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Verena Staedtke, MD, PhD (July 14, 1980 – March 31, 2025) Verena Staedtke,医学博士,博士(1980年7月14日- 2025年3月31日)
IF 2.1 3区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2025-10-27 DOI: 10.1016/j.pediatrneurol.2025.10.019
Carl E. Stafstrom MD, PhD
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引用次数: 0
Prepandemic and Postpandemic: How COVID-19 Pandemic Affected Depression Symptoms in Youth With Epilepsy and Other Neurological Conditions 大流行前和大流行后:COVID-19大流行如何影响患有癫痫和其他神经系统疾病的青少年抑郁症状。
IF 2.1 3区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2025-10-25 DOI: 10.1016/j.pediatrneurol.2025.10.014
Rachna Vipparla , Barbara Storch PhD , Vitória Penido de Paula MD , Naila Makhani MD, MPH , Areti Vassilopoulos PhD

Background

Children and adolescents with neurological diagnoses are more susceptible to experiencing depressive symptoms than their healthy peers. Research to date has found an increase in depressive symptoms among youth during the COVID-19 pandemic, but the impact on youth with neurological conditions has not been directly evaluated.

Methods

This study utilized retrospective data from individuals (n = 1,312) aged 12-21 years to evaluate changes in self-reported depressive symptoms on the Patient Health Questionnaire (Patient Health Questionnaire 2-item and Patient Health Questionnaire 9-item [PHQ-9]) before and after the onset of the COVID-19 pandemic (between March 2018 and March 2022).

Results

The total sample did not exhibit significant differences in depression scores across Patient Health Questionnaire 2-item and PHQ-9 prepandemic and postpandemic onset. However, a significant difference in nonzero PHQ-9 scores was observed in pediatric patients with epilepsy, indicating a shift from minimal to mild depressive symptoms from prepandemic (Mean = 1.69) to postpandemic (Mean = 6.09) onset (P = 0.040). Additional subgroups, including those with neurodevelopmental, psychological, and chronic pain conditions, displayed notable increases in symptom severity based on nonzero scores.

Conclusions

The findings in this study emphasize the need for increased mental health support within pediatric neurology, with a focus on patients with epilepsy. This future research will help attend to and prevent challenges faced by this group of patients when presented with future environmental and social disruptions analogous to the pandemics.
背景:患有神经学诊断的儿童和青少年比健康的同龄人更容易出现抑郁症状。迄今为止的研究发现,在2019冠状病毒病大流行期间,青年抑郁症状有所增加,但尚未直接评估对患有神经系统疾病的青年的影响。方法:本研究利用12-21岁个体(n = 1312)的回顾性数据,评估2019冠状病毒病大流行(2018年3月至2022年3月)前后患者健康问卷(患者健康问卷2项和患者健康问卷9项[PHQ-9])中自我报告抑郁症状的变化。结果:总体样本在大流行前和大流行后发病的患者健康问卷2项和PHQ-9抑郁得分没有显着差异。然而,在儿童癫痫患者中观察到非零PHQ-9评分的显著差异,表明从大流行前(平均= 1.69)到大流行后(平均= 6.09)发作的轻微抑郁症状转变(P = 0.040)。其他亚组,包括那些有神经发育、心理和慢性疼痛状况的患者,在基于非零评分的症状严重程度上显着增加。结论:本研究的发现强调了在儿童神经病学中增加心理健康支持的必要性,重点是癫痫患者。这项未来的研究将有助于关注和预防这组患者在未来面临类似于大流行病的环境和社会破坏时所面临的挑战。
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引用次数: 0
Clinical Profile of Pediatric Neurological Disorders at the Douala Gyneco-Obstetric and Pediatric Hospital in Cameroon 喀麦隆杜阿拉妇产科和儿科医院儿科神经疾病的临床概况。
IF 2.1 3区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2025-10-24 DOI: 10.1016/j.pediatrneurol.2025.10.017
Dominique Enyama MD , Diomède Noukeu Njinkui MD , Maturin Florian Kengni Tefeu MD , Jeanne Mayouego Kouam MD , Daniel Armand Kago Tague MD , Arielle Annick Sime Tchouamo MD , Séraphin Nguefack MD

Background

Neurological conditions account for more than 20% of disease-related morbidity worldwide. Many of those affected by neurological conditions live in Africa, but there are limited descriptions of their prevalence.

Methods

We conducted a descriptive study of all patients aged 3 months to 18 years who were evaluated in the pediatric neurology clinic between January 2020 and December 2023. The data, collected by review of the patients' medical records by the principal investigator, included age, gender, medical history, examination findings, diagnosis, and treatment. Data were analyzed with the Statistical Package for Social Sciences (version 21) software using descriptive statistics.

Results

During the study period, 4,130 patients had pediatric consultations at Douala Gyneco-Obstetric and Pediatric Hospital, of which 1,111 (26.9%) were seen in the pediatric neurology clinic for evaluation of pediatric neurological disorders. A total of 854 patients met our inclusion criteria and were included in the study. The most common conditions were epilepsy (43.4%), cerebral palsy (31.3%), and autism spectrum disorders (13.9%). Regarding the therapeutic itinerary, most patients opted first for traditional practitioners (49.6%), while a smaller proportion (34.2%) chose conventional medicine as the first treatment option.

Conclusions

Pediatric neurological disorders account for a quarter of pediatric consultations at the Douala Gyneco-Obstetric and Pediatric Hospital, with the most common diagnoses being epilepsy and cerebral palsy. This may help in defining priorities, especially as far as rehabilitation, training and resources allocation are concerned.
背景:神经系统疾病占全世界疾病相关发病率的20%以上。许多受神经系统疾病影响的人生活在非洲,但对其流行情况的描述有限。方法:我们对2020年1月至2023年12月在儿科神经病学诊所评估的所有3个月至18岁的患者进行了描述性研究。主要研究者通过审查患者的医疗记录收集的数据包括年龄、性别、病史、检查结果、诊断和治疗。数据分析与统计软件包社会科学(版本21)软件使用描述性统计。结果:在研究期间,有4130名患者在杜阿拉妇产科和儿科医院进行了儿科咨询,其中1111名(26.9%)患者在儿科神经病学门诊进行了儿科神经疾病评估。共有854名患者符合我们的纳入标准并被纳入研究。最常见的疾病是癫痫(43.4%)、脑瘫(31.3%)和自闭症谱系障碍(13.9%)。在治疗行程方面,大多数患者选择传统医生作为首选(49.6%),而选择传统医学作为首选的比例较小(34.2%)。结论:儿童神经系统疾病占杜阿拉妇产科和儿科医院儿科诊断量的四分之一,其中最常见的诊断是癫痫和脑瘫。这可能有助于确定优先事项,特别是在康复、培训和资源分配方面。
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引用次数: 0
Cannabidiol as Adjunctive Treatment in Drug-Resistant Epilepsy With Epileptic Spasms Beyond Two Years of Age 大麻二酚作为2岁以上癫痫痉挛的耐药癫痫的辅助治疗。
IF 2.1 3区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2025-10-24 DOI: 10.1016/j.pediatrneurol.2025.10.013
Elena González-Alguacil MD, J. Jose García Peñas MD, Nuria Lamagrande Casanova MD, Elsa Maria Santana Cabrera MD, Anna Duat Rodríguez PhD, Víctor Soto Insuga PhD

Background

To assess the efficacy and safety of adjunctive cannabidiol (CBD) in children with drug-resistant epileptic spasms (ES) beyond age 2 years.

Methods

We conducted a retrospective longitudinal study of patients with childhood epileptic spasms (CESs) treated with purified CBD (Epidyolex) at Niño Jesus Hospital in Madrid, Spain, from 2020 to 2024. All patients were older than 2 years and had drug-resistant epilepsy and ES as the primary seizure type. Efficacy was assessed by comparing ES frequency before and after CBD treatment, with a good response defined as a ≥50% reduction in ES. Adverse effects (AEs) were recorded to assess safety and tolerability.

Results

Of the 53 patients included, 58.5% achieved a ≥50% reduction in ES frequency, 15% of whom attained complete freedom from ES. Patients with malformations of cortical development and Down syndrome showed the highest response rates. Clobazam was the most frequently associated medication (77.3%), indicating a potential synergistic effect. AEs were reported in 62.2% of patients, with drowsiness, hyperammonemia, and behavioral disturbances being the most common. CBD was discontinued in 17% due to lack of efficacy and in 11.3% due to AEs.

Conclusions

CBD appears to be an effective and well-tolerated adjunctive treatment for drug-resistant ES in children older than 2 years. Significant response rates were found, particularly in patients with Down syndrome and malformations of cortical development. Future studies with larger cohorts and longer follow-up are needed to validate these findings and explore the potential for earlier use of CBD in treatment-resistant epilepsy.
背景:评估辅助大麻二酚(CBD)治疗2岁以上儿童耐药癫痫性痉挛(ES)的疗效和安全性。方法:我们对2020年至2024年在西班牙马德里Niño Jesus医院接受纯化CBD (Epidyolex)治疗的儿童癫痫性痉挛(CESs)患者进行回顾性纵向研究。所有患者年龄均大于2岁,以耐药癫痫和ES为主要发作类型。通过比较CBD治疗前后的ES频率来评估疗效,良好的反应定义为ES减少≥50%。记录不良反应(ae)以评估安全性和耐受性。结果:在纳入的53例患者中,58.5%的患者ES频率降低≥50%,其中15%的患者完全摆脱了ES。皮质发育畸形和唐氏综合症患者的反应率最高。氯巴唑是最常见的相关药物(77.3%),表明有潜在的协同作用。62.2%的患者报告了ae,其中嗜睡、高氨血症和行为障碍最为常见。17%的患者因缺乏疗效而停药,11.3%的患者因不良反应而停药。结论:CBD似乎是2岁以上儿童耐药ES的有效且耐受性良好的辅助治疗。显著的反应率被发现,特别是在患有唐氏综合症和皮质发育畸形的患者中。未来的研究需要更大的队列和更长的随访时间来验证这些发现,并探索早期使用CBD治疗难治性癫痫的潜力。
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引用次数: 0
Mycoplasma Pneumoniae-Associated Arterial Ischemic Stroke in Pediatric Patients: Clinical Manifestations and Neuroimaging Findings 小儿肺炎支原体相关动脉缺血性脑卒中的临床表现和神经影像学结果
IF 2.1 3区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2025-10-23 DOI: 10.1016/j.pediatrneurol.2025.10.016
Zemou Yu PhD , Lingbing Meng PhD , Jingjing Jia PhD , Weihua Zhang MD , Hui Xiong MD , Fang Fang MD , Jiuwei Li MD , Xiuwei Zhuo MD
<div><h3>Background</h3><div>Recently, mycoplasma pneumoniae pneumonia (MPP) has been prevalent among children in China. Literature on the clinical manifestations and neuroradiological findings in pediatric patients with MPP-associated arterial ischemic stroke (AIS) is scarce. This study aimed to describe the clinical characteristics, laboratory and neuroimaging data on pediatric MPP-associated AIS.</div></div><div><h3>Methods</h3><div>This is a single-center retrospective cohort study (COIST study) conducted in Beijing Children's Hospital, consisting of 380 patients with first-ever, imaging-verified ischemic stroke between September 2015 and April 2024. Following rigorous diagnostic protocols, the underlying cause of AIS was definitively determined as mycoplasma pneumoniae infection in a subset of 14 children. A descriptive statistical approach was employed to encapsulate the clinical manifestations, laboratory findings, and neuroradiological characteristics observed in this cohort. The severity of stroke was systematically evaluated using the pediatric National Institutes of Health Stroke Scale, whereas the stroke outcome was assessed with a dual methodology: the pediatric stroke outcome measure and the modified Rankin Scales.</div></div><div><h3>Results</h3><div>A total of 14 children with MPP-associated AIS were enrolled, comprising 8 males (57.14%), with a median age of 6.70 years (interquartile range: 5.12-9.08 years). The incidence of AIS among all hospitalized MPP patients stood at 1.1‰, accounting for 3.68% of all childhood AISs. Notably 12 of the 14 patients exhibited severe MPP. Regarding cerebrospinal fluid analysis, the occurrence of pleocytosis was rarely noted, and the detection of antibodies to mycoplasma pneumoniae within the cerebrospinal fluid was equally rare. The primary neurological manifestations were hemiplegia and facial paralysis, both observed in 78.57% of patients (n = 11). In addition, disturbance of consciousness and aphasia were frequent symptoms, each present in 57.14% of cases (n = 8). Anterior circulation infarcts were the most prevalent, occurring in 78.57% of patients (n = 11), and vascular occlusion was noted in 9 of 14 patients (64.29%). Multiple infarctions were identified in 8 patients (57.14%), while 4 patients (28.57%) experienced bilateral infarctions. The middle cerebral artery was the most commonly affected vascular territory, affected in 50.00% of patients (n = 7), followed by the internal carotid artery and anterior cerebral artery, each affected in 21.43% of cases (n = 3). Hemorrhagic transformation occurred in half of the patients. Regarding treatment, 50% of patients (n = 7) received low molecular weight heparin (LMWH) monotherapy, while 2 patients were treated with both LMWH and aspirin, one with LMWH and dipyridamole, and one with aspirin monotherapy. Despite the onset-to-door time being mostly ≤6 hours, a poor outcome was experienced by 71.43% of patients, with a median pediatric National Insti
近年来,肺炎支原体肺炎(mycoplasma pneumoniae pneumonia, MPP)在中国儿童中流行。关于小儿mpp相关性动脉缺血性脑卒中(AIS)的临床表现和神经影像学表现的文献很少。本研究旨在描述小儿mpp相关AIS的临床特征、实验室和神经影像学数据。方法本研究为单中心回顾性队列研究(COIST),于2015年9月至2024年4月在北京儿童医院开展,纳入380例首次影像学证实的缺血性脑卒中患者。根据严格的诊断方案,14名儿童的潜在病因被明确确定为肺炎支原体感染。采用描述性统计方法来概括该队列中观察到的临床表现、实验室结果和神经放射学特征。卒中严重程度采用美国国立卫生研究院儿童卒中量表进行系统评估,卒中结局采用双重方法进行评估:儿科卒中结局测量和修正Rankin量表。结果共纳入14例mpp相关AIS患儿,其中男性8例(57.14%),中位年龄6.70岁(四分位数范围5.12 ~ 9.08岁)。所有住院MPP患者的AIS发生率为1.1‰,占所有儿童AIS的3.68%。值得注意的是,14例患者中有12例表现为严重的MPP。关于脑脊液分析,很少注意到多细胞增多症的发生,脑脊液中检测到肺炎支原体抗体同样罕见。主要神经系统表现为偏瘫和面瘫,78.57%的患者(n = 11)出现这两种症状。此外,意识障碍和失语是常见症状,分别出现在57.14% (n = 8)的病例中。前循环梗死最为常见,发生率为78.57% (n = 11), 14例患者中有9例(64.29%)出现血管闭塞。多发梗死8例(57.14%),双侧梗死4例(28.57%)。大脑中动脉是最常见的受累血管区域,占50.00% (n = 7),其次是颈内动脉和大脑前动脉,各占21.43% (n = 3)。半数患者发生出血转化。治疗方面,50%的患者(n = 7)接受低分子肝素(LMWH)单药治疗,2例患者同时接受低分子肝素和阿司匹林治疗,1例患者同时接受低分子肝素和双嘧达莫治疗,1例患者同时接受阿司匹林单药治疗。尽管从发病到上门的时间大多≤6小时,但71.43%的患者预后不佳,出院时美国国立卫生研究院卒中量表儿童得分中位数为7分(四分位数范围:2-10分)。结论:在我们的队列中,smpp相关AIS的预后较差。常见的临床症状包括偏瘫、嗜睡和失语。尽管及时意识到mpp相关的卒中,但目前的治疗(低分子肝素和口服阿司匹林)显示出有限的疗效。
{"title":"Mycoplasma Pneumoniae-Associated Arterial Ischemic Stroke in Pediatric Patients: Clinical Manifestations and Neuroimaging Findings","authors":"Zemou Yu PhD ,&nbsp;Lingbing Meng PhD ,&nbsp;Jingjing Jia PhD ,&nbsp;Weihua Zhang MD ,&nbsp;Hui Xiong MD ,&nbsp;Fang Fang MD ,&nbsp;Jiuwei Li MD ,&nbsp;Xiuwei Zhuo MD","doi":"10.1016/j.pediatrneurol.2025.10.016","DOIUrl":"10.1016/j.pediatrneurol.2025.10.016","url":null,"abstract":"&lt;div&gt;&lt;h3&gt;Background&lt;/h3&gt;&lt;div&gt;Recently, mycoplasma pneumoniae pneumonia (MPP) has been prevalent among children in China. Literature on the clinical manifestations and neuroradiological findings in pediatric patients with MPP-associated arterial ischemic stroke (AIS) is scarce. This study aimed to describe the clinical characteristics, laboratory and neuroimaging data on pediatric MPP-associated AIS.&lt;/div&gt;&lt;/div&gt;&lt;div&gt;&lt;h3&gt;Methods&lt;/h3&gt;&lt;div&gt;This is a single-center retrospective cohort study (COIST study) conducted in Beijing Children's Hospital, consisting of 380 patients with first-ever, imaging-verified ischemic stroke between September 2015 and April 2024. Following rigorous diagnostic protocols, the underlying cause of AIS was definitively determined as mycoplasma pneumoniae infection in a subset of 14 children. A descriptive statistical approach was employed to encapsulate the clinical manifestations, laboratory findings, and neuroradiological characteristics observed in this cohort. The severity of stroke was systematically evaluated using the pediatric National Institutes of Health Stroke Scale, whereas the stroke outcome was assessed with a dual methodology: the pediatric stroke outcome measure and the modified Rankin Scales.&lt;/div&gt;&lt;/div&gt;&lt;div&gt;&lt;h3&gt;Results&lt;/h3&gt;&lt;div&gt;A total of 14 children with MPP-associated AIS were enrolled, comprising 8 males (57.14%), with a median age of 6.70 years (interquartile range: 5.12-9.08 years). The incidence of AIS among all hospitalized MPP patients stood at 1.1‰, accounting for 3.68% of all childhood AISs. Notably 12 of the 14 patients exhibited severe MPP. Regarding cerebrospinal fluid analysis, the occurrence of pleocytosis was rarely noted, and the detection of antibodies to mycoplasma pneumoniae within the cerebrospinal fluid was equally rare. The primary neurological manifestations were hemiplegia and facial paralysis, both observed in 78.57% of patients (n = 11). In addition, disturbance of consciousness and aphasia were frequent symptoms, each present in 57.14% of cases (n = 8). Anterior circulation infarcts were the most prevalent, occurring in 78.57% of patients (n = 11), and vascular occlusion was noted in 9 of 14 patients (64.29%). Multiple infarctions were identified in 8 patients (57.14%), while 4 patients (28.57%) experienced bilateral infarctions. The middle cerebral artery was the most commonly affected vascular territory, affected in 50.00% of patients (n = 7), followed by the internal carotid artery and anterior cerebral artery, each affected in 21.43% of cases (n = 3). Hemorrhagic transformation occurred in half of the patients. Regarding treatment, 50% of patients (n = 7) received low molecular weight heparin (LMWH) monotherapy, while 2 patients were treated with both LMWH and aspirin, one with LMWH and dipyridamole, and one with aspirin monotherapy. Despite the onset-to-door time being mostly ≤6 hours, a poor outcome was experienced by 71.43% of patients, with a median pediatric National Insti","PeriodicalId":19956,"journal":{"name":"Pediatric neurology","volume":"175 ","pages":"Pages 58-66"},"PeriodicalIF":2.1,"publicationDate":"2025-10-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145571145","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
Impacts of a Novel Pediatric Neuropalliative Medicine Clinic: Characterization of Referrer Feedback 一种新型儿科神经姑息医学诊所的影响:转诊反馈的特征
IF 2.1 3区 医学 Q2 CLINICAL NEUROLOGY Pub Date : 2025-10-23 DOI: 10.1016/j.pediatrneurol.2025.10.018
Grant L. Lin MD, PhD , Lauren Treat MD

Background

Child neurologists provide care for children with complex chronic health conditions often accompanied by logistical and emotional complexities, as well as acute decision-making scenarios. Pediatric neuropalliative medicine is a nascent subspecialty that aims to augment standard child neurology practice by attending to not only the physical domains of neurologic disease but also the nonphysical issues that accompany advancing illness. The perceptions of clinicians who refer families for this kind of care have not previously been elucidated.

Methods

This survey project queried referrers to a newly established pediatric neuropalliative medicine clinic to characterize their perceptions, including barriers and facilitators of referral and the impact of this service line on clinicians. Specifically, the authors sought to understand whether neurologists viewed this clinic differently than other referring clinicians.

Results

A total of 94 surveys were completed, with 22% (n = 21) of respondents being neurology clinicians and 78% (n = 73) representing other pediatric specialties. Neurology clinicians were significantly more likely to report a preference for a neurologist in the palliative medicine role (76% of neurologists vs 16% of non-neurologists, P < 0.001). While all referrers identified at least one benefit of referral to this clinic, neurologists were more likely to report that this new resource positively impacted their understanding of palliative medicine and their ability to devote more energy to their area of focus.

Conclusions

Pediatric neuropalliative expertise is valued by referring clinicians, with the greatest impact described by neurologists. Referrers noted benefit to patient care as well as enhancement in professional domains.
儿童神经学家为患有复杂慢性健康状况的儿童提供护理,这些儿童通常伴随着后勤和情感复杂性,以及急性决策情景。小儿神经姑息医学是一个新生的亚专科,旨在通过不仅关注神经疾病的物理领域,而且还关注伴随疾病进展的非物理问题,来增强标准的儿童神经病学实践。临床医生的观念谁转介家庭为这种护理以前没有阐明。方法本调查项目询问了新成立的儿科神经姑息医学诊所的转诊者,以描述他们的看法,包括转诊的障碍和便利因素以及该服务线对临床医生的影响。具体来说,作者试图了解神经科医生是否认为这个诊所与其他转诊临床医生不同。结果共完成94份调查,其中22% (n = 21)的受访者为神经内科临床医生,78% (n = 73)的受访者为其他儿科专科医生。神经内科临床医生更倾向于选择神经科医生担任姑息治疗角色(76%的神经科医生对16%的非神经科医生,P < 0.001)。虽然所有的转诊者都认为转诊到这家诊所至少有一个好处,但神经科医生更有可能报告说,这种新的资源对他们对姑息医学的理解产生了积极的影响,并使他们能够将更多的精力投入到他们关注的领域。结论儿科神经姑息治疗专业知识受到转诊临床医生的重视,其中神经内科医生的影响最大。推荐人指出,有利于病人的护理,以及在专业领域的提高。
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引用次数: 0
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 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。
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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 Pub Date : 2025-10-22 DOI: 10.1016/j.pediatrneurol.2025.10.015
Jennifer V. Gettings BMBS, FRCPC
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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 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 ,&nbsp;Alyssa Rust BS ,&nbsp;Leon Dure MD ,&nbsp;Darcy Fehlings MD ,&nbsp;Toni Pearson MBBS ,&nbsp;Roser Pons MD ,&nbsp;Jonathan W. Mink MD, PhD ,&nbsp;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> &lt; 0.05, F-test) and had lower score differences than the stand/walk/run and seated lower extremity tasks (<em>P</em> &lt; 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 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 ,&nbsp;Darcy A. Krueger MD, PhD ,&nbsp;David N. Franz MD ,&nbsp;Paul S. Horn PhD ,&nbsp;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
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Pediatric neurology
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