European Journal of Paediatric Neurology最新文献

筛选
英文 中文
Spatiotemporal coordination in children with unilateral cerebral palsy: Insights from a bimanual goal-directed task 单侧脑瘫儿童的时空协调能力:双臂目标定向任务的启示
IF 2.3 3区 医学
European Journal of Paediatric Neurology Pub Date : 2024-10-06 DOI: 10.1016/j.ejpn.2024.10.003
Lisa Mailleux , Lisa Decraene , Alexandra Kalkantzi , Lize Kleeren , Monica Crotti , Anja Van Campenhout , Geert Verheyden , Els Ortibus , Dido Green , Katrijn Klingels , Hilde Feys
{"title":"Spatiotemporal coordination in children with unilateral cerebral palsy: Insights from a bimanual goal-directed task","authors":"Lisa Mailleux ,&nbsp;Lisa Decraene ,&nbsp;Alexandra Kalkantzi ,&nbsp;Lize Kleeren ,&nbsp;Monica Crotti ,&nbsp;Anja Van Campenhout ,&nbsp;Geert Verheyden ,&nbsp;Els Ortibus ,&nbsp;Dido Green ,&nbsp;Katrijn Klingels ,&nbsp;Hilde Feys","doi":"10.1016/j.ejpn.2024.10.003","DOIUrl":"10.1016/j.ejpn.2024.10.003","url":null,"abstract":"<div><h3>Background</h3><div>In children with unilateral cerebral palsy (uCP), bimanual assessments mostly focus on qualitative assessments of the impaired upper limb during bimanual tasks, which do not capture the spatiotemporal coordination between both hands. Hence, we aimed to advance our understandings in spatiotemporal coordination in children with uCP compared to typically developing children (TDC) using a bimanual, asymmetrical, goal-directed task.</div></div><div><h3>Participants and methodology</h3><div>In this observational study, thirty-seven children with uCP (11y8m±2y10m, 20 males, 16 right-sided uCP, Manual Ability Classification System level I = 23, II = 11, III = 3) and 37 age and sex-matched TDC opened a box with one hand and pressed a button inside using the opposite hand. Spatiotemporal bimanual (movement time, temporal coupling, movement overlap, goal synchronisation) and unimanual (movement time, path length and smoothness) parameters were extracted. Between groups comparisons were investigated using a two-way mixed ANCOVA with age as covariate (<em>α</em> &lt; 0.05). Additionally, correlation coefficients between unimanual and bimanual parameters were calculated.</div></div><div><h3>Results</h3><div>Compared to TDC, children with uCP were slower (p = 0.01, η<sub>p</sub><sup>2</sup> = 0.13) and presented unimanual spatiotemporal deficits in both upper limbs (p &lt; 0.03, η<sub>p</sub><sup>2</sup>&gt;0.10), which worsened in children with lower manual abilities (p &lt; 0.04, η<sub>p</sub><sup>2</sup>&gt;0.19). However, they did not differ in bimanual coupling (p &gt; 0.31, η<sub>p</sub><sup>2</sup>&lt;0.03). Furthermore, slower movement time was related with increased unimanual spatiotemporal deficits bilaterally (r = 0.34–0.80, p = 0.001–0.04), suggesting that reduced performance at both upper limbs contributes to bimanual difficulties in children with uCP.</div></div><div><h3>Conclusions</h3><div>The bilateral reduced spatiotemporal performance, related to longer bimanual movement time, stresses the importance to assess and treat both upper limbs in children with uCP.</div></div>","PeriodicalId":50481,"journal":{"name":"European Journal of Paediatric Neurology","volume":"53 ","pages":""},"PeriodicalIF":2.3,"publicationDate":"2024-10-06","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142442397","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
Predictive value of brain MRI for neurodevelopmental outcome in infants with severe unconjugated hyperbilirubinemia: A systematic review 脑磁共振成像对严重未结合高胆红素血症婴儿神经发育结局的预测价值:系统综述。
IF 2.3 3区 医学
European Journal of Paediatric Neurology Pub Date : 2024-09-27 DOI: 10.1016/j.ejpn.2024.09.010
Noortje M. van der Meulen , Karin L. Meijers , Jeroen Dudink , Laura A. van de Pol
{"title":"Predictive value of brain MRI for neurodevelopmental outcome in infants with severe unconjugated hyperbilirubinemia: A systematic review","authors":"Noortje M. van der Meulen ,&nbsp;Karin L. Meijers ,&nbsp;Jeroen Dudink ,&nbsp;Laura A. van de Pol","doi":"10.1016/j.ejpn.2024.09.010","DOIUrl":"10.1016/j.ejpn.2024.09.010","url":null,"abstract":"<div><h3>Context</h3><div>Debate exists regarding predictive value of brain MRI for long-term neurodevelopmental outcome (NDO) in infants with severe unconjugated hyperbilirubinemia (above exchange transfusion levels).</div></div><div><h3>Objective</h3><div>To investigate whether MRI findings among (pre-)term infants with severe unconjugated hyperbilirubinemia can predict NDO at ≥ 12 months and determine optimal timing for MRI.</div></div><div><h3>Data sources</h3><div>PubMed and Embase. Last update: June 14, 2024.</div></div><div><h3>Study selection</h3><div>Studies in which (pre-)term infants with severe unconjugated hyperbilirubinemia who underwent an MRI before 24 months and had a reported NDO at ≥ 12 months were included.</div></div><div><h3>Data extraction</h3><div>Patient characteristics, MRI and NDO details were extracted.</div></div><div><h3>Results</h3><div>The search yielded 732 studies, of which 22 were included. Individual patient information was obtained for 120 infants (MRI-timing: early (≤6 weeks) n = 75, late (&gt;6 weeks) n = 19, unknown n = 26). Positive predictive value (PPV) of abnormal MRI in the total group for impaired NDO was high (77.5 %). The PPV of late compared to early MRI was much higher, 92.3 % versus 71.7 %. Negative predictive value of normal MRI for normal NDO in the total group was low (29.0 %) and again higher in late compared to early MRI, 50.0 % versus 27.3 %.</div></div><div><h3>Limitations</h3><div>Quantitative synthesis of results was impossible due to large heterogeneity in study designs. Furthermore, selection bias towards patients with impaired outcome might have influenced our results.</div></div><div><h3>Conclusions</h3><div>Brain MRI can serve as prognostic tool for NDO in infants with severe unconjugated hyperbilirubinemia, both in early and late stages, but each timing has inherent constraints. Further prospective studies are necessary.</div></div>","PeriodicalId":50481,"journal":{"name":"European Journal of Paediatric Neurology","volume":"53 ","pages":"Pages 49-60"},"PeriodicalIF":2.3,"publicationDate":"2024-09-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142376272","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Somatosensory profile in individuals with duchenne muscular dystrophy: A quantitative sensory testing (QST) study 杜兴氏肌肉萎缩症患者的体感特征:定量感觉测试 (QST) 研究
IF 2.3 3区 医学
European Journal of Paediatric Neurology Pub Date : 2024-09-19 DOI: 10.1016/j.ejpn.2024.09.007
Meihuan Huang , Ruiqing Cui , Yanfei Xie , Chunming Zhou , Turong Chen , Yujuan Wang , Guojun Yun
{"title":"Somatosensory profile in individuals with duchenne muscular dystrophy: A quantitative sensory testing (QST) study","authors":"Meihuan Huang ,&nbsp;Ruiqing Cui ,&nbsp;Yanfei Xie ,&nbsp;Chunming Zhou ,&nbsp;Turong Chen ,&nbsp;Yujuan Wang ,&nbsp;Guojun Yun","doi":"10.1016/j.ejpn.2024.09.007","DOIUrl":"10.1016/j.ejpn.2024.09.007","url":null,"abstract":"<div><h3>Objective</h3><div>This study aimed to quantify somatosensory profiles in individuals with Duchenne muscular dystrophy (DMD).</div></div><div><h3>Methods</h3><div>We included 28 participants with genetically confirmed DMD (aged 8–17 years), 14 with chronic pain (DMD-CP), and 14 without pain (DMD-NP), compared to 13 healthy controls (HC) matched for age and sex. Three quantitative sensory testing (QST) modalities were examined: pressure pain threshold (PPT), temporal summation of pain (TSP) and conditioned pain modulation (CPM). Characteristics related to chronic pain, fatigue, psychological distress, and health-related quality of life were assessed using questionnaires.</div></div><div><h3>Results</h3><div>Decreased PPTs were found in both DMD cohorts across body areas commonly affected by pain (rectus femoris, medial gastrocnemius, paraspinal muscles, upper trapezius), as well as in a less frequently affected remote area (thenar eminence), compared to HCs (p &lt; 0.001). The DMD-CP group exhibited greater TSP compared to HCs (p = 0.025). There were no differences in CPM effects between DMD groups and HCs. No differences were detected in all QST measures between DMD-CP and DMD-NP.</div></div><div><h3>Significance</h3><div>This study is the first to explore the somatosensory profile in DMD. Preliminary evidence suggests that generalized hyperalgesia may be a common feature in DMD regardless of pain status. QST measures appear to not distinguish individuals with chronic pain from those without and thus are not recommended for assessing pain in DMD or guiding treatment.</div></div>","PeriodicalId":50481,"journal":{"name":"European Journal of Paediatric Neurology","volume":"53 ","pages":"Pages 39-47"},"PeriodicalIF":2.3,"publicationDate":"2024-09-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142312448","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
Diagnostic and prognostic significance of serum interleukins in epileptic encephalopathy with spike wave activation in sleep (EE-SWAS) syndrome 血清白细胞介素对伴有睡眠尖波激活的癫痫性脑病(EE-SWAS)综合征的诊断和预后意义
IF 2.3 3区 医学
European Journal of Paediatric Neurology Pub Date : 2024-09-17 DOI: 10.1016/j.ejpn.2024.09.006
Prashant Jauhari , Prabhjot Kaur , Sheffali Gulati , Ankit Kumar Meena , Tapish Pandey , Ashish Upadhyay
{"title":"Diagnostic and prognostic significance of serum interleukins in epileptic encephalopathy with spike wave activation in sleep (EE-SWAS) syndrome","authors":"Prashant Jauhari ,&nbsp;Prabhjot Kaur ,&nbsp;Sheffali Gulati ,&nbsp;Ankit Kumar Meena ,&nbsp;Tapish Pandey ,&nbsp;Ashish Upadhyay","doi":"10.1016/j.ejpn.2024.09.006","DOIUrl":"10.1016/j.ejpn.2024.09.006","url":null,"abstract":"<div><h3>Objective</h3><p>To study serum interleukin-6(IL-6), interleukin-8(IL-8) and interleukin-10(IL-10) levels in Epilpetic encephalopathy with spike-wave activation in sleep(EE-SWAS), drug refractory epilepsy(DRE) and well controlled epilepsy(WCE).</p></div><div><h3>Methods</h3><p>Children(2–12 years) with immunotherapy naïve EE-SWAS, DRE and WCE were enrolled. Valid psychometric tools were used to assess cognition and behavior. Children with EE-SWAS were longitudinally followed. They received a three-month steroid course alongwith the ongoing antiseizure drugs. Electroclinical responders were defined as change in social quotient by 5-points with improvement in atleast one behavioral domain by 5-points and 50 % reduction in mean seizure frequency if active seizures were present alongwith a 25 % reduction in Spike-wave-index(SWI) at three months. Change in serum Interleukin levels at one month follow up was compared between participants who eventually became responders or non-responders at three months.</p></div><div><h3>Results</h3><p>Twenty children with EE-SWAS, 18 with DRE and WCE each were enrolled. Serum IL-6(pg/ml){(EE-SWAS: 3.775(IQR 2.205, 11.28); DRE: 3.01(IQR 2.04, 4.56); WCE: 1.655(IQR 1.27, 2.29), p = 0.0065} and IL-8(pg/ml){(EE-SWAS: 103.2(IQR 34.01, 200.82); DRE: 19.595(IQR 16.54, 39.7); WCE: 18.97(IQR 16.54, 21.91) p = 0.0002} was significantly different between the three groups. In EE-SWAS group 12/20(60 %) showed electroclinical response to steroids. Responders had significant reduction in IL6 levels (pg/ml){4.045(IQR 2.605, 18.96) to 1.13(IQR 054, 1.74)} at one month follow up compared to non responders {3.12(IQR 1.655, 5.27) to 4.37(IQR 2.83, 9.855)} (p = 0.0069).</p></div><div><h3>Conclusions</h3><p>Proinflammatory cytokines (IL-6 and IL-8) are significantly elevated in EE-SWAS compared to DRE and WCE. Reduction in IL-6 levels at one month post-therapy predicted electroclinical responders at 3months follow up.</p></div>","PeriodicalId":50481,"journal":{"name":"European Journal of Paediatric Neurology","volume":"53 ","pages":"Pages 33-38"},"PeriodicalIF":2.3,"publicationDate":"2024-09-17","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142271702","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 differential diagnosis between acute inflammatory demyelinating polyneuropathy and acute-onset chronic inflammatory demyelinating polyneuropathy in children: Clinical factors and routine biomarkers 儿童急性炎症性脱髓鞘性多发性神经病与急性发作的慢性炎症性脱髓鞘性多发性神经病的早期鉴别诊断:临床因素和常规生物标志物
IF 2.3 3区 医学
European Journal of Paediatric Neurology Pub Date : 2024-09-16 DOI: 10.1016/j.ejpn.2024.09.005
Zhiwei Yu, Yuan Xue, Hanyu Luo, Yuhang Li, Siqi Hong, Min Cheng, Jiannan Ma , Li Jiang
{"title":"Early differential diagnosis between acute inflammatory demyelinating polyneuropathy and acute-onset chronic inflammatory demyelinating polyneuropathy in children: Clinical factors and routine biomarkers","authors":"Zhiwei Yu,&nbsp;Yuan Xue,&nbsp;Hanyu Luo,&nbsp;Yuhang Li,&nbsp;Siqi Hong,&nbsp;Min Cheng,&nbsp;Jiannan Ma ,&nbsp;Li Jiang","doi":"10.1016/j.ejpn.2024.09.005","DOIUrl":"10.1016/j.ejpn.2024.09.005","url":null,"abstract":"<div><h3>Background</h3><p>To identify clinical factors and biomarkers that could contribute to early differential diagnosis of acute inflammatory demyelinating polyneuropathy (AIDP) and acute-onset chronic inflammatory demyelinating polyneuropathy (A-CIDP) in the pediatric population, with limited evidence.</p></div><div><h3>Methods</h3><p>We conducted an observational retrospective study of children diagnosed with AIDP and A-CIDP between January 2014 and December 2022. Demographic data, clinical features, and routine biomarkers were also analyzed. Statistical analysis was used to identify significant features with high sensitivity and specificity.</p></div><div><h3>Results</h3><p>We included 91 AIDP and 17 A-CIDP patients. The A-CIDP group had an older median age (6.33 vs. 4.33 years, p = 0.017), required more complex immunotherapies (p &lt; 0.001), and showed a longer time to nadir over 2 weeks (76.5 % vs. 7.7 %, p &lt; 0.001). Gastrointestinal dysfunction (29.4 % vs. 6.59 %, p = 0.014) and numbness (35.3 % vs. 12.1 %, p = 0.027) were more prevalent in A-CIDP. The AIDP patients had a longer median hospitalization stays (13 vs. 11 days, p &lt; 0.05), more prodromal events (90.1 % vs. 64.7 %, p = 0.013), and more frequent cranial nerve palsy (61.5 % vs. 5.88 %, p &lt; 0.001). The disability scores on admission, discharge, and peak were worse in the AIDP group (p &lt; 0.001). AIDP patients showed higher cerebrospinal fluid protein (p = 0.039), albumin quotient (p = 0.048), leukocytes (p = 0.03), neutrophils (p = 0.010), platelet count (p = 0.005), systemic inflammatory index (SII) (p = 0.009), and gamma-glutamyl transferase (p = 0.039). Multivariable regression identified two independent predictors of early A-CIDP detection: time from onset to peak beyond 2 weeks (OR = 37.927, 95%CI = 7.081–203.15) and lower modified Rankin Scale score on admission (OR = 0.308, 95%CI = 0.121–0.788).</p></div><div><h3>Conclusion</h3><p>Our study found that when the condition continued to deteriorate beyond two weeks with a lower mRS on admission and possibly less cranial nerve involvement, we may favor the diagnosis of pediatric A-CIDP rather than AIDP.</p></div>","PeriodicalId":50481,"journal":{"name":"European Journal of Paediatric Neurology","volume":"53 ","pages":"Pages 25-32"},"PeriodicalIF":2.3,"publicationDate":"2024-09-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142271701","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
Impact of autoantibodies against myelin oligodendrocyte glycoprotein in paediatric acquired demyelinating disease: Intellectual functioning and academic performance 髓鞘少突胶质细胞糖蛋白自身抗体对小儿获得性脱髓鞘疾病的影响:智力功能和学习成绩
IF 2.3 3区 医学
European Journal of Paediatric Neurology Pub Date : 2024-09-03 DOI: 10.1016/j.ejpn.2024.09.001
Daniel Griffiths-King , Charly Billaud , Lydiah Makusha , Ling Lynette Looi , Evangeline Wassmer , Sukhvir Wright , Amanda G. Wood
{"title":"Impact of autoantibodies against myelin oligodendrocyte glycoprotein in paediatric acquired demyelinating disease: Intellectual functioning and academic performance","authors":"Daniel Griffiths-King ,&nbsp;Charly Billaud ,&nbsp;Lydiah Makusha ,&nbsp;Ling Lynette Looi ,&nbsp;Evangeline Wassmer ,&nbsp;Sukhvir Wright ,&nbsp;Amanda G. Wood","doi":"10.1016/j.ejpn.2024.09.001","DOIUrl":"10.1016/j.ejpn.2024.09.001","url":null,"abstract":"<div><p>Paediatric acquired demyelinating syndromes (pADS) attack white matter pathways in the brain during an important period of development. Affected children can experience poor functional outcomes, including deficits in specific cognitive domains. Understanding risk factors for poor outcome will guide clinical management of these children. One clinical phenotype which may differentially impact cognitive outcomes is the presence of autoantibodies to myelin oligodendrocyte glycoprotein (MOG). Preliminary research has suggested that cognitive difficulties exist in paediatric patients who test positive for MOG antibodies or MOGAD (Myelin Oligodendrocyte Glycoprotein Associated Disease) however, they experience a less severe profile compared to seronegative counterparts. The current study assesses children diagnosed with pADS who tested positive or negative for MOG-ab using standardised assessments of both intellectual functioning and academic ability. The results show that a subset of MOGAD patients experience clinically significant sequalae in intellectual functioning and academic ability. The neuropsychological profile also differed between children with and without MOG-ab positivity, with seronegative patients more likely to show a clinically relevant difficulties at the individual patient level. Whilst no differences existed at the group-level; the current study demonstrates the relative additional risk of intellectual/academic difficulty associated with MOG-ab seronegativity. This research further supports the growing perspective that MOG-positivity confers a more favourable neuropsychological outlook than is the case for their seronegative counterparts. This broadening consensus offers reassurance for clinicians, families, and patients.</p></div>","PeriodicalId":50481,"journal":{"name":"European Journal of Paediatric Neurology","volume":"53 ","pages":"Pages 8-17"},"PeriodicalIF":2.3,"publicationDate":"2024-09-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S1090379824001326/pdfft?md5=6f64e0b2d577193f66d708237461c951&pid=1-s2.0-S1090379824001326-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142146756","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
The importance of long term follow-up in children with acquired demyelinating syndromes 对后天性脱髓鞘综合征患儿进行长期随访的重要性
IF 2.3 3区 医学
European Journal of Paediatric Neurology Pub Date : 2024-09-01 DOI: 10.1016/j.ejpn.2024.09.002
Rinze F. Neuteboom
{"title":"The importance of long term follow-up in children with acquired demyelinating syndromes","authors":"Rinze F. Neuteboom","doi":"10.1016/j.ejpn.2024.09.002","DOIUrl":"10.1016/j.ejpn.2024.09.002","url":null,"abstract":"","PeriodicalId":50481,"journal":{"name":"European Journal of Paediatric Neurology","volume":"52 ","pages":"Page A2"},"PeriodicalIF":2.3,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142229673","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
Management, treatment, and clinical approach of Sydenham's chorea in children: Italian survey on expert-based experience 儿童赛登翰舞蹈症的管理、治疗和临床方法:意大利专家经验调查
IF 2.3 3区 医学
European Journal of Paediatric Neurology Pub Date : 2024-09-01 DOI: 10.1016/j.ejpn.2024.08.002
Alessandro Orsini , Andrea Santangelo , Giorgio Costagliola , Massimo Scacciati , Francesco Massart , Francesca Felicia Operto , Sofia D'Elios , Rita Consolini , Fabrizio De Benedetti , Maria Cristina Maggio , Angela Miniaci , Alessandro Ferretti , Duccio Maria Cordelli , Roberta Battini , Alice Bonuccelli , Salvatore Savasta , Pasquale Parisi , Elisa Fazzi , Martino Ruggieri , Pasquale Striano , Thomas Foiadelli
{"title":"Management, treatment, and clinical approach of Sydenham's chorea in children: Italian survey on expert-based experience","authors":"Alessandro Orsini ,&nbsp;Andrea Santangelo ,&nbsp;Giorgio Costagliola ,&nbsp;Massimo Scacciati ,&nbsp;Francesco Massart ,&nbsp;Francesca Felicia Operto ,&nbsp;Sofia D'Elios ,&nbsp;Rita Consolini ,&nbsp;Fabrizio De Benedetti ,&nbsp;Maria Cristina Maggio ,&nbsp;Angela Miniaci ,&nbsp;Alessandro Ferretti ,&nbsp;Duccio Maria Cordelli ,&nbsp;Roberta Battini ,&nbsp;Alice Bonuccelli ,&nbsp;Salvatore Savasta ,&nbsp;Pasquale Parisi ,&nbsp;Elisa Fazzi ,&nbsp;Martino Ruggieri ,&nbsp;Pasquale Striano ,&nbsp;Thomas Foiadelli","doi":"10.1016/j.ejpn.2024.08.002","DOIUrl":"10.1016/j.ejpn.2024.08.002","url":null,"abstract":"<div><p>Sydenham's chorea (SC), an autoimmune disorder affecting the central nervous system, is a pivotal diagnostic criterion for acute rheumatic fever. Primarily prevalent in childhood, especially in developing countries, SC manifests with involuntary movements and neuropsychiatric symptoms. Predominantly occurring between ages 5 and 15, with a female bias, SC may recur, particularly during pregnancy or estrogen use. The autoimmune response affecting the basal ganglia, notably against dopamine, underlies the pathophysiology. Clinical management necessitates an integrated approach, potentially involving immunomodulatory therapies.</p><p>To address discrepancies in SC management, a survey was conducted across Italy, targeting specialists in neurology, pediatrics, child neuropsychiatry, and rheumatology. Of the 51 responding physicians, consensus favored hospitalization for suspected SC, with broad support for laboratory tests and brain MRI. Treatment preferences showed agreement on oral prednisone and IVIG, while opinions varied on duration and plasmapheresis. Haloperidol emerged as the preferred symptomatic therapy. Post-SC penicillin prophylaxis and steroid therapy gained strong support, although opinions differed on duration. Follow-up recommendations included neuropsychological and cardiological assessments.</p><p>Despite offering valuable insights, broader and more studies are needed in order to guide treatment decisions in this well-known yet challenging complication of acute rheumatic fever, which continues to warrant scientific attention and concerted clinical efforts.</p></div>","PeriodicalId":50481,"journal":{"name":"European Journal of Paediatric Neurology","volume":"52 ","pages":"Pages 103-108"},"PeriodicalIF":2.3,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S1090379824001235/pdfft?md5=e9e8305a3841b7d711ae43eb0a744b84&pid=1-s2.0-S1090379824001235-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142121661","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Health-related quality of life in 153 children with neuromuscular disorders in Latin America: is it age, functional dependence or diagnosis? 拉丁美洲 153 名神经肌肉障碍儿童与健康相关的生活质量:是年龄、功能依赖还是诊断?
IF 2.3 3区 医学
European Journal of Paediatric Neurology Pub Date : 2024-09-01 DOI: 10.1016/j.ejpn.2024.08.007
Javiera Ortega , Natalia Vázquez , Imanol Amayra Caro , Javier Muntadas , Magalí Squitín Tasende , Alicia Rodriguez Bermejo
{"title":"Health-related quality of life in 153 children with neuromuscular disorders in Latin America: is it age, functional dependence or diagnosis?","authors":"Javiera Ortega ,&nbsp;Natalia Vázquez ,&nbsp;Imanol Amayra Caro ,&nbsp;Javier Muntadas ,&nbsp;Magalí Squitín Tasende ,&nbsp;Alicia Rodriguez Bermejo","doi":"10.1016/j.ejpn.2024.08.007","DOIUrl":"10.1016/j.ejpn.2024.08.007","url":null,"abstract":"<div><p>Neuromuscular diseases impact on children's health related quality of life but there is a lack of studies in Latin America that measured this construct. To respond to this need, this study aimed to explore quality of life and its relationship with age, functional dependence and specific diagnosis in children and adolescents in Latin America. A cross-sectional correlation study was carried out with 133 caregivers on children (2–18 years old) with various neuromuscular disorders. Parents reported on their children's health related quality of life through the PedsQL GCS and the PedsQL NMM. Differences in quality of life were found when comparing children with high functional dependence with those with mild dependence (<em>p</em> = 0.05). No significant differences were found regarding the child diagnosis. Finally, quality of life was highly correlated with the child's age, even when controlling for functional dependence differences between ages. Children and adolescents with neuromuscular show a diminished health related quality of life, not only in physical functioning but in their psychosocial functioning. Health related quality of life may vary according to the child's age and functional dependence.</p></div>","PeriodicalId":50481,"journal":{"name":"European Journal of Paediatric Neurology","volume":"52 ","pages":"Pages 95-102"},"PeriodicalIF":2.3,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142099299","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
Tocilizumab in acute necrotizing encephalopathy (ANE): How much, how soon, and will it improve outcomes beyond survival? 治疗急性坏死性脑病(ANE)的妥西珠单抗:它能在多大程度上、多长时间内改善患者的生存状况?
IF 2.3 3区 医学
European Journal of Paediatric Neurology Pub Date : 2024-09-01 DOI: 10.1016/j.ejpn.2024.08.010
Velda X. Han, Ming Lim
{"title":"Tocilizumab in acute necrotizing encephalopathy (ANE): How much, how soon, and will it improve outcomes beyond survival?","authors":"Velda X. Han,&nbsp;Ming Lim","doi":"10.1016/j.ejpn.2024.08.010","DOIUrl":"10.1016/j.ejpn.2024.08.010","url":null,"abstract":"","PeriodicalId":50481,"journal":{"name":"European Journal of Paediatric Neurology","volume":"52 ","pages":"Pages A3-A4"},"PeriodicalIF":2.3,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142146755","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
0
×
引用
GB/T 7714-2015
复制
MLA
复制
APA
复制
导出至
BibTeX EndNote RefMan NoteFirst NoteExpress
×
提示
您的信息不完整,为了账户安全,请先补充。
现在去补充
×
提示
您因"违规操作"
具体请查看互助需知
我知道了
×
提示
确定
请完成安全验证×
相关产品
×
本文献相关产品
联系我们:info@booksci.cn Book学术提供免费学术资源搜索服务,方便国内外学者检索中英文文献。致力于提供最便捷和优质的服务体验。 Copyright © 2023 布克学术 All rights reserved.
京ICP备2023020795号-1
ghs 京公网安备 11010802042870号
Book学术文献互助
Book学术文献互助群
群 号:481959085
Book学术官方微信