Brain & Development最新文献_第4页

Safety and efficacy of viltolarsen treatment in patients with Duchenne muscular dystrophy: A retrospective study with 3-year follow-up 维托拉生治疗杜氏肌营养不良症患者的安全性和有效性：一项为期三年的回顾性研究。

IF 1.4 4区医学 Q4 CLINICAL NEUROLOGY

Brain & Development

Pub Date : 2024-11-15 DOI: 10.1016/j.braindev.2024.10.005

Michinori Funato , Reina Iwata , Toru Ando , Motohiro Takeuchi , Yohei Horibe , Takashi Funato , Junko Oota , Ryoya Uno , Misaki Yumioka , Emi Kondo , Miho Katoyama , Chie Ito , Yoshiko Kato , Yuji Yamada , Toshifumi Kusukawa

Background

Duchenne muscular dystrophy (DMD) is a hereditary neuromuscular disorder characterized by severe, progressive muscle wasting. Viltolarsen, a formulation consisting of exon 53-skipping antisense oligonucleotides of the dystrophin gene, has been indicated for some patients with DMD. However, reports describing the efficacy and safety of viltolarsen treatment in patients with DMD, particularly those comparing patients receiving viltolarsen with age- and time-period-matched controls, are limited. This study aimed to confirm the safety and efficacy of viltolarsen treatment in patients with DMD.

Methods

We retrospectively reviewed real-world data of patients with DMD aged 6–19 years who started viltolarsen treatment between July 2020 and February 2021 and compared them with age- and time-period-matched patients with DMD who did not receive viltolarsen.

Results

Five patients received viltolarsen treatment for over 3 years. Three of the four patients who received viltolarsen once weekly maintained motor function, while one ambulatory patient showed remarkably slow motor function decline. Conversely, eight of nine patients who did not receive viltolarsen and one patient who underwent intermittent viltolarsen treatment showed motor function deterioration. Two patients showed significant treatment-related adverse events, namely swelling of the dorsal surface of the right hand due to extravasation of viltolarsen in one patient and axillary lymph node enlargement due to frequent intravenous infusion in another patient. None of the patients discontinued viltolarsen.

Conclusions

Viltolarsen was a safe and effective treatment for patients with DMD, and the findings highlighted the importance of once-weekly and uninterrupted viltolarsen treatment. The distinct safety and efficacy of viltolarsen require further investigation using a large number of cases and a long follow-up period.

背景：杜氏肌营养不良症（DMD）是一种遗传性神经肌肉疾病，以严重的进行性肌肉萎缩为特征。Viltolarsen是一种由dystrophin基因第53外显子缺失反义寡核苷酸组成的制剂，已被用于治疗部分DMD患者。然而，有关DMD患者接受维尔托拉生治疗的疗效和安全性的报道，尤其是将接受维尔托拉生治疗的患者与年龄和时间匹配的对照组进行比较的报道十分有限。本研究旨在证实DMD患者接受维尔托拉生治疗的安全性和有效性：我们回顾性审查了2020年7月至2021年2月期间开始接受维尔托拉生治疗的6-19岁DMD患者的真实世界数据，并将其与未接受维尔托拉生治疗的年龄和时间段匹配的DMD患者进行了比较：结果：5名患者接受了3年以上的维尔托拉生治疗。在每周接受一次维尔托拉生治疗的四名患者中，有三名患者的运动功能得以维持，而一名卧床患者的运动功能下降速度明显较慢。相反，9名未接受维尔托拉生治疗的患者中有8名和1名接受间歇性维尔托拉生治疗的患者出现了运动功能衰退。两名患者出现了明显的治疗相关不良反应，即一名患者因维尔多拉生外渗导致右手背肿胀，另一名患者因频繁静脉输液导致腋窝淋巴结肿大。所有患者均未停用维尔多拉生：结论：维托拉生对DMD患者是一种安全有效的治疗方法，研究结果强调了每周一次不间断维托拉生治疗的重要性。维尔托拉生的独特安全性和有效性需要通过大量病例和长期随访进行进一步研究。

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引用次数: 0

Chaperone therapy: Stabilization and enhancement of endogenous and exogenous lysosomal enzymes 伴侣疗法：稳定和增强内源性和外源性溶酶体酶。

IF 1.4 4区医学 Q4 CLINICAL NEUROLOGY

Brain & Development

Pub Date : 2024-11-15 DOI: 10.1016/j.braindev.2024.10.006

Yoshiyuki Suzuki

Chaperone therapy is a new concept of molecular therapeutic approach to protein misfolding diseases, particularly to lysosomal diseases. Initially we started molecular analysis of culture cells, model animals and patients with Fabry disease and G_M1-gangliosidosis. Some mutant enzyme proteins did not express the catalytic activity because of unstable molecular structure in somatic cells. The small molecule compound (chaperone) corrected misfolding of the unstable mutant protein, resulting in restoration of the enzyme activity (chaperone therapy). This pathological molecular event was studied first in endogenous mutant enzymes. Then a similar molecular interaction was found between the chaperone and the exogenous protein supplied for enzyme replacement therapy (ERT) in Pompe disease. This new chaperone-ERT combination therapy will become another useful technology in order to expand the application of chaperone therapy to a wide range of lysosomal diseases. Thus, chaperone therapy is expected in future for stabilization and enhancement of exogenously supplied ERT enzymes as well as endogenous mutant enzymes.

伴侣疗法是针对蛋白质错误折叠疾病，尤其是溶酶体疾病的分子治疗方法的新概念。最初，我们开始对法布里病和 GM1-神经节苷脂病患者的培养细胞、模型动物和患者进行分子分析。一些突变酶蛋白由于在体细胞中分子结构不稳定而无法表达催化活性。小分子化合物（伴侣素）可以纠正不稳定突变蛋白的错误折叠，从而恢复酶的活性（伴侣素疗法）。首先在内源性突变酶中研究了这一病理分子事件。然后，在伴侣蛋白和用于庞贝氏症酶替代疗法（ERT）的外源蛋白之间发现了类似的分子相互作用。这种新的合子-酶替代组合疗法将成为另一种有用的技术，从而将合子疗法的应用范围扩大到多种溶酶体疾病。因此，伴侣蛋白疗法有望在未来用于稳定和增强外源性提供的 ERT 酶以及内源性突变酶。

引用次数: 0

Looks can be deceiving: An appraisal of Sturge weber syndrome type III case series 外表是会骗人的：对韦伯综合征 III 型病例系列的评估。

IF 1.4 4区医学 Q4 CLINICAL NEUROLOGY

Brain & Development

Pub Date : 2024-11-01 DOI: 10.1016/j.braindev.2024.09.003

Vaishakh Anand , Kollencheri Puthenveettil Vinayan , Shilpa Radhakrishnan

引用次数: 0

Characterizing visual processing deficits in cerebral adrenoleukodystrophy 脑肾上腺白质营养不良症视觉处理缺陷的特征。

IF 1.4 4区医学 Q4 CLINICAL NEUROLOGY

Brain & Development

Pub Date : 2024-11-01 DOI: 10.1016/j.braindev.2024.09.008

Camille S. Corre , Melissa Bambery , Christopher R. Bennett , Amanda Nagy , Claire E. Manley , Ellen Winter , Cary Peregoy , Daniel Kelly , Haley Andonian , Stacy Maciel , Catherine Becker , Lotfi B. Merabet , Florian S. Eichler

Background and objectives

Cerebral adrenoleukodystrophy (CALD) can cause visual impairment, but early symptoms are often missed or misdiagnosed. The framework of cerebral visual impairment (CVI) distinguishes deficits in sensory detection (“lower order”) from those of perception and interpretation (“higher order”). This study describes visual deficits in patients with CALD and higher order visual function assessed with a virtual reality (VR) interface combined with eye tracking.

Methods

A retrospective medical record review assessed the prevalence of visual deficits in patients with CALD, as well as lesion burden on brain MRI using the Loes MRI severity score. A VR-based task measured visual spatial processing performance in participants with CALD and controls.

Results

Out of 89 CALD patients, 69 % had at least one sign or symptom of visual impairment. Lower order deficits were seen in 56 % of patients, and higher order deficits were seen in 59 % of patients who underwent neuropsychological testing. Even in early stage disease (Loes MRI severity score ≤ 3), visual impairment was present in more than half of patients (58 %). On prospective VR-based assessment, the CALD group (n = 30) had impaired visual search performance (lower success rate and longer reaction time) compared with controls (n = 38). In both groups, there was a trend of worsening performance with increasing task difficulty.

Discussion

Higher order visual deficits, not just impairment of visual acuity, visual fields, or oculomotor function, are common in all stages of CALD. Beyond neuropsychological testing, VR-based functional testing allows for quantitative assessment of higher order visual perceptual deficits that are relevant to everyday tasks and may serve as an important marker of neurological decline.

背景和目的：脑性肾上腺白质营养不良症（CALD）可导致视力障碍，但早期症状往往被漏诊或误诊。脑性视力障碍（CVI）框架将感官检测（"低阶"）障碍与感知和解释（"高阶"）障碍区分开来。本研究通过虚拟现实（VR）界面结合眼动追踪技术，对CALD患者的视觉缺陷和高阶视觉功能进行了评估：一项回顾性病历审查评估了CALD患者视觉缺陷的发生率，并使用Loes MRI严重程度评分评估了脑部MRI的病变负荷。一项基于VR的任务测量了CALD患者和对照组患者的视觉空间处理能力：在 89 名 CALD 患者中，69% 的患者至少有一种视觉障碍的迹象或症状。在接受神经心理学测试的患者中，56%的患者存在低阶障碍，59%的患者存在高阶障碍。即使在疾病早期（Loes MRI 严重程度评分≤3），也有超过一半的患者（58%）存在视力障碍。在基于虚拟现实的前瞻性评估中，与对照组（38 人）相比，CALD 组（30 人）的视觉搜索能力受损（成功率较低，反应时间较长）。随着任务难度的增加，两组患者的表现都呈恶化趋势：讨论：高阶视觉障碍，不仅仅是视力、视野或眼球运动功能的损害，在 CALD 的各个阶段都很常见。除神经心理测试外，基于虚拟现实的功能测试可对与日常任务相关的高阶视觉知觉障碍进行定量评估，并可作为神经功能衰退的重要标志。

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引用次数: 0

Translation and validation of the Thai Version of the Japanese Sleep Questionnaire for Preschoolers (JSQ-P) 泰语版日本学龄前儿童睡眠问卷（JSQ-P）的翻译和验证。

IF 1.4 4区医学 Q4 CLINICAL NEUROLOGY

Brain & Development

Pub Date : 2024-11-01 DOI: 10.1016/j.braindev.2024.08.003

Natcha Lueangapapong , Jariya Chuthapisith , Lunliya Thampratankul

Purpose

This study aimed to translate and validate the Japanese Sleep Questionnaire for Preschoolers (JSQ-P) into a Thai version and to evaluate factors associated with sleep problems in preschoolers.

Methods

The cross-cultural adaptation of JSQ-P into Thai version of JSQ-P (TH-JSQ-P) was performed including forward translation, reconciliation, backward translation, and final approval by the original creator. Children aged 2–6 years from community and clinical settings were enrolled. The TH-JSQ-P was completed by parents of preschool children twice within 10–14 days to assess its reliability and validity. Six validity tests including face validity, content validity, structural validity, construct validity, criterion validity and predictive validity were assessed. Demographic data, Epworth sleepiness scale (ESS), medical and behavior problems, and socioeconomic status were collected for analysis.

Results

A total of 2613 participants completed the TH-JSQ-P, with 1207 completing it twice to assess test-retest reliability. The TH-JSQ-P had good internal consistency reliability and test-retest ability with Cronbach's alpha value of 0.898 and intraclass correlation coefficient of 0.814. The content validity was acceptable. The ESS had correlation with sleep problems in almost all subscales except for sleep habit.

Conclusions

The TH-JSQ-P is a valid and reliable instrument for evaluating sleep problems in preschool children in Thailand. It has the potential to be extensively utilized to screen for sleep problems, thereby enabling healthcare professionals to identify them early and implement prompt management.

目的：本研究旨在将日本学龄前儿童睡眠问卷（JSQ-P）翻译成泰语版本并进行验证，同时评估与学龄前儿童睡眠问题相关的因素：方法：将日本学龄前儿童睡眠问卷（JSQ-P）跨文化改编为泰语版 JSQ-P（TH-JSQ-P），包括正译、调和、反译以及原作者的最终批准。调查对象为来自社区和临床环境的 2-6 岁儿童。学龄前儿童的家长在 10-14 天内两次完成 TH-JSQ-P 的填写，以评估其信度和效度。对六项效度测试进行了评估，包括面效度、内容效度、结构效度、构造效度、标准效度和预测效度。此外，还收集了人口统计学数据、埃普沃斯嗜睡量表（ESS）、医疗和行为问题以及社会经济状况，以便进行分析：共有 2613 名参与者完成了 TH-JSQ-P，其中 1207 人完成了两次以评估测试-再测可靠性。TH-JSQ-P具有良好的内部一致性信度和重测能力，Cronbach's alpha值为0.898，类内相关系数为0.814。内容效度可以接受。除睡眠习惯外，ESS 与几乎所有分量表中的睡眠问题都有相关性：TH-JSQ-P 是评估泰国学龄前儿童睡眠问题的有效而可靠的工具。结论：TH-JSQ-P 是评估泰国学龄前儿童睡眠问题的有效而可靠的工具，具有广泛应用于筛查睡眠问题的潜力，从而使医护人员能够及早发现睡眠问题并实施及时的管理。

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引用次数: 0

Recognizing red flags for alternative diagnoses in pediatric chorea beyond Sydenham's 识别小儿舞蹈症除西登汉姆症之外的其他诊断信号。

IF 1.4 4区医学 Q4 CLINICAL NEUROLOGY

Brain & Development

Pub Date : 2024-11-01 DOI: 10.1016/j.braindev.2024.08.005

Jennifer J. Guzmán-Porras , Oscar M. Espitia Segura , Lizeth V. Gómez Diaz

Background

Chorea is a common movement disorder in children, requiring thorough clinical assessment and appropriate tests for etiological diagnosis. Early identification of treatable conditions can lead to effective treatment, reducing morbidity and improving quality of life.

Objective

To describe the clinical, demographic, and epidemiological characteristics of children and adolescents diagnosed with chorea.

Methods

A retrospective cross-sectional study of pediatric patients treated in a fourth-level hospital in Bogotá, Colombia, from January 2008 to January 2022.

Results

81 patients with chorea were found. The most frequent etiologies were rheumatic fever (50.6 %), vascular chorea (29.3 %), and chorea secondary to lupus (11.1 %). Patients with chorea secondary to rheumatic fever (Sydenham's chorea) were older compared to other etiologies, 10.36 ± 3.41 years vs 8.29 ± 5.16, p = 0.037. The presence of another movement disorder or abnormalities during the physical examination suggests a different etiology from rheumatic fever. Moreover, the presence of psychiatric symptoms was similar in all etiologies. However, a correlation between age and psychiatric symptoms was observed with an odds ratio of 1.14 95 % CI 1.02–1.29 per year.

Conclusions

Red flags in Sydenham's chorea suggesting an alternate etiology are younger age, the presence of other abnormal movements or other findings in the neurological exam or in the magnetic resonance imaging. No significant statistical differences were found between the etiologies with the presence of neuro-psychiatric manifestations. Nevertheless, these manifestations are very frequent in abnormal movements. This study evidenced the positive correlation between age and the presence of psychiatric symptoms.

背景介绍舞蹈症是儿童常见的运动障碍，需要进行全面的临床评估和适当的病因诊断测试。及早发现可治疗的病症可促进有效治疗，降低发病率并提高生活质量：描述确诊为舞蹈症的儿童和青少年的临床、人口统计学和流行病学特征：方法：对2008年1月至2022年1月期间在哥伦比亚波哥大一家四级医院接受治疗的儿童患者进行回顾性横断面研究：结果：共发现 81 名舞蹈症患者。最常见的病因是风湿热（50.6%）、血管性舞蹈症（29.3%）和继发于红斑狼疮的舞蹈症（11.1%）。继发于风湿热的舞蹈症（西登纳姆舞蹈症）患者与其他病因的患者相比年龄较大，分别为 10.36 ± 3.41 岁 vs 8.29 ± 5.16 岁，P = 0.037。在体格检查中出现其他运动障碍或异常，表明病因与风湿热不同。此外，所有病因中出现精神症状的情况相似。然而，年龄与精神症状之间存在相关性，每年的几率比为 1.14 95 % CI 1.02-1.29：结论：年龄较小、存在其他异常运动或在神经系统检查或磁共振成像中出现其他发现，都是导致西登姆舞蹈症出现其他病因的信号。在出现神经-精神表现的病因之间没有发现明显的统计学差异。然而，这些表现在异常运动中非常常见。这项研究证明，年龄与出现精神症状之间存在正相关。

{"title":"Recognizing red flags for alternative diagnoses in pediatric chorea beyond Sydenham's","authors":"Jennifer J. Guzmán-Porras , Oscar M. Espitia Segura , Lizeth V. Gómez Diaz","doi":"10.1016/j.braindev.2024.08.005","DOIUrl":"10.1016/j.braindev.2024.08.005","url":null,"abstract":"<div><h3>Background</h3><div>Chorea is a common movement disorder in children, requiring thorough clinical assessment and appropriate tests for etiological diagnosis. Early identification of treatable conditions can lead to effective treatment, reducing morbidity and improving quality of life.</div></div><div><h3>Objective</h3><div>To describe the clinical, demographic, and epidemiological characteristics of children and adolescents diagnosed with chorea.</div></div><div><h3>Methods</h3><div>A retrospective cross-sectional study of pediatric patients treated in a fourth-level hospital in Bogotá, Colombia, from January 2008 to January 2022.</div></div><div><h3>Results</h3><div>81 patients with chorea were found. The most frequent etiologies were rheumatic fever (50.6 %), vascular chorea (29.3 %), and chorea secondary to lupus (11.1 %). Patients with chorea secondary to rheumatic fever (Sydenham's chorea) were older compared to other etiologies, 10.36 ± 3.41 years vs 8.29 ± 5.16, <em>p</em> = 0.037. The presence of another movement disorder or abnormalities during the physical examination suggests a different etiology from rheumatic fever. Moreover, the presence of psychiatric symptoms was similar in all etiologies. However, a correlation between age and psychiatric symptoms was observed with an odds ratio of 1.14 95 % CI 1.02–1.29 per year.</div></div><div><h3>Conclusions</h3><div>Red flags in Sydenham's chorea suggesting an alternate etiology are younger age, the presence of other abnormal movements or other findings in the neurological exam or in the magnetic resonance imaging. No significant statistical differences were found between the etiologies with the presence of neuro-psychiatric manifestations. Nevertheless, these manifestations are very frequent in abnormal movements. This study evidenced the positive correlation between age and the presence of psychiatric symptoms.</div></div>","PeriodicalId":56137,"journal":{"name":"Brain & Development","volume":"46 10","pages":"Pages 326-331"},"PeriodicalIF":1.4,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142333182","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"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

Sleep problems among Asian preschool children with neurodevelopmental disorders 患有神经发育障碍的亚洲学龄前儿童的睡眠问题。

IF 1.4 4区医学 Q4 CLINICAL NEUROLOGY

Brain & Development

Pub Date : 2024-11-01 DOI: 10.1016/j.braindev.2024.10.001

Mantita Tippawanich , Lunliya Thampratankul , Jariya Chuthapisith

Background

Sleep plays an important role in children's behavior, emotional control, and cognitive development. There is a higher prevalence of sleep problems in children with neurodevelopmental disorders (NDDs) compared to those without disease. There are a few studies regarding sleep problems among Thai preschoolers with NDDs. This study examined sleep problems in preschoolers with NDDs, compared with typically developing children (TD), and studied the association between sleep problems and behavioral problems among preschoolers with NDDs.

Methods

Sleep problems in 140 children aged 2 to 6 years with NDDs; autism spectrum disorders, attention deficit/hyperactivity disorder, global developmental delay, and developmental language disorder were evaluated by using the Thai version of Japanese Sleep Questionnaire for Preschoolers (TH-JSQ-P) and compared with 285 TD children at the same age. Behavioral problems in the NDDs group were evaluated by using the Child Behavioral Checklist (CBCL). Sleep and behavioral problem scores were analyzed by adjusted age, sex, underlying diseases, and medications used and compared between the two groups.

Results

The study found that 48 % of the TD group and 71 % of the NDDs group were identified as having sleep problems. The NDDs group had significantly higher total TH-JSQ-P score and in five subscales, including obstructive sleep apnea, restless leg syndrome-motor, parasomnias, daytime behaviors, and insomnia/circadian rhythm disorders. Total TH-JSQ-P score was found to have a moderate correlation with internalizing and externalizing behavioral problems.

Conclusion

Findings suggest that sleep problems are more common in preschool children with NDDs than in TD children and are associated with internalizing and externalizing behavioral problems.

背景：睡眠对儿童的行为、情绪控制和认知发展起着重要作用。与未患病的儿童相比，患有神经发育障碍（NDDs）的儿童中睡眠问题的发生率更高。有关泰国学龄前 NDD 儿童睡眠问题的研究为数不多。本研究对患有神经发育障碍的学龄前儿童与发育正常儿童（TD）的睡眠问题进行了比较，并研究了患有神经发育障碍的学龄前儿童的睡眠问题与行为问题之间的关联：使用泰语版《日本学龄前儿童睡眠问卷》（TH-JSQ-P）评估了140名2至6岁患有NDDs（自闭症谱系障碍、注意缺陷/多动障碍、全面发育迟缓和发育性语言障碍）的儿童的睡眠问题，并与285名同龄TD儿童进行了比较。使用儿童行为检查表（CBCL）评估了NDDs组儿童的行为问题。根据调整后的年龄、性别、基础疾病和所用药物对睡眠和行为问题得分进行分析，并在两组之间进行比较：研究发现，48% 的 TD 组和 71% 的 NDDs 组被认定有睡眠问题。NDDs组的TH-JSQ-P总分和五个分量表（包括阻塞性睡眠呼吸暂停、运动性不安腿综合征、寄生虫病、日间行为和失眠/昼夜节律紊乱）的得分明显更高。TH-JSQ-P总分与内化和外化行为问题呈中度相关：研究结果表明，睡眠问题在学龄前 NDD 儿童中比 TD 儿童更常见，并且与内化和外化行为问题相关。

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引用次数: 0

Color density spectral array findings on continuous EEG during therapeutic hypothermia in children with acute encephalopathy 急性脑病患儿治疗性低温期间连续脑电图的彩色密度谱阵列结果。

IF 1.4 4区医学 Q4 CLINICAL NEUROLOGY

Brain & Development

Pub Date : 2024-11-01 DOI: 10.1016/j.braindev.2024.08.002

Anna Shiraki , Hiroyuki Yamamoto , Atsuko Ohno , Sumire Kumai , Ryosuke Suzui , Fumi Sawamura , Masahiro Kawaguchi , Takeshi Suzuki , Yuki Maki , Yuji Ito , Tomohiko Nakata , Hiroyuki Kidokoro , Atsushi Numaguchi , Jun Natsume

Background: Quantitative EEG is frequently used to monitor children affected by acute encephalopathy (AE), with the expectation of providing comprehensive insights into continuous EEG monitoring. However, the potential of quantitative EEG for estimating outcomes in this context remains unclear. We sought reliable prognostic markers within the color density spectral array (CDSA) of the continuous EEG for AE-affected children undergoing therapeutic hypothermia (TH). Methods: This retrospective study analyzed CDSA data from eight scalp electrodes of 15 AE-affected children undergoing TH. Two CDSA features were investigated—high-frequency lines (HFLs) and periodic elevation in the low frequency band (PLFB)—along with the corresponding EEG characteristics. The inter-rater reliability for CDSA was assessed by four pediatric neurologists. Outcomes were grouped into either no/mild or severe decline in motor and cognitive functions, then compared with CDSA features. Results: The median EEG recording time was 114 (81–151) h per child. While at least 41 % of HFLs corresponded to typical sleep spindles, 94 % of PLFB aligned with cyclic changes in the amplitude of delta/theta waves on the raw EEG. Inter-rater reliability was higher for HFLs than for PLFB (kappa values: 0.69 vs. 0.46). HFLs were significantly more prevalent in children with no/mild decline than in children with severe decline (p = 0.017), whereas PLFB did not differ significantly (p = 0.33). Conclusions: This study provides preliminary evidence that reduced HFLs on CDSA predict unfavorable outcomes in AE-affected children undergoing TH. This suggests that maintaining high-frequency waves is critical for optimal brain function.

背景：定量脑电图经常用于监测受急性脑病（AE）影响的儿童，期望为连续脑电图监测提供全面的见解。然而，在这种情况下，定量脑电图在估计预后方面的潜力仍不明确。我们为接受治疗性低温（TH）的受 AE 影响的儿童寻找连续脑电图彩色密度谱阵列（CDSA）中可靠的预后标志物：这项回顾性研究分析了 15 名接受治疗性低温的 AE 受影响儿童的 8 个头皮电极的 CDSA 数据。研究了 CDSA 的两个特征--高频线（HFLs）和低频段周期性升高（PLFB）--以及相应的脑电图特征。四名儿科神经学家对 CDSA 的评分者间可靠性进行了评估。结果分为运动和认知功能无/轻度下降或严重下降，然后与 CDSA 特征进行比较：每个儿童的中位脑电图记录时间为 114 (81-151) h。至少 41% 的 HFLs 与典型的睡眠棘波相对应，而 94% 的 PLFB 与原始脑电图上 delta/theta 波振幅的周期性变化相对应。HFLs 的评分者间可靠性高于 PLFB（卡帕值：0.69 对 0.46）。HFLs在无/轻度衰退儿童中的发生率明显高于严重衰退儿童（p = 0.017），而PLFB则无明显差异（p = 0.33）：本研究提供了初步证据，表明 CDSA 的高频率降低可预测接受 TH 的受 AE 影响儿童的不良预后。这表明，保持高频波对于优化大脑功能至关重要。

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引用次数: 0

Efficacy and safety of buccal midazolam for seizures outside the hospital: Real-world clinical experience 口服咪达唑仑治疗医院外癫痫发作的有效性和安全性：真实世界的临床经验。

IF 1.4 4区医学 Q4 CLINICAL NEUROLOGY

Brain & Development

Pub Date : 2024-11-01 DOI: 10.1016/j.braindev.2024.09.005

Takuya Ueda , Masahiro Nishiyama , Hiroshi Yamaguchi , Kento Soma , Yusuke Ishida , Azusa Maruyama , Kandai Nozu , Hiroaki Nagase

Introduction

Buccal midazolam (buc MDL) is the first buccal mucosal delivery formulation applied for status epilepticus in Japan. Herein, we aimed to investigate the effectiveness and adverse events of buc MDL as a pre-hospital treatment for epileptic seizures in real-world clinical practice.

Methods

This study involved a retrospective review based on medical records. We included children who received buc MDL as pre-hospital treatment for epileptic seizures and were subsequently transported to the emergency department between April 2021 and November 2023.

Results

This study included 26 patients (136 episodes). The overall efficacy rate, which was defined as seizure cessation within 10 min after buc MDL administration with no recurrence within 30 min, was 43 %. Moreover, 70 % of the episodes did not require additional medications. None of the episodes required bag-mask ventilation or intubation following seizure cessation with buc MDL alone. The efficacy was decreased when buc MDL was administered longer than 15 min from seizure onset. Furthermore, the efficacy did not decrease as long as it was within 0.2–0.5 mg/kg, even if the dose was smaller than the appropriate dose for the specific age.

Conclusions

The response rate was significantly higher in episodes where buc MDL was administered within 15 min. Additionally, there was no concern regarding respiratory depression with buc MDL alone.

简介：颊黏膜咪达唑仑（buc MDL）是日本首个用于治疗癫痫状态的颊黏膜给药制剂。在此，我们旨在调查 buc MDL 在实际临床实践中作为癫痫发作院前治疗的有效性和不良事件：本研究根据病历进行回顾性分析。我们纳入了2021年4月至2023年11月期间接受buc MDL作为癫痫发作院前治疗并随后被送往急诊科的儿童：本研究共纳入 26 名患者（136 次发作）。总体有效率为 43%，即服用 buc MDL 后 10 分钟内癫痫发作停止，且 30 分钟内不再复发。此外，70%的发作无需额外用药。在仅使用 buc MDL 停止发作后，没有一次发作需要进行面罩通气或插管。从癫痫发作开始 15 分钟后再使用 buc MDL，疗效会有所下降。此外，只要剂量在 0.2-0.5 毫克/千克以内，即使剂量小于特定年龄的适当剂量，疗效也不会降低：结论：在15分钟内服用布克MDL的患者应答率明显更高。结论：在 15 分钟内注射 buc MDL 的病例应答率明显更高，此外，单独使用 buc MDL 不存在呼吸抑制的问题。

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引用次数: 0

A retrospective study on post-operative recovery of daily living activity after total corpus callosotomy 关于胼胝体全切除术后日常生活活动恢复情况的回顾性研究。

IF 1.4 4区医学 Q4 CLINICAL NEUROLOGY

Brain & Development

Pub Date : 2024-11-01 DOI: 10.1016/j.braindev.2024.09.006

Hiroki Tsuchiya , Takashi Shibata , Tatsuya Sasaki , Mari Akiyama , Tomoyuki Akiyama , Katsuhiro Kobayashi

Introduction

Post-operative complications of corpus callosotomy (CC) in children, prolonged hospitalization due to inactivity as acute disconnection syndrome is occasionally experienced. We aimed to clarify this issue and its risk factors with a hypothesis that electroencephalogram (EEG) findings as measures of functional lateralization may be among prognostic factors for post-operative recovery.

Materials and methods

Twenty-three patients with childhood-onset drug-resistant epilepsy who underwent total CC between April 2017 and December 2023 were included in the study and they were divided into two groups based on the duration of post-operative hospitalization as an indicator of recovery of daily living activity. We compared scalp EEG findings and the other factors including clinical characteristics between the two groups.

Results

Of 22 patients (14 males) without specific complications, post-operative hospitalization clustered in 9–14 days in 15 patients (Group A) with range 16–118 days in seven patients (Group B). The ratios of patients with non-lateralized spikes on pre-operative EEG and that of patients with symmetric background activity on post-operative EEG were significantly greater in Group B (7/7, 100 %; 6/7, 86 %, respectively) than in Group A (8/15, 53 %; 4/15, 27 %, respectively) (p = 0.038; p = 0.020, respectively, by Fisher's exact test), while other factors were not significantly different between the two groups.

Conclusions

Delayed recovery of living activity should be anticipated, especially in patients with non-lateralized epileptic discharges on pre-operative EEG.

导言：儿童胼胝体切开术（CC）术后并发症中，偶尔会出现因急性断裂综合征而无法活动导致的长期住院。我们旨在澄清这一问题及其风险因素，并假设脑电图（EEG）结果作为功能侧化的测量指标，可能是术后恢复的预后因素之一：研究纳入了在2017年4月至2023年12月期间接受全CC手术的23例儿童期耐药性癫痫患者，并根据术后住院时间作为日常生活活动恢复的指标将其分为两组。我们比较了两组患者的头皮脑电图结果和其他因素，包括临床特征：在 22 名无特殊并发症的患者（14 名男性）中，15 名患者（A 组）的术后住院时间集中在 9-14 天，7 名患者（B 组）的住院时间集中在 16-118 天。术前脑电图显示非侧位棘波的患者比例和术后脑电图显示对称背景活动的患者比例，B 组（分别为 7/7，100%；6/7，86%）明显高于 A 组（分别为 8/15，53%；4/15，27%）（经费雪精确检验，分别为 p = 0.038；p = 0.020），而其他因素在两组之间无明显差异：结论：尤其是术前脑电图显示有非侧位癫痫放电的患者，应预计到生活活动的延迟恢复。

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