require orthopedic surgery. Botulinum toxin A (BoNT-A), a muscle relaxant, is commonly pre- or perioperatively injected to improve surgical outcomes and reduce postoperative pain and muscle tone. However, inconsistent evidence supporting its efficacy, potential research bias from industry sponsorship, and numerous adverse effects, such as long-term changes to muscle morphology, highlight the need for a better alternative. Preoperatively increasing the dose of oral baclofen, a first-line treatment for generalized CP-related hypertonia, may improve surgical outcomes with fewer long-term adverse effects. To date, the impact of an increased oral baclofen dose for this purpose has not yet been studied. This article reviews the current evidence on the effectiveness and safety of the three more commonly used antispastic treatments, BoNT-A, oral baclofen, and intrathecal baclofen, with the goal of trialing increased oral baclofen dose as an adjunct to surgery.</div></div>","PeriodicalId":19956,"journal":{"name":"Pediatric neurology","volume":"172 ","pages":"Pages 28-34"},"PeriodicalIF":2.1,"publicationDate":"2025-08-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144912320","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}

{"title":"Electroencephalographic Functional Connectivity Patterns in Children With Tourette Syndrome and Attention-Deficit/Hyperactivity Disorder","authors":"Simon Morand-Beaulieu PhD ,&nbsp;Julia Zhong MS ,&nbsp;Karim Ibrahim PsyD ,&nbsp;Michael J. Crowley PhD ,&nbsp;Heidi Grantz LCSW ,&nbsp;James F. Leckman MD, PhD ,&nbsp;Denis G. Sukhodolsky PhD","doi":"10.1016/j.pediatrneurol.2025.08.001","DOIUrl":"10.1016/j.pediatrneurol.2025.08.001","url":null,"abstract":"<div><h3>Background</h3><div>Tourette syndrome (TS) and attention-deficit/hyperactivity disorder (ADHD) often co-occur and are linked to emotional and behavioral difficulties. However, their shared and distinct neural underpinnings, particularly in terms of functional connectivity, remain unclear. Here, we assessed how functional connectivity differs across TS and ADHD as well as its association with emotional and behavioral difficulties.</div></div><div><h3>Methods</h3><div>Resting-state electroencephalography (EEG) was recorded from 137 children with TS (n = 51), ADHD (n = 24), or TS + ADHD (n = 29) or from typically developing control subjects (n = 33). Functional connectivity was computed from source-reconstructed EEG data in five frequency bands (delta, theta, alpha, beta, and gamma). Behavioral and emotional problems were assessed with the Child Behavior Checklist.</div></div><div><h3>Results</h3><div>Both TS and ADHD were independently associated with reduced functional connectivity across different brain regions, with no interaction effect. However, externalizing problems showed a TS by ADHD interaction across three frequency bands, such that distinct patterns of functional connectivity were associated with externalizing problems in children with TS + ADHD, relative to those with either TS or ADHD.</div></div><div><h3>Conclusions</h3><div>Although TS and ADHD are associated with decreased functional connectivity in different networks, their effects appear additive rather than interactive at the neural level. However, interactions emerged when examining behavioral problems, suggesting that although TS and ADHD contribute independently to brain connectivity disruptions, their combined impact may uniquely influence emotional and behavioral functioning. This fact highlights the need to consider both shared and disorder-specific mechanisms when studying TS and ADHD.</div></div>","PeriodicalId":19956,"journal":{"name":"Pediatric neurology","volume":"172 ","pages":"Pages 53-62"},"PeriodicalIF":2.1,"publicationDate":"2025-08-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144912319","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}

{"title":"Case Report of Pediatric HPCA-Associated Dystonia: Analysis of Ca2+ and K+ Channel Dynamics and Experience With Pallidal Deep Brain Stimulation","authors":"Laia Nou-Fontanet MD ,&nbsp;Jiraporn Ousingsawat PhD ,&nbsp;Majid Aziz MD ,&nbsp;Reza Maroofian PhD ,&nbsp;Ehsan Ghayoor Karimiani MD, PhD ,&nbsp;Anna Fernández-López MD ,&nbsp;Santiago Candela-Cantó MD, PhD ,&nbsp;Jordi Rumià MD, PhD ,&nbsp;Ana Maria Dominguez MD ,&nbsp;Rainer Schreiber PhD ,&nbsp;Karl Kunzelmann MD ,&nbsp;Juan Dario Ortigoza-Escobar MD, PhD","doi":"10.1016/j.pediatrneurol.2025.07.020","DOIUrl":"10.1016/j.pediatrneurol.2025.07.020","url":null,"abstract":"<div><h3>Background</h3><div>Biallelic pathogenic variants in the <em>HPCA</em> gene cause HPCA-associated dystonia (DYT-<em>HPCA</em>), a rare autosomal recessive disorder characterized by generalized dystonia and complex motor symptoms. <em>HPCA</em> encodes hippocalcin, a Ca²⁺ sensor that modulates neuronal activity through K⁺ channel activation. Here, we describe the clinical and molecular features of two children with novel <em>HPCA</em> variants and assess the impact of deep brain stimulation (DBS) (globus pallidus internus [Gpi]-DBS) on their movement disorders.</div></div><div><h3>Methods</h3><div>Two sisters with <em>HPCA</em> variants (c.91_98del/p.Tyr31Leufs14) were evaluated. Functional studies in fibroblasts from one sister and a previously reported case (<em>HPCA</em> c.49C &gt; T/p.Arg17) analyzed Ca²⁺ signaling and K⁺ channel activity. One sister underwent GPi-DBS, with therapeutic response monitored using the Burke-Fahn-Marsden Dystonia Rating Scale and the Abnormal Involuntary Movement Scale.</div></div><div><h3>Results</h3><div>GPi-DBS improved dystonia and chorea in one patient, reducing Burke-Fahn-Marsden scores by 51%. Fibroblast analyses showed no differences in Ca²⁺ signaling or K⁺ channel activation between cells expressing mutated <em>HPCA</em> and wild-type <em>HPCA</em>.</div></div><div><h3>Conclusions</h3><div>Preliminary evidence from a single pediatric patient suggests that GPi-DBS may be effective for DYT-<em>HPCA</em> in children, but the molecular mechanisms remain unclear, highlighting the need for further research.</div></div>","PeriodicalId":19956,"journal":{"name":"Pediatric neurology","volume":"172 ","pages":"Pages 46-52"},"PeriodicalIF":2.1,"publicationDate":"2025-08-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144912318","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}

{"title":"Risk for Sleep-Disordered Breathing Among Children and Adolescents With Perinatal Stroke and the Impact on Mental Health, Quality of Life, and Caregivers","authors":"Lisa Smithson PhD ,&nbsp;Muhammad Khan MD, CCFP ,&nbsp;Jacqueline Pei R.Psych, PhD ,&nbsp;John Andersen MD ,&nbsp;Jerome Yager MD ,&nbsp;Piushkumar Mandhane MD, PhD ,&nbsp;Adam Kirton MD, MSc ,&nbsp;Carmen Rasmussen PhD","doi":"10.1016/j.pediatrneurol.2025.07.019","DOIUrl":"10.1016/j.pediatrneurol.2025.07.019","url":null,"abstract":"<div><h3>Background</h3><div>Perinatal stroke is a leading cause of cerebral palsy and lifelong neurological disability and a potential risk factor for sleep-disordered breathing (SDB).</div></div><div><h3>Methods</h3><div>We examined the risk for SDB and associated psychosocial outcomes among 77 children with perinatal stroke. Caregivers completed validated questionnaires evaluating their children's sleep (Pediatric Sleep Questionnaire), mental health (Behavior Assessment System for Children, Third Edition [BASC-3]), and quality of life (Pediatric Quality of Life Inventory [PedsQL]) as well as the psychosocial impact on themselves (Parental Outcome Measure [POM]).</div></div><div><h3>Results</h3><div>The risk of SDB (SDB ratio of 0.33 or higher) in children with perinatal stroke was 34%. SDB symptomology was adversely associated with composite measures assessed by the BASC-3 including Externalizing and Internalizing Problems, Behavioral Symptoms Index, and Adaptability. SDB symptoms were associated with greater impairment in the following domains of the PedsQL: Daily Activities, Movement and Balance, Pain and Hurt, Fatigue, and Eating Activities. SDB symptomatology was also correlated with worse psychosocial impact and overall caregiver outcomes on the POM.</div></div><div><h3>Conclusions</h3><div>In conclusion, SDB symptoms are prevalent in children with perinatal stroke and may be associated with adverse psychosocial outcomes for children and caregivers. As a treatable modifier of long-term outcomes, increased awareness, screening, and study are required.</div></div>","PeriodicalId":19956,"journal":{"name":"Pediatric neurology","volume":"172 ","pages":"Pages 21-27"},"PeriodicalIF":2.1,"publicationDate":"2025-08-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144893775","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}

{"title":"Rett Syndrome: Specific MECP2 Variants are Associated With Elevated Serum Neurofilament Light Chain","authors":"Emma Hirn MD ,&nbsp;Brenda Huppke MD ,&nbsp;Bernd Wilken MD ,&nbsp;Michael Kiehntopf MD ,&nbsp;Peter Huppke MD","doi":"10.1016/j.pediatrneurol.2025.07.016","DOIUrl":"10.1016/j.pediatrneurol.2025.07.016","url":null,"abstract":"<div><h3>Background</h3><div>Rett syndrome, a neurodevelopmental disorder predominantly affecting females, is caused by variants in <em>MECP2</em>. Individuals experience a decline in skills, particularly involving language and hand function; nevertheless, studies of brain pathology suggest that neurodegeneration is not involved. To further investigate the presence of neurodegeneration, we measured serum neurofilament light chain (sNfL), a sensitive biomarker of neuronal damage.</div></div><div><h3>Methods</h3><div>Cross-sectional study performed in a cohort of Rett syndrome females with a confirmed pathogenic <em>MECP2</em> variant. sNfL levels were measured using single-molecule array assay, converted to an age-adjusted z-score, and compared with <em>MECP2</em> variant type and clinical characteristics.</div></div><div><h3>Results</h3><div>Included were 77 patients; mean age 14 years, median sNfL level 6.8 pg/mL. sNfL z-scores were higher in the Rett cohort compared to healthy age-matched females (<em>P</em> &lt; 0.001). Elevated sNfL levels were associated with pathogenic variant type; only patients carrying variants affecting the nuclear receptor corepressor interaction domain had elevated sNfL z-scores (<em>P</em> &lt; 0.001) greater than healthy age-matched females, while those with C-terminal deletions or missense variants outside this domain did not. Consistently, patients unable to walk independently and without residual hand function had higher sNfL levels than patients with residual function in these respective areas (<em>P</em> = 0.04).</div></div><div><h3>Conclusions</h3><div>sNfL levels were elevated in our Rett syndrome cohort, irrespective of age, indicating ongoing neuronal damage. However, on closer inspection, this finding was true only for a subset of patients with more severe pathogenic variants affecting the nuclear receptor corepressor interaction domain. sNfL may prove a useful biomarker in upcoming therapeutic trials.</div></div>","PeriodicalId":19956,"journal":{"name":"Pediatric neurology","volume":"172 ","pages":"Pages 1-7"},"PeriodicalIF":2.1,"publicationDate":"2025-08-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144879559","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}

{"title":"Gut Microbiota-Derived Short-Chain Fatty Acids and Sleep Disturbances in Pediatric Attention-Deficit/Hyperactivity Disorder: Insights Into Neurobiological Links and Treatment Implications","authors":"Nonglak Boonchooduang MD, PhD (Assoc. Prof.) ,&nbsp;Orawan Louthrenoo MD (Prof.) ,&nbsp;Narueporn Likhitweerawong MD, PhD (Assoc. Prof.) ,&nbsp;Chanon Kunasol PhD ,&nbsp;Wichwara Nawara MSc ,&nbsp;Chanisa Thonusin MD, PhD (Assoc. Prof.) ,&nbsp;Nipon Chattipakorn MD, PhD (Distinguished Prof.) ,&nbsp;Siriporn C. Chattipakorn DDS, PhD (Distinguished Prof.)","doi":"10.1016/j.pediatrneurol.2025.07.017","DOIUrl":"10.1016/j.pediatrneurol.2025.07.017","url":null,"abstract":"<div><h3>Background</h3><div>Sleep disturbances frequently affect individuals diagnosed with Attention-Deficit/Hyperactivity Disorder (ADHD). Current research emphasizes that the gut-brain axis is a significant pathway influencing both sleep and ADHD symptoms. Metabolites such as short-chain fatty acids (SCFAs), generated by gut microbiota, are crucial in gut-brain interaction, but their connection to sleep issues in ADHD is not well understood. This study assessed the relationship between fecal SCFA levels and sleep disturbances in children diagnosed with ADHD. Differences in SCFA profiles between methylphenidate-medicated and unmedicated children to explore the impact of medication were also investigated.</div></div><div><h3>Methods</h3><div>This cross-sectional study included 25 children diagnosed with ADHD. Sleep disturbance was assessed using the Children's Sleep Habits Questionnaire, and fecal SCFA concentrations were quantified via gas chromatography-mass spectrometry.</div></div><div><h3>Results</h3><div>Most participants (92%) exhibited clinically significant sleep disturbances, as indicated by Children's Sleep Habits Questionnaire scores above the threshold. Unmedicated children showed higher bedtime resistance and greater concentrations of most SCFAs. The partial correlation analysis showed a positive correlation between sleep duration problems and acetate, while negative correlations between bedtime resistance were shown in relation to iso-butyrate, iso-valerate, and valerate. From multiple linear regression, acetate and propionate emerged as significant predictors of sleep issues.</div></div><div><h3>Conclusions</h3><div>This study highlights distinct SCFA profiles in medicated and unmedicated ADHD children, with acetate and propionate emerging as potential predictors of sleep disturbance. These findings highlight the importance of SCFAs in the gut-brain axis and emphasize the need for personalized ADHD management strategies targeting gut health to improve sleep.</div></div>","PeriodicalId":19956,"journal":{"name":"Pediatric neurology","volume":"172 ","pages":"Pages 8-14"},"PeriodicalIF":2.1,"publicationDate":"2025-08-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144886192","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}

{"title":"Movement Disorders Rating Scale for Children Aged 0-3 years: A Revision Study Design","authors":"Roberta Battini MD, PhD ,&nbsp;Roberta Scalise MD, PhD ,&nbsp;Valentina Menici PT, PhD ,&nbsp;Eleonora Gazzanelli PT ,&nbsp;Eleonora Bonaventura MD ,&nbsp;Roberta Di Pietro PT ,&nbsp;Camilla Antonelli MD, PhD ,&nbsp;Andrea Guzzetta MD, PhD ,&nbsp;Giuseppina Sgandurra MD, PhD ,&nbsp;Giovanni Cioni MD","doi":"10.1016/j.pediatrneurol.2025.07.012","DOIUrl":"10.1016/j.pediatrneurol.2025.07.012","url":null,"abstract":"<div><h3>Background</h3><div>Movement Disorders - Childhood Rating Scale (MD-CRS 0-3) is a tool aimed to evaluate movement disorders in the first 3 years of life; however, with the experience gained in 10 years clinical practice, it has been possible, and necessary, to improve the characterization of the scale defining age-appropriate tasks according to age time windows and moving the MD severity from a three-point to a five-point scoring system. By using the scale in our clinical practice and in research, we realized that it was necessary to have more quantitative specifications, allowing a more precise scoring and a better definition of the psychometric properties. This study is a measurement-focused study of recorded video sessions and of video sessions which were carried out in in- and out-patient settings of IRCCS Fondazione Stella Maris aiming to revise the MD-CRS 0-3 and created the MD-CRS 0-3 R to promote and increase its use by improving its structure and items.</div></div><div><h3>Methods</h3><div>A clinical sample of 90 eligible individuals—30 females and 60 males, mean age 2 years and 6 months, and age range 3 months – 3 years and 11 months—was included. Five raters independently and blindly scored the videotapes according to the MD-CRS 0-3 and the MD-CRS 0-3 R, with an interval of at least 6 months.</div></div><div><h3>Results</h3><div>Inter-rater and Intraclass Correlation Coefficient values in all indexes of MD-CRS 0-3 R all exceeded 0.85. Standard Errors of the Measurement and Minimally Detectable Differences were very low. Overall, the MD-CRS 0-3 R exhibited improved psychometric properties compared to the original scale.</div></div><div><h3>Conclusions</h3><div>The present study supports the use of the revised MD-CRS 0-3 in children under 4 years as a trustworthy, sensitive tool as for longitudinal assessments of movement disorder severity.</div></div>","PeriodicalId":19956,"journal":{"name":"Pediatric neurology","volume":"171 ","pages":"Pages 100-106"},"PeriodicalIF":2.1,"publicationDate":"2025-08-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144867468","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}

{"title":"Global Pediatric Epilepsy Burden: Analysis of the Global Burden of Disease Database (1990-2021) With Projections to 2035","authors":"Chen-Qi Zhang MD ,&nbsp;Hong-Yan Li MD ,&nbsp;Li Li MD ,&nbsp;Yu-Xin Zeng BD ,&nbsp;Hua-Fang Yuan BD ,&nbsp;Si-Yu Liang BD ,&nbsp;Yang-Mei Chen PhD","doi":"10.1016/j.pediatrneurol.2025.07.014","DOIUrl":"10.1016/j.pediatrneurol.2025.07.014","url":null,"abstract":"<div><h3>Background</h3><div>To examine the trends in epilepsy prevalence, incidence, mortality, and disability-adjusted life years (DALYs) among children aged 0-14 years from 1990 to 2021, and projected the epidemiological patterns for the next decade to 2035.</div></div><div><h3>Methods</h3><div>Employing standardized Global Burden of Disease approaches, our study evaluated the burden of pediatric epilepsy in 204 countries and regions from 1990 to 2021. Our objective was to provide a comprehensive assessment of the health impact of pediatric epilepsy by incorporating four critical epidemiological indicators—prevalence, incidence, mortality, and DALYs—including their trends and projecting them for the next decade. These data are presented as both raw counts and age-standardized rates per 100,000 people, accompanied by 95% uncertainty intervals (UIs).</div></div><div><h3>Results</h3><div>From 1990 to 2021, global age-standardized Prevalence Rate (ASPR), age-standardized Mortality Rate (ASMR), and age-standardized Disability-Adjusted Life Years rate (ASDR) generally declined, while age-standardized Incidence Rate (ASIR) slightly increased. In 2021, global ASPR and ASIR were 302.99 (95% UI: 212.38-411.09) and 61.00 (95% UI: 39.09-86.21), respectively, while ASMR and ASDR were 0.90 (95% UI: 0.69-1.06) and 177.17 (95% UI: 134.25-236.27), with males generally higher than females. The 10-14 years age group had the highest prevalence, and the under-one age group the lowest, with ASIR, ASMR, and ASDR age distributions reversed. ASPR and ASIR were highest in high Socio-Demographic Index (SDI) regions, with Equatorial Guinea, Ecuador, and Gabon highest, and North Korea, Solomon Islands, and China lowest. ASMR and ASDR were highest in low SDI regions, with South Sudan, Zambia, and Tanzania highest, while Vietnam had the lowest ASMR, and Denmark and Sweden had the lowest ASDR.</div></div><div><h3>Conclusions</h3><div>Over the past 32 years, incidence, mortality, and DALYs for pediatric epilepsy have generally declined, yet regions with lower SDI still report higher mortality and DALYs. Males and infants under 1 year face a disproportionately higher burden of epilepsy. Projected declines in the global burden of pediatric epilepsy are anticipated through 2035, particularly among children under five years, with significant reductions in prevalence and DALYs. The decrease in epilepsy incidence and DALYs among males is more pronounced and rapid compared to females.</div></div>","PeriodicalId":19956,"journal":{"name":"Pediatric neurology","volume":"171 ","pages":"Pages 107-118"},"PeriodicalIF":2.1,"publicationDate":"2025-07-29","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"144867469","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}