Pediatric neurology最新文献_第2页

IF 3.2 3区医学

Pediatric neurology Pub Date : 2024-09-06 DOI: 10.1016/S0887-8994(24)00314-X

引用次数: 0

Efficacy and Safety of Rituximab Treatment for Anti-N-Methyl-d-Aspartate Receptor Encephalitis Without Tumor in Children 利妥昔单抗治疗儿童无肿瘤抗 N-甲基-d-天冬氨酸受体脑炎的有效性和安全性

IF 3.2 3区医学

Pediatric neurology Pub Date : 2024-09-06 DOI: 10.1016/j.pediatrneurol.2024.09.001

{"title":"Efficacy and Safety of Rituximab Treatment for Anti-N-Methyl-d-Aspartate Receptor Encephalitis Without Tumor in Children","authors":"","doi":"10.1016/j.pediatrneurol.2024.09.001","DOIUrl":"10.1016/j.pediatrneurol.2024.09.001","url":null,"abstract":"<div><h3>Background</h3><div>To evaluate the efficacy and safety of rituximab treatment for anti-<em>N</em>-methyl-<span>d</span>-aspartate receptor (NMDAR) encephalitis without tumor in children.</div></div><div><h3>Methods</h3><div>Eighteen pediatric patients with NMDAR encephalitis treated with rituximab after failure of intravenous immunoglobulin (IVIG) and methylprednisolone treatment were analyzed retrospectively in terms of their medical history, clinical features, laboratory examination results, and treatments. The modified Rankin scale (mRS) score, peripheral blood CD19+ B cells, recurrence, and adverse events were used to evaluate the efficacy and safety of rituximab.</div></div><div><h3>Results</h3><div>The patients were treated with rituximab 3.2 ± 1.0 days after the end of IVIG and methylprednisolone treatment. After initial rituximab treatment for four weeks, the mRS score and number of CD19+ B cells in all patients were significantly lower than those before treatment (<em>P</em> < 0.05). At the last follow-up (44.1 months, 17.7 S.D.), all patients had recovered well (mRS ≤2), 14 patients (77.8%) recovered completely (mRS = 0), three patients had recurrent seizures, and one patient had mental and language impairment. Two patients had transient mild adverse events during infusion, and none of the other patients experienced severe adverse events during hospitalization or follow-up.</div></div><div><h3>Conclusions</h3><div>Rituximab appears safe and may be effective for the treatment of anti-NMDAR encephalitis without tumor in children refractory to first-line agents.</div></div>","PeriodicalId":19956,"journal":{"name":"Pediatric neurology","volume":null,"pages":null},"PeriodicalIF":3.2,"publicationDate":"2024-09-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142323182","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

Editorial Board and Masthead 编辑委员会和刊头

IF 3.2 3区医学

Pediatric neurology Pub Date : 2024-09-05 DOI: 10.1016/S0887-8994(24)00292-3

引用次数: 0

Research Priorities for Children With Hypoxic-Ischemic Encephalopathy or Myelomeningocele: A Survey of Parents 缺氧缺血性脑病或骨髓缺失症患儿的研究重点：家长调查

IF 3.2 3区医学

Pediatric neurology Pub Date : 2024-08-26 DOI: 10.1016/j.pediatrneurol.2024.08.005

引用次数: 0

A Case Series of Children With Medulloblastoma Depicting the Disparities in Care and the Challenges in the Detection and Treatment of Pediatric Central Nervous System Tumors in Low-Resource Settings: A Case Study of Uganda 髓母细胞瘤患儿的病例系列，描绘了在资源匮乏地区儿童中枢神经系统肿瘤的检测和治疗中存在的护理差异和挑战：乌干达案例研究

IF 3.2 3区医学

Pediatric neurology Pub Date : 2024-08-23 DOI: 10.1016/j.pediatrneurol.2024.08.006

{"title":"A Case Series of Children With Medulloblastoma Depicting the Disparities in Care and the Challenges in the Detection and Treatment of Pediatric Central Nervous System Tumors in Low-Resource Settings: A Case Study of Uganda","authors":"","doi":"10.1016/j.pediatrneurol.2024.08.006","DOIUrl":"10.1016/j.pediatrneurol.2024.08.006","url":null,"abstract":"<div><h3>Background</h3><p>Primary central nervous system tumors are the second most common cancer among children in high-income countries (HICs). These tumors are also the leading cause of cancer-related deaths in children in this setting. Studies from HICs report gliomas as the most common pediatric cancer. However, there is paucity of data from low- and middle-income countries as not many publications have been made in this field.</p></div><div><h3>Methods</h3><p>The objective was to describe the disparities in detection, treatment, and survival of children with central nervous system tumors in low-income countries (LICs) when compared with HICs, using a case series. A retrospective chart review of three children treated for medulloblastoma in Uganda was done. In addition, a review of the literature about management of pediatric central nervous system tumors in both LICs and HICs was conducted.</p></div><div><h3>Results</h3><p>There are no quantifiable results for this case series.</p></div><div><h3>Conclusion</h3><p>There are notable differences in the quality of care for children with pediatric central nervous system tumors in LICs when compared with HICs. In Uganda, the challenges in management of these children include few multidisciplinary specialists, long distance from the neurosurgery centers, and difficulties in making a correct pathologic diagnosis, among others.</p></div>","PeriodicalId":19956,"journal":{"name":"Pediatric neurology","volume":null,"pages":null},"PeriodicalIF":3.2,"publicationDate":"2024-08-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142271321","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

Early Biomarkers in the Prediction of Later Functional Impairment in Preterm Children With Cerebral Palsy 预测早产脑瘫儿童日后功能障碍的早期生物标志物

IF 3.2 3区医学

Pediatric neurology Pub Date : 2024-08-23 DOI: 10.1016/j.pediatrneurol.2024.08.013

{"title":"Early Biomarkers in the Prediction of Later Functional Impairment in Preterm Children With Cerebral Palsy","authors":"","doi":"10.1016/j.pediatrneurol.2024.08.013","DOIUrl":"10.1016/j.pediatrneurol.2024.08.013","url":null,"abstract":"<div><h3>Background</h3><p>To identify early biomarkers that could predict later functional capabilities in preterm children with later cerebral palsy (CP).</p></div><div><h3>Methods</h3><p>Data from 968 preterm children with later CP were extracted from the Canadian Cerebral Palsy Registry. One hundred eighty-two infants were born before 27 weeks of gestation, 461 infants were born between 27 and 33 weeks, and 325 infants were born between 34 and 37 weeks. Univariate and chi-square analyses were conducted to measure the association between early objective biomarkers and later mobility status defined as Gross Motor Function Classification System (GMFCS) levels IV and V as well as tube feeding dependence.</p></div><div><h3>Results</h3><p>Univariate analysis suggested no significant association between GMFCS levels IV and V or impaired feeding status and bilateral white matter injury on magnetic resonance imaging, high-grade intraventricular hemorrhage on head ultrasound, chorioamnionitis, a birth weight of 1000 to 1500 g or <1000 g, as well as an Apgar score of ≤5 at five minutes of life. Similar results were found for gestational age <28 weeks at birth. Only a significant association between GMFCS levels IV and V and a cord or first hour of life pH of ≤7 was reported (mobility status: odds ratio [OR] 1.95, 95% confidence interval [CI] 1.09 to 3.57) and feeding status: OR 2.23, CI 0.97 to 4.65)].</p></div><div><h3>Conclusions</h3><p>Prediction of functional outcomes based on specific early biomarkers appears hard to obtain in children with CP born preterm in contrast to those born at term. The complications and causal pathways inherent to prematurity may contribute to making prognostication less determinant.</p></div>","PeriodicalId":19956,"journal":{"name":"Pediatric neurology","volume":null,"pages":null},"PeriodicalIF":3.2,"publicationDate":"2024-08-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142228736","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

A Complex Presentation of Vestibular Paroxysmia in an Adolescent With Wolff- Parkinson-White Syndrome 患有沃尔夫-帕金森-怀特综合征的青少年前庭阵痛的复杂表现。

IF 3.2 3区医学

Pediatric neurology Pub Date : 2024-08-22 DOI: 10.1016/j.pediatrneurol.2024.08.010

{"title":"A Complex Presentation of Vestibular Paroxysmia in an Adolescent With Wolff- Parkinson-White Syndrome","authors":"","doi":"10.1016/j.pediatrneurol.2024.08.010","DOIUrl":"10.1016/j.pediatrneurol.2024.08.010","url":null,"abstract":"<div><p>Vestibular paroxysmia is an episodic vestibular disorder resulting from compression or irritation of the eighth cranial nerve. This disorder is a rare and difficult diagnosis in children. We report the case of a 16-year-old adolescent male with a history of syncope and coronavirus disease 2019 infection four months prior who presented with intermittent episodes of <em>vertigo</em> and unsteadiness several times a week. These events started abruptly, and he appeared frozen. However, he remained conscious and was able to answer questions. He subsequently resumed normal activity in less than a minute without seizure stigmata or postictal period. His general and neurological examinations were unremarkable. Extensive diagnostic evaluation yielded negative results, except for an electrocardiogram consistent with Wolff-Parkinson-White syndrome. However, his symptoms persisted after cardiac ablation, suggesting they were not related to this arrhythmia. Following unsuccessful trials with various medications, his symptoms resolved with carbamazepine. Early recognition and appropriate treatment of this condition could substantially improve the quality of life for affected individuals.</p></div>","PeriodicalId":19956,"journal":{"name":"Pediatric neurology","volume":null,"pages":null},"PeriodicalIF":3.2,"publicationDate":"2024-08-22","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142146139","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

Adequacy of an In–Neonatal Intensive Care Unit 1T Magnetic Resonance Imaging Compared With 3T Magnetic Resonance Imaging for Clinical Management 新生儿重症监护室 1T 磁共振成像与 3T 磁共振成像在临床管理中的充分性比较

IF 3.2 3区医学

Pediatric neurology Pub Date : 2024-08-16 DOI: 10.1016/j.pediatrneurol.2024.08.004

{"title":"Adequacy of an In–Neonatal Intensive Care Unit 1T Magnetic Resonance Imaging Compared With 3T Magnetic Resonance Imaging for Clinical Management","authors":"","doi":"10.1016/j.pediatrneurol.2024.08.004","DOIUrl":"10.1016/j.pediatrneurol.2024.08.004","url":null,"abstract":"<div><h3>Background</h3><p>This retrospective study aims to assess the added diagnostic utility and clinical value of a 3-Tesla neonatal brain magnetic resonance imaging after obtaining a 1-Tesla magnetic resonance imaging within the neonatal intensive care unit.</p></div><div><h3>Methods</h3><p>A cohort of 34 infants had an initial 1-Tesla magnetic resonance imaging and repeat imaging within 14 days in a 3-Tesla scanner. All infants were admitted to the level III neonatal intensive care unit at Brigham and Women's Hospital, and all images were interpreted by pediatric neuroradiologists.</p></div><div><h3>Results</h3><p>For 31 infants (91%), the 3-Tesla magnetic resonance imaging showed similar or expected evolution of known findings found on 1 Tesla. For infants with change between the 1-Tesla and 3-Tesla imaging results, there was no clinical impact.</p></div><div><h3>Conclusion</h3><p>Images from 1-Tesla magnetic resonance imaging were sufficient for characterizing a wide range of neonatal brain injuries and abnormalities and repeated 3-Tesla magnetic resonance imaging did not yield further clinical benefit.</p></div>","PeriodicalId":19956,"journal":{"name":"Pediatric neurology","volume":null,"pages":null},"PeriodicalIF":3.2,"publicationDate":"2024-08-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142162118","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

Exploring the Clinical Utility of Targeted MECP2 Testing in Real-World Practice 探索 MECP2 靶向测试在实际工作中的临床实用性

IF 3.2 3区医学

Pediatric neurology Pub Date : 2024-08-14 DOI: 10.1016/j.pediatrneurol.2024.08.001

{"title":"Exploring the Clinical Utility of Targeted MECP2 Testing in Real-World Practice","authors":"","doi":"10.1016/j.pediatrneurol.2024.08.001","DOIUrl":"10.1016/j.pediatrneurol.2024.08.001","url":null,"abstract":"<div><h3>Background</h3><p>This study aimed to explore the clinical utility of targeted <em>MECP2</em> testing in a large cohort of females with neurodevelopmental delays. Our aim was to identify suitable candidates for testing based on prevailing diagnostic criteria.</p></div><div><h3>Methods</h3><p>Eligible participants with global developmental delay/arrest or regression before age 36 months underwent <em>MECP2</em> testing. <em>MECP2</em>-positive patients were further categorized based on Rett syndrome (RTT) diagnostic criteria, including typical, atypical, possible, and unclassified, to assess disease typicality and progression with respect to age.</p></div><div><h3>Results</h3><p>Of the 683 patients, 162 (23.7%) were diagnosed with <em>MECP2</em>-related RTT. Global developmental delay was the predominant initial symptom in approximately 75% of the cohort with developmental arrest/regression at testing. Symptoms emerged before age six months in 14 patients (8.6%). The average age at the time of <em>MECP2</em> testing was 3.7 years, with 31.5% of the patients tested under two years. Of those under two years, 15 were initially categorized into the unclassified group; however, 12 were later reclassified into the typical/atypical RTT groups based on follow-up evaluation. Among the 119 patients monitored beyond age five years, 80% displayed typical RTT symptoms, 10 remained unclassified, and 9.8% had exonic deletions, posing challenges for detection using next-generation sequencing.</p></div><div><h3>Conclusions</h3><p>Targeted <em>MECP2</em> testing has emerged as a clinically valuable tool with a high diagnostic yield, including the identification of small deletions. Given that younger patients may not always meet the classic RTT criteria, this study recommends targeted <em>MECP2</em> testing in younger patients without typical RTT features.</p></div>","PeriodicalId":19956,"journal":{"name":"Pediatric neurology","volume":null,"pages":null},"PeriodicalIF":3.2,"publicationDate":"2024-08-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142162117","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

A Case Series of Novel Monogenic Abnormalities Associated With Developmental Epileptic Encephalopathy With Spike-and-Wave Activation in Sleep 与发育性癫痫脑病相关的新型单基因异常病例系列，伴有睡眠中的棘波和波激活

IF 3.2 3区医学

Pediatric neurology Pub Date : 2024-08-12 DOI: 10.1016/j.pediatrneurol.2024.08.003

引用次数: 0