Pediatric neurology最新文献_第10页

Unravelling the Inflammatory and Metabolic Insights in Subacute Sclerosing Panencephalitis: A Prospective Multimodal Evaluation 揭示亚急性硬化性全脑炎的炎症和代谢：一项前瞻性多模式评估。

IF 2.1 3区医学 Q2 CLINICAL NEUROLOGY

Pediatric neurology

Pub Date : 2025-10-22 DOI: 10.1016/j.pediatrneurol.2025.10.012

Prashant Jauhari MD, DM , Sakshi Ojha MD, DM , Madhavi Tripathi MD , Lata Singh PhD , Vishwesh Bharadiya MBBS , Amulya Gupta MBBS , Parul Berry MBBS , Gagneet Kaur MBBS , Atin Kumar MD , Ashish Upadhyay PhD , Sheffali Gulati MD

Background

This study discusses results of multimodal evaluation of biomarkers of neuroinflammation and neurodegeneration in cerebrospinal fluid (CSF) and Fluorodeoxyglucose-positron emission tomography (18F-FDG-PET) findings in Subacute sclerosing panencephalitis (SSPE).

Methods

Consecutive children (3-14 years) with SSPE fulfilling the Dyken criteria during the study period (2020-2022) were enrolled. Clinical profile, radiological and electroencephalography data were chronicled. CSF interleukins (ILs) IL-4, IL-6, and IL-10; interferon-gamma; and neurofilament heavy chain (NFH) levels were analysed and compared with age matched pseudo-controls. 18F-FDG-PET imaging was performed in a subset of children who consented for the same.

Results

Thirty-three children with SSPE were enrolled [mean age 8.06 ± 2.67 years; males: 82%; Jabbour Stage I-6%, Stage II-67%, Stage III-27%]. Two forms of presentation were seen: short/absent stage I with early onset myoclonic jerks (N = 22; mean age of onset 7.85 ± 2.45) and prolonged stage I with late onset epilepsy (N = 11; mean age 6 ± 1.9 years, P = 0.03). CSF NFH was significantly elevated in SSPE [132.62 (53.6-388.9) vs 0.0 (0-16.9) pg/ml; P = 0.0001] compared to the pseudo-controls. CSF IL-6 (pg/ml) in 21/22 SSPE [1.72 (0.85-3.04)] children and 16/17 pseudo-controls [2.60 (1.72-3.83); P = 0.17] were similar. IL-4, IL-10, and interferon-gammas were undetectable in both the groups. 18F-FDG-PET showed putaminal hypermetabolism in 7/9 (78%) and thalamo-cerebellar hypometabolism in 8/9 (88%) with variable foci of cortical hypometabolism.

Conclusions

Elevated CSF NFH levels suggest ongoing neuronal loss in SSPE. Cell mediated immunity is altered, and active neuroinflammation (T-cell mediated) may not be contributing to the ongoing neurodegeneration. 18F-FDG-PET imaging consistently reveals putaminal hypermetabolism alongside thalamo-cerebellar hypometabolism, which may prove valuable in diagnosing challenging SSPE cases.

背景：本研究讨论了亚急性硬化性全脑炎（SSPE）患者脑脊液（CSF）神经炎症和神经退行性变生物标志物的多模式评估结果和氟脱氧葡萄糖-正电子发射断层扫描（18F-FDG-PET）结果。方法：在研究期间（2020-2022年）连续招募符合Dyken标准的SSPE患儿（3-14岁）。记录临床资料、放射学和脑电图资料。CSF白介素（il） IL-4、IL-6、IL-10；移行细胞;和神经丝重链（NFH）水平进行分析，并与年龄匹配的伪对照进行比较。18F-FDG-PET成像在一组同意的儿童中进行。结果：33例SSPE患儿入组[平均年龄8.06±2.67岁；男性:82%;Jabbour阶段I-6%，阶段II-67%，阶段III-27%]。两种表现形式：短/无I期伴早发性肌阵挛痉挛（N = 22，平均发病年龄7.85±2.45）和延长I期伴晚发性癫痫（N = 11，平均年龄6±1.9岁，P = 0.03）。SSPE患者脑脊液NFH显著升高[132.62 (53.6-388.9)vs 0.0 (0-16.9) pg/ml；P = 0.0001]。CSF IL-6 （pg/ml）在21/22 SSPE患儿[1.72(0.85-3.04)]和16/17伪对照组[2.60 (1.72-3.83)]；P = 0.17]相似。两组均未检测到IL-4、IL-10和干扰素γ。18F-FDG-PET表现为7/9的壳层代谢高（78%），8/9的丘脑-小脑代谢低（88%），皮质代谢低的病灶不同。结论：脑脊液NFH水平升高提示SSPE患者神经元持续丢失。细胞介导的免疫被改变，活动性神经炎症（t细胞介导）可能不会导致持续的神经变性。18F-FDG-PET成像一致显示壳层代谢高和丘脑-小脑代谢低，这可能对诊断具有挑战性的SSPE病例有价值。

{"title":"Unravelling the Inflammatory and Metabolic Insights in Subacute Sclerosing Panencephalitis: A Prospective Multimodal Evaluation","authors":"Prashant Jauhari MD, DM , Sakshi Ojha MD, DM , Madhavi Tripathi MD , Lata Singh PhD , Vishwesh Bharadiya MBBS , Amulya Gupta MBBS , Parul Berry MBBS , Gagneet Kaur MBBS , Atin Kumar MD , Ashish Upadhyay PhD , Sheffali Gulati MD","doi":"10.1016/j.pediatrneurol.2025.10.012","DOIUrl":"10.1016/j.pediatrneurol.2025.10.012","url":null,"abstract":"<div><h3>Background</h3><div>This study discusses results of multimodal evaluation of biomarkers of neuroinflammation and neurodegeneration in cerebrospinal fluid (CSF) and Fluorodeoxyglucose-positron emission tomography (18F-FDG-PET) findings in Subacute sclerosing panencephalitis (SSPE).</div></div><div><h3>Methods</h3><div>Consecutive children (3-14 years) with SSPE fulfilling the Dyken criteria during the study period (2020-2022) were enrolled. Clinical profile, radiological and electroencephalography data were chronicled. CSF interleukins (ILs) IL-4, IL-6, and IL-10; interferon-gamma; and neurofilament heavy chain (NFH) levels were analysed and compared with age matched pseudo-controls. 18F-FDG-PET imaging was performed in a subset of children who consented for the same.</div></div><div><h3>Results</h3><div>Thirty-three children with SSPE were enrolled [mean age 8.06 ± 2.67 years; males: 82%; Jabbour Stage I-6%, Stage II-67%, Stage III-27%]. Two forms of presentation were seen: short/absent stage I with early onset myoclonic jerks (N = 22; mean age of onset 7.85 ± 2.45) and prolonged stage I with late onset epilepsy (N = 11; mean age 6 ± 1.9 years, <em>P</em> = 0.03). CSF NFH was significantly elevated in SSPE [132.62 (53.6-388.9) vs 0.0 (0-16.9) pg/ml; <em>P</em> = 0.0001] compared to the pseudo-controls. CSF IL-6 (pg/ml) in 21/22 SSPE [1.72 (0.85-3.04)] children and 16/17 pseudo-controls [2.60 (1.72-3.83); <em>P</em> = 0.17] were similar. IL-4, IL-10, and interferon-gammas were undetectable in both the groups. 18F-FDG-PET showed putaminal hypermetabolism in 7/9 (78%) and thalamo-cerebellar hypometabolism in 8/9 (88%) with variable foci of cortical hypometabolism.</div></div><div><h3>Conclusions</h3><div>Elevated CSF NFH levels suggest ongoing neuronal loss in SSPE. Cell mediated immunity is altered, and active neuroinflammation (T-cell mediated) may not be contributing to the ongoing neurodegeneration. 18F-FDG-PET imaging consistently reveals putaminal hypermetabolism alongside thalamo-cerebellar hypometabolism, which may prove valuable in diagnosing challenging SSPE cases.</div></div>","PeriodicalId":19956,"journal":{"name":"Pediatric neurology","volume":"174 ","pages":"Pages 110-117"},"PeriodicalIF":2.1,"publicationDate":"2025-10-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145496397","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}

引用次数: 0

First Line Management of Pediatric Status Epilepticus 儿童癫痫持续状态的一线处理。

IF 2.1 3区医学 Q2 CLINICAL NEUROLOGY

Pediatric neurology

Pub Date : 2025-10-22 DOI: 10.1016/j.pediatrneurol.2025.10.015

Jennifer V. Gettings BMBS, FRCPC

引用次数: 0

Tasks for Assessing Dystonia in Young People With Cerebral Palsy 评估青少年脑瘫患者肌张力障碍的任务。

IF 2.1 3区医学 Q2 CLINICAL NEUROLOGY

Pediatric neurology

Pub Date : 2025-10-22 DOI: 10.1016/j.pediatrneurol.2025.10.010

Emma Lott BA , Alyssa Rust BS , Leon Dure MD , Darcy Fehlings MD , Toni Pearson MBBS , Roser Pons MD , Jonathan W. Mink MD, PhD , Bhooma R. Aravamuthan MD, DPhil

Background

Dystonia in childhood is typically associated with cerebral palsy (CP). Dystonia severity scales for CP require prolonged exam protocols with numerous tasks, often making them onerous for routine clinical use. We aimed to identify which individual tasks best approximate dystonia severity compared to the gold standard full protocol.

Methods

In this cross-sectional study, comprehensive exam protocol videos were taken during routine care of ambulatory people with CP age 5 years and up. Five pediatric dystonia experts reviewed individual tasks and the full protocol for dystonia using the Global Dystonia Severity Rating Scale. Experts’ written scoring justifications were qualitatively analyzed to determine commonly cited features of dystonia.

Results

When examining the difference in dystonia severity ratings between each task and the full protocol, seated upper extremity tasks had the lowest variance (P < 0.05, F-test) and had lower score differences than the stand/walk/run and seated lower extremity tasks (P < 0.05, repeated measures Friedman test). Experts most commonly identified the following movements as dystonic: wrist flexion (8.4% of all movement statements), finger flexion (7.3%), wrist ulnar deviation (6.8%), toe dorsiflexion (8.4%), ankle inversion (7.9%), and ankle plantarflexion (6.4%). Experts rated dystonic movements as more severe if they were consistently triggered by multiple stimuli (26.8% of all severity statements) or functionally impactful (20.7%).

Conclusions

Seated upper extremity tasks may be valuable for identifying dystonia and estimating its severity during routine clinical care. Clinical dystonia severity assessment could be guided by assessing specific dystonic movements, the consistency with which they are triggered, and their functional impact.

背景：儿童肌张力障碍通常与脑瘫（CP）有关。肌张力障碍严重程度量表需要长时间的检查方案和许多任务，通常使其在常规临床应用中变得繁重。我们的目的是确定与金标准完整方案相比，哪些单独的任务最接近肌张力障碍的严重程度。方法：在本横断面研究中，对5岁及以上非卧床的CP患者进行常规护理时拍摄综合检查方案录像。五名儿童肌张力障碍专家使用全球肌张力障碍严重程度评定量表审查了肌张力障碍的个体任务和完整方案。对专家的书面评分理由进行定性分析，以确定肌张力障碍的常见特征。结果：在检查各任务与完整方案之间肌张力障碍严重程度评分差异时，坐式上肢任务的方差最小（P < 0.05， f检验），得分差异低于站立/行走/跑步和坐式下肢任务（P < 0.05，重复测量Friedman检验）。专家最常见的肌张力障碍是：手腕屈曲（占所有动作的8.4%）、手指屈曲（7.3%）、腕尺偏离（6.8%）、脚趾背屈（8.4%）、踝关节倒置（7.9%）和踝关节跖屈（6.4%）。专家认为，如果肌张力障碍运动持续受到多种刺激（占所有严重程度陈述的26.8%）或对功能有影响（20.7%），则该运动更严重。结论：在常规临床护理中，坐式上肢任务可能对识别肌张力障碍和估计其严重程度有价值。临床肌张力障碍严重程度的评估可以通过评估特定的肌张力障碍运动、它们触发的一致性以及它们对功能的影响来指导。

{"title":"Tasks for Assessing Dystonia in Young People With Cerebral Palsy","authors":"Emma Lott BA , Alyssa Rust BS , Leon Dure MD , Darcy Fehlings MD , Toni Pearson MBBS , Roser Pons MD , Jonathan W. Mink MD, PhD , Bhooma R. Aravamuthan MD, DPhil","doi":"10.1016/j.pediatrneurol.2025.10.010","DOIUrl":"10.1016/j.pediatrneurol.2025.10.010","url":null,"abstract":"<div><h3>Background</h3><div>Dystonia in childhood is typically associated with cerebral palsy (CP). Dystonia severity scales for CP require prolonged exam protocols with numerous tasks, often making them onerous for routine clinical use. We aimed to identify which individual tasks best approximate dystonia severity compared to the gold standard full protocol.</div></div><div><h3>Methods</h3><div>In this cross-sectional study, comprehensive exam protocol videos were taken during routine care of ambulatory people with CP age 5 years and up. Five pediatric dystonia experts reviewed individual tasks and the full protocol for dystonia using the Global Dystonia Severity Rating Scale. Experts’ written scoring justifications were qualitatively analyzed to determine commonly cited features of dystonia.</div></div><div><h3>Results</h3><div>When examining the difference in dystonia severity ratings between each task and the full protocol, seated upper extremity tasks had the lowest variance (<em>P</em> < 0.05, F-test) and had lower score differences than the stand/walk/run and seated lower extremity tasks (<em>P</em> < 0.05, repeated measures Friedman test). Experts most commonly identified the following movements as dystonic: wrist flexion (8.4% of all movement statements), finger flexion (7.3%), wrist ulnar deviation (6.8%), toe dorsiflexion (8.4%), ankle inversion (7.9%), and ankle plantarflexion (6.4%). Experts rated dystonic movements as more severe if they were consistently triggered by multiple stimuli (26.8% of all severity statements) or functionally impactful (20.7%).</div></div><div><h3>Conclusions</h3><div>Seated upper extremity tasks may be valuable for identifying dystonia and estimating its severity during routine clinical care. Clinical dystonia severity assessment could be guided by assessing specific dystonic movements, the consistency with which they are triggered, and their functional impact.</div></div>","PeriodicalId":19956,"journal":{"name":"Pediatric neurology","volume":"174 ","pages":"Pages 126-134"},"PeriodicalIF":2.1,"publicationDate":"2025-10-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145506326","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}

引用次数: 0

Limited Utility for Head Ultrasounds in the Evaluation of Tuberous Sclerosis Complex 头部超声在评估结节性硬化症复合体中的应用有限

IF 2.1 3区医学 Q2 CLINICAL NEUROLOGY

Pediatric neurology

Pub Date : 2025-10-21 DOI: 10.1016/j.pediatrneurol.2025.10.011

Savannah Stultz BS , Darcy A. Krueger MD, PhD , David N. Franz MD , Paul S. Horn PhD , David M. Ritter MD, PhD

Background

Tuberous sclerosis complex (TSC) is a genetic disorder leading to multiorgan disease. Three cerebral structural manifestations in TSC are tubers, subependymal nodules, and subependymal giant cell astrocytomas (SEGAs). Head ultrasound (HUS) has been established as a diagnostic tool for early head imaging. However, the utility of HUS in detecting TSC cerebral manifestations is not known.

Methods

We conducted a retrospective chart review of clinical brain imaging reports in TSC patients followed by the Cincinnati Children's TSC Clinic. From the clinical reports, the presence of TSC and other lesions were recorded. 373 individuals had either a HUS (N = 42) or a magnetic resonance imaging (MRI; N = 372) before 5 years of age.

Results

Of the 42 patients with HUS, 24 were found to have completely normal HUS (57%). However, on the first MRI, only 3 had normal brain MRIs (13% of normal HUS). One had not had an MRI at the time of data collection and the other 20 had typical TSC lesions. No SEGA was seen on the HUS reports although 5 in the total cohort had a SEGA on MRI reports before 1 month of age. If the HUS was normal, there was a trend toward delaying the first MRI (0.2 ± 0.49 years vs 0.66 ± 0.98 years, P = 0.06).

Conclusions

Compared to the patient's initial MRI results, HUS underdetected the cerebral changes associated with TSC and a normal HUS tends to delay definitive MRI imaging. Thus, HUS has limited utility during the routine workup for TSC.

背景：结节性硬化症（TSC）是一种导致多器官疾病的遗传性疾病。TSC的三种脑结构表现为结节、室管膜下结节和室管膜下巨细胞星形细胞瘤（SEGAs）。头部超声（HUS）已被确立为早期头部成像的诊断工具。然而，HUS在检测TSC脑表现方面的应用尚不清楚。方法回顾性分析辛辛那提儿童TSC诊所对TSC患者的临床脑成像报告。从临床报告中，记录了TSC和其他病变的存在。373例患者在5岁前有溶血性尿毒综合征（N = 42）或磁共振成像（MRI; N = 372）。结果42例溶血性尿毒综合征患者中，24例溶血性尿毒综合征完全正常（57%）。然而，在第一次MRI中，只有3例脑MRI正常（正常溶血性尿毒综合征的13%）。其中一人在数据收集时未进行MRI检查，另外20人有典型的TSC病变。在HUS报告中未发现世嘉，尽管在整个队列中有5例在1个月前的MRI报告中有世嘉。如果HUS正常，则有延迟首次MRI检查的趋势（0.2±0.49年vs 0.66±0.98年，P = 0.06）。结论与患者最初的MRI结果相比，溶血性尿毒综合征未发现与TSC相关的大脑变化，正常的溶血性尿毒综合征往往会延迟最终的MRI成像。因此，在TSC的常规检查中，HUS的效用有限。

{"title":"Limited Utility for Head Ultrasounds in the Evaluation of Tuberous Sclerosis Complex","authors":"Savannah Stultz BS , Darcy A. Krueger MD, PhD , David N. Franz MD , Paul S. Horn PhD , David M. Ritter MD, PhD","doi":"10.1016/j.pediatrneurol.2025.10.011","DOIUrl":"10.1016/j.pediatrneurol.2025.10.011","url":null,"abstract":"<div><h3>Background</h3><div>Tuberous sclerosis complex (TSC) is a genetic disorder leading to multiorgan disease. Three cerebral structural manifestations in TSC are tubers, subependymal nodules, and subependymal giant cell astrocytomas (SEGAs). Head ultrasound (HUS) has been established as a diagnostic tool for early head imaging. However, the utility of HUS in detecting TSC cerebral manifestations is not known.</div></div><div><h3>Methods</h3><div>We conducted a retrospective chart review of clinical brain imaging reports in TSC patients followed by the Cincinnati Children's TSC Clinic. From the clinical reports, the presence of TSC and other lesions were recorded. 373 individuals had either a HUS (N = 42) or a magnetic resonance imaging (MRI; N = 372) before 5 years of age.</div></div><div><h3>Results</h3><div>Of the 42 patients with HUS, 24 were found to have completely normal HUS (57%). However, on the first MRI, only 3 had normal brain MRIs (13% of normal HUS). One had not had an MRI at the time of data collection and the other 20 had typical TSC lesions. No SEGA was seen on the HUS reports although 5 in the total cohort had a SEGA on MRI reports before 1 month of age. If the HUS was normal, there was a trend toward delaying the first MRI (0.2 ± 0.49 years vs 0.66 ± 0.98 years, <em>P</em> = 0.06).</div></div><div><h3>Conclusions</h3><div>Compared to the patient's initial MRI results, HUS underdetected the cerebral changes associated with TSC and a normal HUS tends to delay definitive MRI imaging. Thus, HUS has limited utility during the routine workup for TSC.</div></div>","PeriodicalId":19956,"journal":{"name":"Pediatric neurology","volume":"174 ","pages":"Pages 86-90"},"PeriodicalIF":2.1,"publicationDate":"2025-10-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145468459","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}

引用次数: 0

Infants and Toddlers With Moderate-To-Severe Cerebral Palsy Receive Very Low Doses of In-Person Rehabilitation 患有中度至重度脑瘫的婴幼儿接受非常低剂量的面对面康复治疗

IF 2.1 3区医学 Q2 CLINICAL NEUROLOGY

Pediatric neurology

Pub Date : 2025-10-15 DOI: 10.1016/j.pediatrneurol.2025.10.009

Rachel Bican PT, DPT, PhD , Jill Heathcock PT, MPT, PhD

Background

Dose of rehabilitation services is important for optimizing developmental outcomes for children with cerebral palsy (CP). However, little is known about how much in-person rehabilitation infants and toddlers with CP currently receive, and how dose relates to function. The purpose of this study is to describe the dose of in-person rehabilitation services received by infants and toddlers with CP and to evaluate the relationship between physical therapy (PT) dose, age, and gross motor function.

Methods

We enrolled 53 participants (6 months-2 years old) with moderate-to-severe CP, defined as Gross Motor Function Classification System levels III–V. Parent-reported data on outpatient and early intervention PT, occupational therapy, and speech-language pathology services over the past 6 months were used to calculate monthly service dose. The Gross Motor Function Measure, 88-item was administered to quantify motor function.

Results

Participants received very low doses of therapy across disciplines. The mean total hours of rehabilitation services the participants received were between 0.4 and 3.0 hours per discipline per month. Combined in-person services averaged less than 9 total hours per month. There was no relationship found between the total hours of PT the child received and their age (P = 0.27, F = 1.22) or gross motor function (as measured by the Gross Motor Function Measure, 88-item) (P = 0.82, F = 0.05).

Conclusions

Infants and toddlers with moderate-to-severe CP receive low doses of in-person rehabilitation, and the dose of PT does not appear to be individualized based on age or functional severity. These findings suggest a need for more tailored, intensive, and family-centered rehabilitation planning in early childhood.

康复服务的剂量对于优化脑瘫儿童的发育结局是重要的。然而，对于目前患有CP的婴幼儿接受了多少面对面的康复治疗，以及剂量与功能之间的关系，人们知之甚少。本研究的目的是描述婴幼儿CP患者接受的面对面康复服务的剂量，并评估物理治疗（PT）剂量、年龄和大运动功能之间的关系。方法我们招募了53名患有中度至重度CP的参与者（6个月-2岁），定义为大运动功能分类系统等级III-V。过去6个月的门诊和早期干预PT、职业治疗和语言病理学服务的家长报告数据用于计算每月服务剂量。采用大运动功能量表（共88项）量化运动功能。结果：参与者接受了非常低剂量的跨学科治疗。参与者接受康复服务的平均总时间在每个学科每月0.4至3.0小时之间。综合起来，每个月的上门服务时间平均少于9小时。儿童接受PT的总时数与他们的年龄（P = 0.27, F = 1.22）或大运动功能（用大运动功能量表测量，88项）（P = 0.82, F = 0.05）之间没有关系。结论：中重度CP的婴幼儿接受低剂量的面对面康复治疗，并且PT的剂量似乎并不是基于年龄或功能严重程度的个体化治疗。这些发现表明，需要在儿童早期制定更有针对性、更密集、更以家庭为中心的康复计划。

{"title":"Infants and Toddlers With Moderate-To-Severe Cerebral Palsy Receive Very Low Doses of In-Person Rehabilitation","authors":"Rachel Bican PT, DPT, PhD , Jill Heathcock PT, MPT, PhD","doi":"10.1016/j.pediatrneurol.2025.10.009","DOIUrl":"10.1016/j.pediatrneurol.2025.10.009","url":null,"abstract":"<div><h3>Background</h3><div>Dose of rehabilitation services is important for optimizing developmental outcomes for children with cerebral palsy (CP). However, little is known about how much in-person rehabilitation infants and toddlers with CP currently receive, and how dose relates to function. The purpose of this study is to describe the dose of in-person rehabilitation services received by infants and toddlers with CP and to evaluate the relationship between physical therapy (PT) dose, age, and gross motor function.</div></div><div><h3>Methods</h3><div>We enrolled 53 participants (6 months-2 years old) with moderate-to-severe CP, defined as Gross Motor Function Classification System levels III–V. Parent-reported data on outpatient and early intervention PT, occupational therapy, and speech-language pathology services over the past 6 months were used to calculate monthly service dose. The Gross Motor Function Measure, 88-item was administered to quantify motor function.</div></div><div><h3>Results</h3><div>Participants received very low doses of therapy across disciplines. The mean total hours of rehabilitation services the participants received were between 0.4 and 3.0 hours per discipline per month. Combined in-person services averaged less than 9 total hours per month. There was no relationship found between the total hours of PT the child received and their age (<em>P</em> = 0.27, F = 1.22) or gross motor function (as measured by the Gross Motor Function Measure, 88-item) (<em>P</em> = 0.82, F = 0.05).</div></div><div><h3>Conclusions</h3><div>Infants and toddlers with moderate-to-severe CP receive low doses of in-person rehabilitation, and the dose of PT does not appear to be individualized based on age or functional severity. These findings suggest a need for more tailored, intensive, and family-centered rehabilitation planning in early childhood.</div></div>","PeriodicalId":19956,"journal":{"name":"Pediatric neurology","volume":"174 ","pages":"Pages 91-96"},"PeriodicalIF":2.1,"publicationDate":"2025-10-15","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145468461","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}

引用次数: 0

Powassan Encephalitis: A Unique Endophenotype in 2 Cases 波瓦桑脑炎：2例独特的内表型

IF 2.1 3区医学 Q2 CLINICAL NEUROLOGY

Pediatric neurology

Pub Date : 2025-10-14 DOI: 10.1016/j.pediatrneurol.2025.10.007

Aaron J. Hauptman MD , Molly Wilson-Murphy MD , James H. Powers MD , Tamar Katz MD, PhD

引用次数: 0

Current State of the Global Pediatric Stroke Care-Results From the Global Alliance for Pediatric Stroke Epidemiology and Resources Survey 全球儿童卒中护理现状——来自全球儿童卒中流行病学和资源调查联盟的结果

IF 2.1 3区医学 Q2 CLINICAL NEUROLOGY

Pediatric neurology

Pub Date : 2025-10-14 DOI: 10.1016/j.pediatrneurol.2025.09.020

Ilona Kopyta , Manish Parakh , Gabrielle deVeber , Yenny C. Zuñiga Z , Maria Fernanda Rios Pistoia , Kevin O'Connor , Fernanda Paz Balut , Jenny Wilson , Marilyn Tan , Allyson Schenk , Piotr Rodak , Ankit Kumar Meena , Suman Das

Background

Pediatric stroke care varies across institutions, but global differences have not been explored. Our objective was to evaluate global variability and challenges in pediatric stroke care.

Methods

The Global Alliance for Pediatric Stroke Epidemiology and Resources Working Group of the International Pediatric Stroke Organization distributed a 43-question online survey to international child neurology organizations.

Results

A total of 135 responses were received, from Europe, North America, and Australia (n = 66), South America (n = 27) and Asia and Africa (AA; n = 42). Respondents were mainly pediatric neurologists from quaternary/tertiary teaching hospitals (>90%) and worked in urban areas (85%-90%) or large cities (40%-60%). Outside of Asia, women comprised 70% of the respondents, whereas males made up the majority in Asia. Globally, pediatric neurologists manage emergency room triage and inpatient treatment stroke in most facilities (65%-68%). Experienced (>20 years) pediatric stroke subspecialists were available in a minority of centers (7%-17%). Multidisciplinary teams and institutional algorithms were more common in Europe and the Americas compared to Asia (53% vs 7-11% and 80-85% vs 20%, respectively). Reduced awareness of pediatric stroke was identified as a reason for the delay in seeking care in Europe, North America, and Australia by caregivers (44%) and health providers (38%), while in AA it was 67% and 58%.

Conclusions

Across all regions, significant challenges in pediatric stroke management exist. Serious gaps exist in Asia related to personnel, expertise, and infrastructure. Development and improvement of local policies and resources are urgently needed in AA to improve pediatric stroke outcomes.

不同机构的儿童中风护理各不相同，但尚未探讨全球差异。我们的目的是评估小儿卒中护理的全球变异性和挑战。方法国际小儿卒中组织的全球小儿卒中流行病学联盟和资源工作组向国际儿童神经病学组织分发了一份包含43个问题的在线调查。结果共收到135份回复，分别来自欧洲、北美和澳大利亚（n = 66）、南美（n = 27）和亚洲和非洲（n = 42）。受访者主要是来自第四/三级教学医院的儿科神经科医生（占90%），工作地点在城市（85%-90%）或大城市（40%-60%）。在亚洲以外，女性占受访者的70%，而在亚洲，男性占多数。在全球范围内，大多数机构（65%-68%）的儿科神经科医生负责急诊室分诊和住院治疗。少数中心（7%-17%）有经验丰富（20岁）的儿科卒中专科医生。与亚洲相比，多学科团队和机构算法在欧洲和美洲更为常见（分别为53%对7-11%和80-85%对20%）。在欧洲、北美和澳大利亚，护理人员（44%）和卫生服务提供者（38%）认为儿童卒中意识降低是延迟就医的原因，而在美国，这一比例分别为67%和58%。结论：在所有地区，儿童脑卒中管理都存在重大挑战。亚洲在人员、专业知识和基础设施方面存在严重差距。AA迫切需要制定和完善当地政策和资源，以改善儿童卒中预后。

{"title":"Current State of the Global Pediatric Stroke Care-Results From the Global Alliance for Pediatric Stroke Epidemiology and Resources Survey","authors":"Ilona Kopyta , Manish Parakh , Gabrielle deVeber , Yenny C. Zuñiga Z , Maria Fernanda Rios Pistoia , Kevin O'Connor , Fernanda Paz Balut , Jenny Wilson , Marilyn Tan , Allyson Schenk , Piotr Rodak , Ankit Kumar Meena , Suman Das","doi":"10.1016/j.pediatrneurol.2025.09.020","DOIUrl":"10.1016/j.pediatrneurol.2025.09.020","url":null,"abstract":"<div><h3>Background</h3><div>Pediatric stroke care varies across institutions, but global differences have not been explored. Our objective was to evaluate global variability and challenges in pediatric stroke care.</div></div><div><h3>Methods</h3><div>The Global Alliance for Pediatric Stroke Epidemiology and Resources Working Group of the International Pediatric Stroke Organization distributed a 43-question online survey to international child neurology organizations.</div></div><div><h3>Results</h3><div>A total of 135 responses were received, from Europe, North America, and Australia (n = 66), South America (n = 27) and Asia and Africa (AA; n = 42). Respondents were mainly pediatric neurologists from quaternary/tertiary teaching hospitals (>90%) and worked in urban areas (85%-90%) or large cities (40%-60%). Outside of Asia, women comprised 70% of the respondents, whereas males made up the majority in Asia. Globally, pediatric neurologists manage emergency room triage and inpatient treatment stroke in most facilities (65%-68%). Experienced (>20 years) pediatric stroke subspecialists were available in a minority of centers (7%-17%). Multidisciplinary teams and institutional algorithms were more common in Europe and the Americas compared to Asia (53% vs 7-11% and 80-85% vs 20%, respectively). Reduced awareness of pediatric stroke was identified as a reason for the delay in seeking care in Europe, North America, and Australia by caregivers (44%) and health providers (38%), while in AA it was 67% and 58%.</div></div><div><h3>Conclusions</h3><div>Across all regions, significant challenges in pediatric stroke management exist. Serious gaps exist in Asia related to personnel, expertise, and infrastructure. Development and improvement of local policies and resources are urgently needed in AA to improve pediatric stroke outcomes.</div></div>","PeriodicalId":19956,"journal":{"name":"Pediatric neurology","volume":"174 ","pages":"Pages 101-109"},"PeriodicalIF":2.1,"publicationDate":"2025-10-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145467817","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}

引用次数: 0

Rituximab Administration in Kleine–Levin Syndrome: A Case Report 利妥昔单抗治疗Kleine-Levin综合征1例报告。

IF 2.1 3区医学 Q2 CLINICAL NEUROLOGY

Pediatric neurology

Pub Date : 2025-10-14 DOI: 10.1016/j.pediatrneurol.2025.10.008

Naoki Yamada MD, Ichiro Kuki MD, PhD, Masataka Fukuoka MD, Megumi Nukui MD, Takeshi Inoue MD, Shin Okazaki MD

引用次数: 0

Application of Delphi Method-Based Definition of Treatment-Refractory Tourette Disorder in a Large Italian Clinical Cohort of Children and Adolescents 基于德尔菲法的难治性抽动秽语障碍定义在意大利儿童和青少年临床队列中的应用

IF 2.1 3区医学 Q2 CLINICAL NEUROLOGY

Pediatric neurology

Pub Date : 2025-10-13 DOI: 10.1016/j.pediatrneurol.2025.10.002

Adriana Prato MD, PhD , Rita Barone MD, PhD (Professor) , Maria Chiara Milana MD , Lara Cirnigliaro MD (PhD Student) , Davide Martino MD, PhD (Professor) , Renata Rizzo MD, PhD (Professor)

Background

Tourette syndrome (TS) is characterized by the presence of tics and possible associated neuropsychiatric comorbidities. A subset of patients with TS present with severe and “treatment-refractory” tics, requiring multiple therapeutic interventions.

Methods

In 2023, a group of international specialist clinicians with specific expertise in tics used a multistep Delphi approach to propose an expert consensus-based definition of treatment failure for tics. In this study, we applied this recently published algorithm to establish treatment-refractory TS patients in a large Italian cohort of 614 pediatric patients.

Results

Of the entire cohort, 20 patients (7.3%) fulfilled all the criteria proposed in this algorithm to define refractoriness to prescribed antitic medications. Compared to non–treatment-refractory (n = 254), treatment-refractory TS patients (n = 20) had significantly higher mean total intelligence quotient (P = 0.008) and mean Yale Global Tic Severity Scale-Total Tic Score scores (P < 0.00001). The 20 treatment-refractory TS patients reported a higher frequency of a family history of TS (60% vs. 46.1%), and a lower rate of associated neuropsychiatric comorbidities (55% vs. 66.9%), although these differences did not reach statistical significance.

Conclusions

In general, a greater tic severity might be associated with higher risk of refractoriness to pharmacological treatment for tics. Considering the paucity of literature research exploring the frequency of severe cases of TS who were refractory to medical treatment, further studies are needed to consolidate applicability within routine clinical care of this recently developed consensus definition of pharmacological treatment refractoriness for TS.

背景：抽动秽语综合征（TS）的特点是存在抽搐和可能相关的神经精神合并症。一部分TS患者存在严重的“难治性”抽搐，需要多种治疗干预。方法2023年，一组具有抽动学专业知识的国际专家临床医生使用多步骤德尔菲法提出了基于专家共识的抽动学治疗失败定义。在这项研究中，我们应用了最近发表的算法，在意大利614名儿科患者中建立了难治性TS患者。结果在整个队列中，20例患者（7.3%）符合该算法中提出的所有标准，以确定处方抗炎药物的难治性。与非治疗难治性TS患者（n = 254）相比，治疗难治性TS患者（n = 20）的平均总智商（P = 0.008）和平均耶鲁整体抽动严重程度量表-总抽动评分（P < 0.00001）显著高于非治疗难治性TS患者（n = 254）。20例难治性TS患者报告有较高的TS家族史（60%对46.1%）和较低的相关神经精神合并症（55%对66.9%），尽管这些差异没有达到统计学意义。结论一般情况下，抽搐严重程度越高，抽搐药物治疗难治的风险越高。考虑到文献研究的缺乏，探讨难治性TS重症病例的医学治疗频率，需要进一步的研究来巩固在常规临床护理中的适用性，这是最近形成的共识定义的难治性TS的药物治疗。

{"title":"Application of Delphi Method-Based Definition of Treatment-Refractory Tourette Disorder in a Large Italian Clinical Cohort of Children and Adolescents","authors":"Adriana Prato MD, PhD , Rita Barone MD, PhD (Professor) , Maria Chiara Milana MD , Lara Cirnigliaro MD (PhD Student) , Davide Martino MD, PhD (Professor) , Renata Rizzo MD, PhD (Professor)","doi":"10.1016/j.pediatrneurol.2025.10.002","DOIUrl":"10.1016/j.pediatrneurol.2025.10.002","url":null,"abstract":"<div><h3>Background</h3><div>Tourette syndrome (TS) is characterized by the presence of tics and possible associated neuropsychiatric comorbidities. A subset of patients with TS present with severe and “treatment-refractory” tics, requiring multiple therapeutic interventions.</div></div><div><h3>Methods</h3><div>In 2023, a group of international specialist clinicians with specific expertise in tics used a multistep Delphi approach to propose an expert consensus-based definition of treatment failure for tics. In this study, we applied this recently published algorithm to establish treatment-refractory TS patients in a large Italian cohort of 614 pediatric patients.</div></div><div><h3>Results</h3><div>Of the entire cohort, 20 patients (7.3%) fulfilled all the criteria proposed in this algorithm to define refractoriness to prescribed antitic medications. Compared to non–treatment-refractory (n = 254), treatment-refractory TS patients (n = 20) had significantly higher mean total intelligence quotient (<em>P</em> = 0.008) and mean Yale Global Tic Severity Scale-Total Tic Score scores (<em>P</em> < 0.00001). The 20 treatment-refractory TS patients reported a higher frequency of a family history of TS (60% vs. 46.1%), and a lower rate of associated neuropsychiatric comorbidities (55% vs. 66.9%), although these differences did not reach statistical significance.</div></div><div><h3>Conclusions</h3><div>In general, a greater tic severity might be associated with higher risk of refractoriness to pharmacological treatment for tics. Considering the paucity of literature research exploring the frequency of severe cases of TS who were refractory to medical treatment, further studies are needed to consolidate applicability within routine clinical care of this recently developed consensus definition of pharmacological treatment refractoriness for TS.</div></div>","PeriodicalId":19956,"journal":{"name":"Pediatric neurology","volume":"174 ","pages":"Pages 54-61"},"PeriodicalIF":2.1,"publicationDate":"2025-10-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145419783","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}

引用次数: 0

Gaps and Challenges in the Transition of Care in Neuromuscular Disorders With a Focus on Duchenne Muscular Dystrophy 神经肌肉疾病护理过渡中的差距和挑战——以杜氏肌营养不良为重点。

IF 2.1 3区医学 Q2 CLINICAL NEUROLOGY

Pediatric neurology

Pub Date : 2025-10-13 DOI: 10.1016/j.pediatrneurol.2025.10.006

Alessia Marcassoli MSc , Nethmi Rajapakse BHSc , Erika Guastafierro MSc , Jo-Anne Petropoulos MIST , Giulia Trucco MSc , Angelica Mazzilli MSc , Sebastian Friedrich MD , Sunil Rodger MD , Jana Willems MSc , Delaney Ringer BHSc , Julia Frei MD , Anna Swain MSc , Matilde Leonardi MD , Nardo Nardocci MD , Thorsten Langer MD , Olaf Kraus De Camargo MD , Jan Willelm Gorter MD, PhD , Kinga Pozniak PhD , Anne Fournier MD , Rocio Gutierrez MSc , Isabella Moroni MD

Background

Transition from pediatric to adult care in neuromuscular disorders represents a great challenge for both patients and their families, and especially for patients with Duchenne Muscular Dystrophy. This review aims to investigate the transition experience from pediatric to adult care of patients and their families, considering different aspects of daily life.

Methods

Medline, Embase, PsycINFO, CINHAL, Web of Science, and SCOPUS electronic databases were searched to identify studies published from January 2000 to December 2024. From the initial 1955 research articles, 26 were finally included for qualitative synthesis. Studies reported information about transition process, activities of daily living, and relationships.

Results

Our results show the need, as expressed by patients and caregivers, for multidisciplinary collaboration between pediatric and adult care settings, proposing the adoption of an adolescent-centered approach that prompts patients to share their perspectives on specific health needs directly with caregivers. The findings underscore the importance of also considering the psychological and social aspects that can influence the transition path across different areas of life.

Conclusions

Without structured guidelines and indications, patients and families feel left alone during the transition process. Transition programs should consider all the life areas in which patients are involved, including strategies to improve engagement in social, educational, and professional settings.

背景：神经肌肉疾病从儿科到成人护理的转变对患者及其家庭来说都是一个巨大的挑战，尤其是对杜氏肌营养不良患者。本综述旨在探讨从儿童到成人护理患者及其家属的过渡经验，考虑到日常生活的不同方面。方法：检索Medline、Embase、PsycINFO、CINHAL、Web of Science、SCOPUS等电子数据库，检索2000年1月至2024年12月发表的相关研究。从最初的1955篇研究文章中，最终纳入了26篇进行定性综合。研究报告了关于过渡过程、日常生活活动和人际关系的信息。结果：我们的研究结果表明，正如患者和护理人员所表达的那样，儿科和成人护理机构之间需要多学科合作，建议采用以青少年为中心的方法，促使患者直接与护理人员分享他们对特定健康需求的看法。研究结果强调了考虑心理和社会因素的重要性，这些因素可能会影响生活中不同领域的过渡路径。结论：如果没有结构化的指南和适应症，患者和家属在过渡过程中会感到孤独。过渡方案应考虑到患者所涉及的所有生活领域，包括提高社会、教育和专业环境参与度的策略。

{"title":"Gaps and Challenges in the Transition of Care in Neuromuscular Disorders With a Focus on Duchenne Muscular Dystrophy","authors":"Alessia Marcassoli MSc , Nethmi Rajapakse BHSc , Erika Guastafierro MSc , Jo-Anne Petropoulos MIST , Giulia Trucco MSc , Angelica Mazzilli MSc , Sebastian Friedrich MD , Sunil Rodger MD , Jana Willems MSc , Delaney Ringer BHSc , Julia Frei MD , Anna Swain MSc , Matilde Leonardi MD , Nardo Nardocci MD , Thorsten Langer MD , Olaf Kraus De Camargo MD , Jan Willelm Gorter MD, PhD , Kinga Pozniak PhD , Anne Fournier MD , Rocio Gutierrez MSc , Isabella Moroni MD","doi":"10.1016/j.pediatrneurol.2025.10.006","DOIUrl":"10.1016/j.pediatrneurol.2025.10.006","url":null,"abstract":"<div><h3>Background</h3><div>Transition from pediatric to adult care in neuromuscular disorders represents a great challenge for both patients and their families, and especially for patients with Duchenne Muscular Dystrophy. This review aims to investigate the transition experience from pediatric to adult care of patients and their families, considering different aspects of daily life.</div></div><div><h3>Methods</h3><div>Medline, Embase, PsycINFO, CINHAL, Web of Science, and SCOPUS electronic databases were searched to identify studies published from January 2000 to December 2024. From the initial 1955 research articles, 26 were finally included for qualitative synthesis. Studies reported information about transition process, activities of daily living, and relationships.</div></div><div><h3>Results</h3><div>Our results show the need, as expressed by patients and caregivers, for multidisciplinary collaboration between pediatric and adult care settings, proposing the adoption of an adolescent-centered approach that prompts patients to share their perspectives on specific health needs directly with caregivers. The findings underscore the importance of also considering the psychological and social aspects that can influence the transition path across different areas of life.</div></div><div><h3>Conclusions</h3><div>Without structured guidelines and indications, patients and families feel left alone during the transition process. Transition programs should consider all the life areas in which patients are involved, including strategies to improve engagement in social, educational, and professional settings.</div></div>","PeriodicalId":19956,"journal":{"name":"Pediatric neurology","volume":"174 ","pages":"Pages 74-80"},"PeriodicalIF":2.1,"publicationDate":"2025-10-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145452549","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}

引用次数: 0