Brain & Development最新文献

英文中文

Response to the letter: “AESD and vitamin therapy: The need for biomarkers and follow-up” 对这封信的回应：“AESD和维生素治疗：对生物标志物和随访的需求”。

IF 1.3 4区医学 Q4 CLINICAL NEUROLOGY

Brain & Development

Pub Date : 2025-12-01 Epub Date: 2025-10-16 DOI: 10.1016/j.braindev.2025.104463

Yuichi Abe , Tatsuya Takahashi , Itaru Hayakawa , Saeko Irie , Nobuaki Tsuiki , Atsuhi Nishioka , Hiroto Ida , Tsuyoshi Aihara , Kentaro Ide

引用次数: 0

Letter regarding the article “The impact of intraventricular hemorrhage on brainstem auditory function in preterm babies” 关于文章“脑室内出血对早产儿脑干听觉功能的影响”的来信。

IF 1.3 4区医学 Q4 CLINICAL NEUROLOGY

Brain & Development

Pub Date : 2025-12-01 Epub Date: 2025-10-17 DOI: 10.1016/j.braindev.2025.104474

Gül Yücel , Nur Yücel Ekici

引用次数: 0

Associations between cerebrospinal fluid pressure levels, clinical features, and MRI abnormalities in pediatric idiopathic intracranial hypertension: A retrospective study 儿童特发性颅内高压的脑脊液压力水平、临床特征和MRI异常之间的关系：一项回顾性研究

IF 1.3 4区医学 Q4 CLINICAL NEUROLOGY

Brain & Development

Pub Date : 2025-12-01 Epub Date: 2025-10-16 DOI: 10.1016/j.braindev.2025.104477

Hasan Emral , Tülay Kamaşak , İlker Eyüboğlu , Yusuf Emre Bostan , Ali Cansu

Background

Idiopathic intracranial hypertension (IIH) is a condition characterized by increased cerebrospinal fluid (CSF) pressure without an identifiable cause. Although neuroimaging features are often used to support diagnosis, the relationship between CSF pressure and MRI findings remains unclear, particularly in pediatric populations.

Objective

To investigate the association between CSF opening pressure and clinical as well as MRI features in children diagnosed with IIH.

Methods

We retrospectively reviewed 30 pediatric patients (aged 6–18 years) diagnosed with IIH between 2015 and 2020. Patients were divided into two groups based on CSF opening pressure: Group 1 (200–350 mm H₂O) and Group 2 (≥350 mm H₂O). Clinical symptoms and MRI features—including optic nerve sheath dilation, posterior globe flattening, optic nerve tortuosity, and transverse sinus stenosis—were compared between groups.

Results

The mean CSF opening pressure was 363.8 mm H₂O. No statistically significant differences were observed between groups with respect to demographic features, clinical symptoms, or MRI abnormalities (p > 0.05). The most frequent imaging findings were posterior globe flattening (73.3 %) and optic nerve sheath dilation (70 %). Spearman correlation analysis demonstrated a significant positive association between CSF opening pressure and optic nerve sheath dilation (r = 0.417, p = 0.022), with borderline correlations noted for optic nerve tortuosity (r = 0.358, p = 0.052) and transverse sinus stenosis (r = 0.438, p = 0.069).

Discussion

CSF opening pressure alone did not distinguish clinical or imaging features between groups. However, higher pressures correlated with optic nerve sheath dilation and showed trends toward tortuosity and venous stenosis, suggesting these may be secondary markers of disease severity.

Conclusion

Pediatric IIH appears multifactorial rather than purely pressure-driven. Multimodal evaluation is essential, and larger prospective studies are warranted to clarify the prognostic value of imaging correlates.

背景：特发性颅内高压（IIH）是一种以脑脊液（CSF）压力升高为特征的无明确原因的疾病。虽然神经影像学特征常用于支持诊断，但脑脊液压力与MRI结果之间的关系尚不清楚，特别是在儿科人群中。目的：探讨脑脊液开口压力与IIH患儿临床及MRI表现的关系。方法：我们回顾性分析了2015年至2020年间诊断为IIH的30例儿童患者（6-18岁）。根据脑脊液开口压力将患者分为两组：1组（200-350 mm H₂O）和2组（≥350 mm H₂O）。比较两组患者的临床症状和MRI表现，包括视神经鞘扩张、后球扁平、视神经扭曲和横窦狭窄。结果：脑脊液平均开口压力为363.8 mm H₂O。在人口学特征、临床症状或MRI异常方面，组间无统计学差异（p < 0.05）。最常见的影像学表现为后球变平（73.3%）和视神经鞘扩张（70%）。Spearman相关分析显示脑脊液开口压力与视神经鞘扩张呈显著正相关（r = 0.417, p = 0.022），视神经扭曲（r = 0.358, p = 0.052）和横窦狭窄（r = 0.438, p = 0.069）呈临界相关性。讨论：单独的脑脊液开口压力不能区分两组的临床或影像学特征。然而，较高的压力与视神经鞘扩张相关，并表现出扭曲和静脉狭窄的趋势，表明这些可能是疾病严重程度的次要标志。结论：儿童IIH是多因素的，而不是单纯的压力驱动。多模式评估是必要的，需要更大的前瞻性研究来阐明影像学相关因素的预后价值。

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引用次数: 0

Adrenoleukodystrophy: Current understanding of disease mechanisms, diagnosis, and therapeutic advances–a recent review 肾上腺脑白质营养不良：目前对疾病机制、诊断和治疗进展的理解-最近的综述

IF 1.3 4区医学 Q4 CLINICAL NEUROLOGY

Brain & Development

Pub Date : 2025-12-01 Epub Date: 2025-10-24 DOI: 10.1016/j.braindev.2025.104476

Chakresh Kumar Jain, Sarita Maurya, Pankaj Kumar Tripathi

Adrenoleukodystrophy (ALD) is a complex and devastating X-linked neurodegenerative disorder classified as rare brain disease with profound effects on patients and their families. It can manifest in various clinical forms, ranging from adult-onset adrenomyeloneuropathy (AMN) to rapidly developing childhood cerebral adrenoleukodystrophy (CCALD), each presenting unique diagnostic and treatment challenges. At the molecular level, ALD results from mutations in the ATP-binding cassette subfamily D member 1 (ABCD1) gene, which disrupts peroxisomal β-oxidation, leading to the accumulation of very long-chain fatty acids (VLCFAs) and subsequent neurological damage. Understanding these pathogenic mechanisms has led to significant improvements in diagnosis and treatment strategies. Advances such as newborn screening and molecular profiling have facilitated early detection and intervention, contributing to improved clinical outcomes. Therapeutic innovations, including gene therapy and hematopoietic stem cell transplantation (HSCT), hold promise for modifying disease progression and enhancing quality of life. Despite these developments, major challenges remain, including the identification of disease modifiers, discovery of new therapeutic targets, and establishing the personalized treatment strategies. This review summarizes the current understanding of ALD, including its clinical spectrum, molecular basis, diagnostic approaches, and emerging therapies. Ongoing research and interdisciplinary collaboration are crucial for establishing the effective management of this debilitating disorder.

肾上腺脑白质营养不良症（ALD）是一种复杂的、毁灭性的x连锁神经退行性疾病，被归类为罕见的脑部疾病，对患者及其家属有深远的影响。它可以表现为各种临床形式，从成人发病的肾上腺髓神经病变（AMN）到快速发展的儿童脑肾上腺白质营养不良（CCALD），每种都有独特的诊断和治疗挑战。在分子水平上，ALD是由atp结合盒亚家族D成员1 （ABCD1）基因突变引起的，该基因破坏过氧化物酶体β-氧化，导致甚长链脂肪酸（VLCFAs）的积累和随后的神经损伤。了解这些致病机制导致了诊断和治疗策略的重大改进。新生儿筛查和分子谱分析等进步促进了早期发现和干预，有助于改善临床结果。包括基因治疗和造血干细胞移植（HSCT）在内的治疗创新有望改变疾病进展并提高生活质量。尽管取得了这些进展，但主要的挑战仍然存在，包括确定疾病调节剂、发现新的治疗靶点和建立个性化的治疗策略。本文综述了目前对ALD的认识，包括其临床谱、分子基础、诊断方法和新兴治疗方法。持续的研究和跨学科合作对于建立这种使人衰弱的疾病的有效管理至关重要。

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引用次数: 0

Acute encephalopathy with biphasic seizures: Is cerebrospinal fluid glutamate truly specific? 急性脑病伴双相癫痫发作：脑脊液谷氨酸真的是特异性的吗？

IF 1.3 4区医学 Q4 CLINICAL NEUROLOGY

Brain & Development

Pub Date : 2025-12-01 Epub Date: 2025-10-14 DOI: 10.1016/j.braindev.2025.104466

Christian Messina

引用次数: 0

Development of the human cerebellum from midgestation to the perinatal period: A morphological study 人类小脑从妊娠中期到围产期的发育：形态学研究

IF 1.3 4区医学 Q4 CLINICAL NEUROLOGY

Brain & Development

Pub Date : 2025-12-01 Epub Date: 2025-10-10 DOI: 10.1016/j.braindev.2025.104453

Katsuyuki Yamaguchi, Takuya Yazawa

Introduction

The human cerebellum has large hemispheres (HS) and develops slowly over the pre- and postnatal periods. Several morphological studies have reported regional differences during the fetal period. This study aimed to investigate these differences by evaluating serial brain sections.

Materials and methods

Ten cerebellar samples aged 21–43 postmenstrual weeks (PW) were analyzed. Horizontal serial sections were cut and stained using the Klüver–Barrera method. After microscopic observations, a volumetric analysis was performed for the cerebellar cortex and medulla among the HS, vermis (VM), and flocculus (FL).

Results

The lamina dissecans (LD) was more prominent and lasted longer in the HS than in the VM and FL. The maturation of Purkinje cells and the accumulation of granule cells in the internal granular layer started earlier in the latter than in the former. Cortical differentiation was most delayed in the inferior semilunar and tonsillar lobules. Volumetric analysis showed that volume increased more in the HS than in the VM and FL after midgestation, and that it increased more intensively in the cortex than in the medulla in each region.

Conclusions

The human cerebellar cortex may develop through three stages in fetal period: (1) three-layers stage (before 20 PW); (2) five-layers stage (20–30 PW); (3) four-layers stage (after 30 PW). Particularly, the second stage is characterized by a transient appearance of the LD, which is most evident in the HS. Regional differences should be carefully considered when interpreting morphogenetic data.

人类小脑有较大的半球（HS），在产前和产后发育缓慢。一些形态学研究报告了胎儿时期的区域差异。本研究旨在通过评估连续脑切片来研究这些差异。材料与方法对21 ~ 43岁月经后小脑样本进行分析。水平序列切片采用kl ver - barrera法切割染色。显微镜观察后，对小脑皮层和髓质、蚓部（VM）和小叶（FL）进行体积分析。结果与VM和FL相比，HS的夹层更明显，持续时间更长，浦肯野细胞的成熟和颗粒细胞在内颗粒层的积累开始早于VM和FL。下半月小叶和扁桃体小叶的皮质分化最迟。体积分析显示，妊娠中期后脑区体积增加幅度大于后脑区和前脑区，且各区域皮层体积增加幅度大于髓质。结论胎儿期人小脑皮层发育可分为三个阶段：(1)三层发育阶段（20 PW前）；(2)五层阶段（20-30 PW）；(3)四层阶段（30pw后）。特别是，第二阶段的特征是LD的短暂出现，这在HS中最为明显。在解释形态发生数据时，应仔细考虑区域差异。

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引用次数: 0

Interaction with individuals with severe motor and intellectual disabilities and nurses' positive mental well-being 与严重运动和智力障碍患者的互动以及护士的积极心理健康

IF 1.3 4区医学 Q4 CLINICAL NEUROLOGY

Brain & Development

Pub Date : 2025-12-01 Epub Date: 2025-10-10 DOI: 10.1016/j.braindev.2025.104461

Yuto Arai , Sawako Ohba , Ryuki Kadekaru , Tohru Okanishi , Hisashi Noma , Yoshihiro Maegaki

Background

Severe motor and intellectual disabilities (SMID) involve intense physical and intellectual disabilities resulting in extreme dependence. Studies investigating the association between the experience of interacting with individuals with SMID and nurses' positive mental well-being are lacking. The comprehensive analysis of factors associated with the positive mental well-being of nurses who interact with individuals with SMID may enhance the nurses' quality of life and contribute to improving the quality of care provided to individuals with SMID.

Methods

We conducted a two-month cross-sectional online survey among 263 nurses at a university hospital in 2023. Happiness and resilience were measured using the Subjective Happiness Scale (SHS) and the short version of the Resilience Scale (RS-14), respectively. Multiple regression analysis was conducted to investigate the relationship between the interaction experience with individuals with SMID and the SHS and RS-14 scores, alongside other factors possibly influencing positive mental well-being.

Results

Regarding subjective happiness, no factor related to individuals with SMID was significantly associated with the SHS scores. However, regarding resilience, the number of years spent engaging with them was positively associated with the RS-14 scores [regression coefficient (B), 2.401; 95 % confidence interval (CI), 0.073 to 4.728; p = 0.043].

Conclusion

Interaction experiences with individuals with SMID were not associated with subjective happiness; however, they were associated with resilience. Our findings suggest the necessity for nurses to establish sustainable relationships with individuals with SMID.

重度运动和智力残疾（SMID）涉及严重的身体和智力残疾，导致极度依赖。调查与SMID患者互动的经验与护士积极心理健康之间关系的研究缺乏。综合分析与SMID患者互动的护士积极心理健康的相关因素，可以提高护士的生活质量，有助于提高对SMID患者的护理质量。方法对某大学附属医院263名护士进行为期2个月的横断面在线调查。幸福感和弹性分别采用主观幸福感量表（SHS）和短版弹性量表（RS-14）进行测量。通过多元回归分析，探讨与SMID个体的互动体验与SHS和RS-14评分的关系，以及其他可能影响积极心理健康的因素。结果主观幸福感方面，与SMID个体相关的因素与SHS得分无显著相关。然而，在弹性方面，与他们相处的年数与RS-14分数呈正相关[回归系数(B), 2.401；95%置信区间（CI）， 0.073 ~ 4.728；p = 0.043]。结论与SMID个体的互动体验与主观幸福感无关；然而，它们与适应力有关。我们的研究结果表明护士有必要与SMID患者建立可持续的关系。

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引用次数: 0

A pediatric case of anti-PF4 antibody-induced cerebral venous sinus thrombosis and thrombocytopenia following adenovirus infection: a literature review 小儿腺病毒感染后抗pf4抗体诱导脑静脉窦血栓形成和血小板减少症1例：文献回顾。

IF 1.3 4区医学 Q4 CLINICAL NEUROLOGY

Brain & Development

Pub Date : 2025-12-01 Epub Date: 2025-11-10 DOI: 10.1016/j.braindev.2025.104483

Kohei Nagai , Tadahiro Mitani , Yuta Kawahara , Hirofumi Oguma , Akira Gomi , Atsushi Yasumoto , Toshihiro Tajima , Kazuhiro Muramatsu , Hitoshi Osaka

Introduction

Vaccine-induced immune thrombocytopenia and thrombosis (VITT) is a rare disorder caused by antibodies against platelet factor 4 (PF4) triggered by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccines using non-replicable adenoviral vectors. It emerged during the pandemic, with patients typically presenting with thrombosis at uncommon sites, thrombocytopenia, and elevated D-dimer levels. VITT antibodies and heparin-dependent antibodies bind to distinct PF4 epitopes. Recently, VITT-like clinical, laboratory, and anti-PF4 antibody features have also been observed in patients with adenoviral infections. Only four pediatric cases of cerebral venous sinus thrombosis (CVST) have been reported.

Case report

The patient was a previously healthy 2-year-old girl with no history of heparin exposure or SARS-CoV-2 vaccination. She presented with fever and was diagnosed with adenovirus infection. The fever resolved by day 4, but by day 6 she became increasingly lethargic and experienced vomiting. On day 12, Laboratory data showed severe thrombocytopenia and elevated D-dimer levels. Computed tomography revealed CVST along with a secondary hemorrhage in the right temporal lobe. She underwent hematoma removal with external/internal decompression and was started on continuous intravenous unfractionated heparin, and she was switched to warfarin. The thrombus decreased, platelet count spontaneously increased. Platelet activation assays using acute-phase serum identified a PF4-dependent platelet-activating antibody.

Conclusion

We report a case of CVST in a 2-year-old girl following adenovirus infection. Unlike heparin-induced thrombocytopenia, where heparin exacerbates the condition, it is effective here by competitively inhibiting anti-PF4 antibody binding. In patients with prior adenovirus infection presenting with CVST and thrombocytopenia, anti-PF4 disorders should be considered.

疫苗诱导的免疫性血小板减少和血栓形成（VITT）是一种罕见的疾病，由使用不可复制腺病毒载体的严重急性呼吸综合征冠状病毒2 （SARS-CoV-2）疫苗引发的血小板因子4 （PF4）抗体引起。它在大流行期间出现，患者通常表现为罕见部位血栓形成、血小板减少和d -二聚体水平升高。VITT抗体和肝素依赖性抗体结合不同的PF4表位。最近，在腺病毒感染患者中也观察到类似vitt的临床、实验室和抗pf4抗体特征。目前仅报道了4例小儿脑静脉窦血栓形成（CVST）。病例报告：患者为既往健康的2岁女童，无肝素暴露史或SARS-CoV-2疫苗接种史。她出现发烧并被诊断为腺病毒感染。发烧在第4天消退，但在第6天，她变得越来越嗜睡并出现呕吐。第12天，实验室数据显示严重的血小板减少和d -二聚体水平升高。计算机断层扫描显示CVST伴继发性出血在右侧颞叶。她接受了体外/内部减压去除血肿，并开始持续静脉注射肝素，然后改用华法林。血栓减少，血小板计数自然增加。急性期血清血小板活化试验鉴定出pf4依赖性血小板活化抗体。结论：我们报告一例2岁女童在腺病毒感染后发生CVST。与肝素诱导的血小板减少症不同，肝素会加重病情，它通过竞争性抑制抗pf4抗体结合而有效。在既往腺病毒感染患者表现为CVST和血小板减少，抗pf4疾病应考虑。

{"title":"A pediatric case of anti-PF4 antibody-induced cerebral venous sinus thrombosis and thrombocytopenia following adenovirus infection: a literature review","authors":"Kohei Nagai , Tadahiro Mitani , Yuta Kawahara , Hirofumi Oguma , Akira Gomi , Atsushi Yasumoto , Toshihiro Tajima , Kazuhiro Muramatsu , Hitoshi Osaka","doi":"10.1016/j.braindev.2025.104483","DOIUrl":"10.1016/j.braindev.2025.104483","url":null,"abstract":"<div><h3>Introduction</h3><div>Vaccine-induced immune thrombocytopenia and thrombosis (VITT) is a rare disorder caused by antibodies against platelet factor 4 (PF4) triggered by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) vaccines using non-replicable adenoviral vectors. It emerged during the pandemic, with patients typically presenting with thrombosis at uncommon sites, thrombocytopenia, and elevated D-dimer levels. VITT antibodies and heparin-dependent antibodies bind to distinct PF4 epitopes. Recently, VITT-like clinical, laboratory, and anti-PF4 antibody features have also been observed in patients with adenoviral infections. Only four pediatric cases of cerebral venous sinus thrombosis (CVST) have been reported.</div></div><div><h3>Case report</h3><div>The patient was a previously healthy 2-year-old girl with no history of heparin exposure or SARS-CoV-2 vaccination. She presented with fever and was diagnosed with adenovirus infection. The fever resolved by day 4, but by day 6 she became increasingly lethargic and experienced vomiting. On day 12, Laboratory data showed severe thrombocytopenia and elevated D-dimer levels. Computed tomography revealed CVST along with a secondary hemorrhage in the right temporal lobe. She underwent hematoma removal with external/internal decompression and was started on continuous intravenous unfractionated heparin, and she was switched to warfarin. The thrombus decreased, platelet count spontaneously increased. Platelet activation assays using acute-phase serum identified a PF4-dependent platelet-activating antibody.</div></div><div><h3>Conclusion</h3><div>We report a case of CVST in a 2-year-old girl following adenovirus infection. Unlike heparin-induced thrombocytopenia, where heparin exacerbates the condition, it is effective here by competitively inhibiting anti-PF4 antibody binding. In patients with prior adenovirus infection presenting with CVST and thrombocytopenia, anti-PF4 disorders should be considered.</div></div>","PeriodicalId":56137,"journal":{"name":"Brain & Development","volume":"47 6","pages":"Article 104483"},"PeriodicalIF":1.3,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145490967","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

Reply to “Understanding cancer risk in severe motor and intellectual disabilities: The role of external influences” 回复“了解严重运动和智力残疾的癌症风险：外部影响的作用”。

IF 1.3 4区医学 Q4 CLINICAL NEUROLOGY

Brain & Development

Pub Date : 2025-12-01 Epub Date: 2025-10-16 DOI: 10.1016/j.braindev.2025.104470

Hiroshi Terashima , Yoshiaki Saito

引用次数: 0

Etiology-specific diagnosis of cytotoxic lesions of the corpus callosum (CLOCC) in a single-center pediatric cohort: diagnostic challenges and outcome characteristics 单中心儿童队列中胼胝体细胞毒性病变（CLOCC）的病因特异性诊断：诊断挑战和结果特征

IF 1.3 4区医学 Q4 CLINICAL NEUROLOGY

Brain & Development

Pub Date : 2025-12-01 Epub Date: 2025-10-26 DOI: 10.1016/j.braindev.2025.104472

Yavuz Ataş , Cenk Eraslan , Tuğce Ince , Javid Islamli , Pınar Öcbe , Dilara Ece Toprak , Gürsel Şen , Cemile Büşra Ölçülü , Özlem Yilmaz , Özlem Acar , Güliz Çırpanlı Ari , Gamze Görgülü , Pınar Yazıcı Özkaya , Bülent Karapinar , Coşkun Ekemen , Zümrüt Şahbudak Bal , Erdem Şimşek , Seda Kanmaz , Sanem Yilmaz , Cem Çalli , Hasan Tekgül

Objective

To evaluate the clinical and radiological characteristics of cytotoxic lesions of the corpus callosum (CLOCC) in a pediatric cohort.

Methods

A pediatric CLOCC cohort consisting of 16 patients was derived from the radiology database of a single tertiary institution, Ege Children's Hospital. The CLOCC diagnosis was based on previously established magnetic resonance imaging (MRI) criteria by Starkey et al.: (i) a small, round lesion centered in the splenium; (ii) a lesion centered in the splenium extending laterally through adjacent callosal fibers; or (iii) a lesion located posteriorly in the splenium extending anteriorly. The MRI characteristics of the study cohort were re-evaluated with those of other neurologic disorder groups with corpus callosum involvement: vascular lesions (n = 200); demyelinating disorders (n = 114); autoimmune encephalitis (n = 23); traumatic brain injury (n = 151). Etiology-specific diagnostic approaches, therapeutic challenges, and clinical outcomes were also analyzed.

Results

All patients displayed characteristic MRI features of CLOCC, including high signal on T2-weighted images, isointense-to-hypointense signal on T1-weighted images, and restricted diffusion on diffusion-weighted imaging (DWI), without contrast enhancement. The CLOCC lesions resolved within a median follow-up of 20 days (range: 5–150 days). The cohort was classified as infection-related CLOCC (62.5 %) and non-infectious CLOCC (37.5 %). The most frequent neurological symptoms at presentation were seizures (50 %) and altered consciousness (43.8 %). Immunotherapy was administered to 37.5 % of patients. None developed CLOCC-related neurological sequelae during the follow-up period.

Conclusion

Pediatric CLOCC is predominantly associated with symptoms related to underlying etiologic diseases, presenting diagnostic and therapeutic challenges in certain neurological conditions. The CLOCC lesions exhibit highly homogeneous MRI findings, though clinical outcomes and lesion resolution times vary depending on specific etiologies.

目的：探讨小儿胼胝体细胞毒性病变（CLOCC）的临床和影像学特点。方法：一个由16例患者组成的儿科CLOCC队列来自单一三级机构Ege儿童医院的放射学数据库。CLOCC的诊断是基于Starkey等人先前建立的磁共振成像（MRI）标准：(i)以脾为中心的小而圆的病变；（ii）以脾为中心，通过相邻胼胝体纤维向外侧延伸的病变；或（iii）位于脾脏后方并向前延伸的病变。将研究队列的MRI特征与其他胼胝体受累的神经系统疾病组的MRI特征重新评估：血管病变（n = 200）；脱髓鞘疾病（n = 114）；自身免疫性脑炎（n = 23）；创伤性脑损伤（n = 151）。对病因特异性诊断方法、治疗挑战和临床结果也进行了分析。结果：所有患者均表现为CLOCC的特征性MRI表现，包括t2加权高信号、t1加权等低信号、DWI弥散受限，未见增强。CLOCC病变在中位随访20天（范围：5-150天）内消退。该队列分为感染相关CLOCC（62.5%）和非感染性CLOCC（37.5%）。发病时最常见的神经症状是癫痫发作（50%）和意识改变（43.8%）。37.5%的患者接受免疫治疗。在随访期间没有发生clocc相关的神经系统后遗症。结论：儿童CLOCC主要与潜在病因疾病相关的症状相关，在某些神经系统疾病中提出诊断和治疗挑战。CLOCC病变表现出高度均匀的MRI表现，尽管临床结果和病变消退时间因具体病因而异。

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