European Journal of Paediatric Neurology最新文献

{"title":"Assessment of somatosensory profiles by quantitative sensory testing in children and adolescents with and without cerebral palsy and chronic pain","authors":"Johanne Villars Jørgensen ,&nbsp;Mads Utke Werner ,&nbsp;Josephine Sandahl Michelsen ,&nbsp;Christian Nai En Tierp-Wong","doi":"10.1016/j.ejpn.2024.05.007","DOIUrl":"https://doi.org/10.1016/j.ejpn.2024.05.007","url":null,"abstract":"<div><h3>Objective</h3><p>We investigated differences in somatosensory profiles (SSPs) assessed by quantitative sensory testing in children and adolescents with cerebral palsy (CCP) with and without chronic pain and compared these differences to those in a group of typically developed children and adolescents (TDC) with and without chronic pain.</p></div><div><h3>Method</h3><p>All included subjects were consecutively recruited from and tested at the same outpatient orthopedic clinic by the same investigator. The subjects had their reaction times tested. The SSP consisted of the following tests: warmth (WDT), cool (CDT), mechanical (MDT), and vibration (VDT) detection thresholds; heat (HPT), pressure (PPT), and mechanical (MPT) pain thresholds; wind-up ratio (WUR); dynamic mechanical allodynia (DMA) and cold pressor test (CPT) using a conditioned pain modulation (CPM) paradigm.</p></div><div><h3>Results</h3><p>We included 25 CCP and 26 TDC. TDC without chronic pain served as controls. In TDC with chronic pain, WDT, HPT, HPT intensity, and PPT were higher than in controls. No differences in SSPs between CCP with and without chronic pain were observed. In CCP, the MDT, WDT, CDT, and HPT intensity were higher than in controls. CCP had longer reaction times than TDC. There were no differences regarding the remaining variables.</p></div><div><h3>Discussion</h3><p>In CCP, the SSPs were independent of pain status and findings on MR images. In all CCP the SSPs resembled TDC with chronic pain, compared to TDC without chronic pain. This suggests that CCP do not have the normal neuroplastic adaptive processes that activate and elicit functional changes in the central and peripheral nervous systems.</p></div>","PeriodicalId":50481,"journal":{"name":"European Journal of Paediatric Neurology","volume":"51 ","pages":"Pages 32-40"},"PeriodicalIF":3.1,"publicationDate":"2024-05-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S1090379824000655/pdfft?md5=1eefc3f631ed6abcc4cea9136e4d12e0&pid=1-s2.0-S1090379824000655-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141095402","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}

{"title":"The when, why, and how of using glycopyrronium to diminish drooling in children with neurodevelopmental disabilities: Implications for clinical practice","authors":"Lynn B. Orriëns ,&nbsp;Jan J.W. van der Burg ,&nbsp;Frank J.A. van den Hoogen ,&nbsp;Michèl A.A.P. Willemsen ,&nbsp;Karen van Hulst ,&nbsp;Corrie E. Erasmus","doi":"10.1016/j.ejpn.2024.05.004","DOIUrl":"10.1016/j.ejpn.2024.05.004","url":null,"abstract":"<div><h3>Background</h3><p>Despite the established efficacy of glycopyrronium bromide in reducing drooling among children with neurodevelopmental disabilities, evidence on its impact on the daily lives of children and parents and effectiveness in a real-world setting are scarce, especially among long-term users. This study explored timing and duration of glycopyrronium treatment, effect and impact on daily life, and occurrence of side effects to inform clinical practice.</p></div><div><h3>Methods</h3><p>This was a retrospective cohort study at a national referral centre for drooling, including 61 children with nonprogressive neurodevelopmental disabilities, treated with glycopyrronium for anterior and/or posterior drooling between 2011 and 2021. Data were obtained from medical records and supplemented by structured telephone interviews with parents.</p></div><div><h3>Results</h3><p>Anterior drooling severity decreased in 82% of the included children. Changes in the impact of drooling on burden of care, social interaction, and self-esteem were reported in 55%, 31%, and 36%, respectively. Side effects were noted for 71% of cases, yet only 36% of parents deemed these as outweighing the positive impact of treatment. A substantial majority (77%) of the included children were long-term users (≥6 months). Among these, 38% of parents reported decreasing effectiveness and 27% noticed more prominent side effects over time.</p></div><div><h3>Conclusions</h3><p>Glycopyrronium demonstrated potential in mitigating the impact of drooling on daily life, although variations were observed in the specific aspects and extent of improvement. The real-world context of our study provides important insights for refining clinical practices, emphasizing the need for balanced consideration of treatment benefits and potential side effects to facilitate shared decision-making.</p></div>","PeriodicalId":50481,"journal":{"name":"European Journal of Paediatric Neurology","volume":"51 ","pages":"Pages 24-31"},"PeriodicalIF":3.1,"publicationDate":"2024-05-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S109037982400062X/pdfft?md5=d1cfc3a612ce86d8e06ded0a063572b9&pid=1-s2.0-S109037982400062X-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141037338","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}

{"title":"Different trajectories of post-concussive symptom subscales after pediatric mild traumatic brain injury: Data from a prospective longitudinal study","authors":"Martina Studer ,&nbsp;Lara Mischler ,&nbsp;Fabrizio Romano ,&nbsp;Karen Lidzba ,&nbsp;Sandra Bigi","doi":"10.1016/j.ejpn.2024.05.003","DOIUrl":"https://doi.org/10.1016/j.ejpn.2024.05.003","url":null,"abstract":"<div><h3>Purpose</h3><p>The aim of this study was to investigate the trajectory of parent-rated post-concussive symptoms (PCS), attentional performance and participation within 6 months in children after mild traumatic brain injury (mTBI).</p></div><div><h3>Methods</h3><p>For this prospective longitudinal study, we included data on 64 children after mTBI and 57 healthy control children (age 8–16 years). Parents rated PCS using the Post-Concussion Symptom Inventory (PCSI) immediately (T0), 1 week (T1), and 3–6 months after injury (T2). Attentional performance (alertness, selective and divided attention) was measured using the Test of Attentional Performance (TAP) at T1 and T2 and participation was measured using the Child and Adolescent Scale of Participation (CASP) at T2.</p></div><div><h3>Results</h3><p>Friedman tests showed different trajectories of PCS subscales over time: Compared to pre-injury level, the amount of somatic and cognitive PCS was still elevated at T1, while emotional PCS at T1 were already comparable to pre-injury level. The rating of sleep-related PCS at T2 was significantly elevated compared to the pre-injury rating. Quade ANCOVAs indicated group differences in PCS subscales between patients and controls at T1, but not at T2. Patients and controls showed a similar performance in tests of attention at T1 and T2, but parental rating of participation at school was significantly reduced. Although cognitive PCS and attention were not correlated, there were significantly negative Spearman correlations between participation at home and pre-injury and concurrent PCS at T2.</p></div><div><h3>Conclusions</h3><p>Our data imply that sleep-related PCS are still elevated weeks after injury and are thus a target for interventions after mTBI.</p></div>","PeriodicalId":50481,"journal":{"name":"European Journal of Paediatric Neurology","volume":"51 ","pages":"Pages 9-16"},"PeriodicalIF":3.1,"publicationDate":"2024-05-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140914560","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}

{"title":"Cognitive function in SMA patients with 2 or 3 SMN2 copies treated with SMN-modifying or gene addition therapy during the first year of life","authors":"Paula Steffens ,&nbsp;Deike Weiss ,&nbsp;Anna Perez ,&nbsp;Manuel Appel ,&nbsp;Philipp Weber ,&nbsp;Claudia Weiss ,&nbsp;Corinna Stoltenburg ,&nbsp;Ute Ehinger ,&nbsp;Maja von der Hagen ,&nbsp;Jens Schallner ,&nbsp;Birte Claussen ,&nbsp;Ilka Lode ,&nbsp;Andreas Hahn ,&nbsp;Rahel Schuler ,&nbsp;Lena Ruß ,&nbsp;Andreas Ziegler ,&nbsp;Jonas Denecke ,&nbsp;Jessika Johannsen","doi":"10.1016/j.ejpn.2024.05.002","DOIUrl":"10.1016/j.ejpn.2024.05.002","url":null,"abstract":"<div><h3>Background</h3><p>Spinal muscular atrophy (SMA) is a neuromuscular disease, causing progressive muscle weakness due to loss of lower motoneurons. Since 2017, three therapies, two modifying gene transcription and one adding the defective gene, have been approved with comparable efficacy on motor outcome. Data on cognitive outcomes of treated SMA type 1 patients is limited. The aim of this study was to evaluate cognitive function in symptomatic and presymptomatic SMA type 1 patients with two or three <em>SMN2</em> copies who received SMN-modifying or gene-addition therapy in the first year of life.</p></div><div><h3>Methods</h3><p>Cognitive testing was performed in 20 patients, including 19 symptomatic SMA type 1 patients with up to three SMN2 copies and 1 pre-symptomatically treated patient. Children were tested using Bayley Scales of Infant Development (BSID-III) at the age of 2 or 3 years or the Wechsler Preschool and Primary Scale of Intelligence (WPSII-IV) at the of age of 5 years.</p></div><div><h3>Results</h3><p>11/20 patients showed subnormal cognitive development. Boys had significantly lower cognitive scores. Patients requiring assisted ventilation or feeding support were more likely to have cognitive deficits. Achieving more motor milestones was associated with a better cognitive outcome.</p></div><div><h3>Conclusion</h3><p>Treated patients with SMA type 1 have heterogeneous cognitive function with 55 % of patients showing deficits. Risk factors for cognitive impairment in our cohort were male gender and need for assisted ventilation or feeding support. Therefore, cognitive assessment should be included in the standard of care to allow early identification of deficits and potential therapeutic interventions.</p></div>","PeriodicalId":50481,"journal":{"name":"European Journal of Paediatric Neurology","volume":"51 ","pages":"Pages 17-23"},"PeriodicalIF":3.1,"publicationDate":"2024-05-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S1090379824000606/pdfft?md5=5e1814a093678023085fa10f43835981&pid=1-s2.0-S1090379824000606-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141049200","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}

{"title":"Insights into Rett Syndrome girls' receptive vocabulary through eye movements and parental perception","authors":"Jucineide S. Xavier ,&nbsp;Fernanda T. Orsati ,&nbsp;Paulo G. Laurence ,&nbsp;Lourenço K. Barbosa ,&nbsp;Andressa S. Gouveia ,&nbsp;Maria Elóisa F. D'Antino ,&nbsp;Katerina Lukasova ,&nbsp;Elizeu C. Macedo ,&nbsp;José Salomão Schwartzman","doi":"10.1016/j.ejpn.2024.05.001","DOIUrl":"10.1016/j.ejpn.2024.05.001","url":null,"abstract":"<div><h3>Objective</h3><p>This study evaluated the receptive vocabulary of girls diagnosed with Rett Syndrome (RS) by employing eye-tracking technology and examined how these objective measures compared with parents' perceptions of their daughters' language abilities.</p></div><div><h3>Method</h3><p>Fourteen girls with RS and eleven typically developing peers participated. Instruments included the Kerr Scale, a parental questionnaire on communication skills, the Peabody Picture Vocabulary Test - PPVT-4, and eye-tracking equipment. The PPVT-4's initial 12 cards, each displaying four images, were presented on screen for 5 s for the child's initial inspection. Subsequently, the same card was shown for 5 s with instructions to identify the picture matching the word, repeated three times in a randomized order.</p></div><div><h3>Results</h3><p>Eye-tracking assessments revealed that girls with RS with less severe symptoms, performed below typically developing peers with aged 3–4 years. The number of eye fixations emerged as a reliable metric for assessing their performance. A positive correlation was found between parental reports of their daughters' vocabulary knowledge and their ability to identify the correct stimulus, suggesting comprehension and active engagement in the testing process.</p></div><div><h3>Conclusion</h3><p>This study presents a pioneering approach by combining parental perceptions with eye-tracking measures to assess receptive vocabulary in girls diagnosed with Rett Syndrome. Despite showing lower performance levels compared to significantly younger peers, the findings suggest that these individuals use less effectively eye contact as a mode of communication.</p></div>","PeriodicalId":50481,"journal":{"name":"European Journal of Paediatric Neurology","volume":"51 ","pages":"Pages 1-8"},"PeriodicalIF":3.1,"publicationDate":"2024-05-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140904505","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}

{"title":"Commentary on Phase IV PROVE study: Perampanel in real-world clinical care of pediatric patients with epilepsy [Moretz K, Wheless J, Santos C, Segal E, Lancman M, Patten A, Malhotra M]","authors":"Frank M.C. Besag,&nbsp;Michael J. Vasey","doi":"10.1016/j.ejpn.2024.05.008","DOIUrl":"10.1016/j.ejpn.2024.05.008","url":null,"abstract":"","PeriodicalId":50481,"journal":{"name":"European Journal of Paediatric Neurology","volume":"50 ","pages":"Page vii"},"PeriodicalIF":3.1,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141082842","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}

{"title":"Blood parameters in pediatric myelin oligodendrocyte glycoprotein antibody-associated disorders","authors":"Alina Peternell ,&nbsp;Christian Lechner ,&nbsp;Markus Breu ,&nbsp;Martin Preisel ,&nbsp;Mareike Schimmel ,&nbsp;Astrid Eisenkölbl ,&nbsp;Joachim Zobel ,&nbsp;Eva-Maria Wendel ,&nbsp;Markus Reindl ,&nbsp;Kevin Rostásy ,&nbsp;Matthias Baumann","doi":"10.1016/j.ejpn.2024.04.011","DOIUrl":"10.1016/j.ejpn.2024.04.011","url":null,"abstract":"<div><h3>Background and objectives</h3><p>Patients with myelin oligodendrocyte glycoprotein antibody-associated disorders (MOGAD) clinically present e.g. with acute disseminated encephalomyelitis (ADEM), optic neuritis (ON), transverse myelitis (TM) or aquaporin-4-IgG (AQP4-IgG) negative neuromyelitis optica spectrum disorders (NMOSD)-like phenotypes. We aimed to analyze and compare blood parameters in children with MOGAD, AQP4-IgG-positive NMOSD (hence NMOSD), multiple sclerosis (MS) and healthy controls (HC).</p></div><div><h3>Methods</h3><p>We evaluated differences in complete blood counts (CBC), neutrophil-to-lymphocyte ratio (NLR), platelet-to-lymphocyte ratio (PLR), monocyte-to-lymphocyte ratio (MLR) and C-reactive protein (CRP) between these four groups and within the groups between clinical attack, acute treatment and remission.</p></div><div><h3>Results</h3><p>Our cohort consisted of 174 children and adolescents with a total of 550 timepoints: 66 patients had MOGAD (202 timepoints), 11 NMOSD (76 timepoints), 58 MS (219 timepoints) and 39 were HC (53 timepoints). At clinical attack, leukocyte counts were elevated in MOGAD compared to remission (p &lt; 0.001) and compared to all other groups (p &lt; 0.001). NLR was high in MOGAD and NMOSD, and PLR was high in NMOSD, however, after correction for multiple testing these findings did not remain significant. While glucocorticoids caused an increase of leukocyte counts and NLR in NMOSD and MS, these values remained stable during acute treatment in MOGAD. In remission, NLR normalized in MOGAD, while it stayed high in NMOSD. PLR increased in NMOSD and was significantly higher compared to all other groups.</p></div><div><h3>Discussion</h3><p>Some blood parameters, mainly leukocyte and differential counts, might help clinicians to evaluate disease activity, differentiate relapses from pseudo-relapses and even distinguish between different disease entities.</p></div>","PeriodicalId":50481,"journal":{"name":"European Journal of Paediatric Neurology","volume":"50 ","pages":"Pages 86-95"},"PeriodicalIF":3.1,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.sciencedirect.com/science/article/pii/S1090379824000576/pdfft?md5=c76d803f4359c49728209b43ab083a78&pid=1-s2.0-S1090379824000576-main.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140782794","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}

{"title":"Neurologic complications of thiamine (B1) deficiency following bariatric surgery in adolescents","authors":"Lital Cohen Vig ,&nbsp;Rachel Straussberg ,&nbsp;Noa Ziv ,&nbsp;Lior Hirschfeld-Dicker ,&nbsp;Osnat Konen ,&nbsp;Sharon Aharoni","doi":"10.1016/j.ejpn.2024.04.008","DOIUrl":"https://doi.org/10.1016/j.ejpn.2024.04.008","url":null,"abstract":"<div><h3>Background</h3><p>The prevalence of obesity among children and adolescents is rising and poses a major health concern. Bariatric surgery is well established in adults and has become an option for adolescents. Thiamine (B1) deficiency is common following bariatric surgery in adults. It may present as Beri-Beri, Wernicke encephalopathy, or Korsakoff psychosis.</p></div><div><h3>Objective</h3><p>Our aim was to describe the clinical features, diagnosis, and treatment of adolescents who presented with B1 deficiency after bariatric surgery at one center, and to summarize the data from the literature.</p></div><div><h3>Patients</h3><p>Three adolescents with morbid obesity (two boys and one girl, aged 15.5 to- 17-years-old), presented at Schneider Children's Medical Center of Israel with progressive lower limb pain and weakness 2–3 month following a bariatric procedure (sleeve gastrectomy or narrowing of a bariatric band). The girl also had upper limb involvement and cerebellar signs. All three were non-compliant with micronutrient supplementation. After admission, they received intravenous B1 and oral multivitamin supplementation, and their symptoms improved considerably.</p></div><div><h3>Conclusions</h3><p>Micronutrient supplementation following bariatric surgery is crucial to prevent deficiencies. In adolescents, compliance with micronutrient supplementation should be assessed before and after such surgery. Thiamine deficiency may cause polyneuropathy, among other symptoms. Treatment reduces the severity of neurological complications.</p></div>","PeriodicalId":50481,"journal":{"name":"European Journal of Paediatric Neurology","volume":"50 ","pages":"Pages 74-80"},"PeriodicalIF":3.1,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140822528","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}

{"title":"“Deconstructing” upper limb function in dyskinetic cerebral palsy","authors":"Roser Pons,&nbsp;Dalivigka Zoi","doi":"10.1016/j.ejpn.2024.05.005","DOIUrl":"10.1016/j.ejpn.2024.05.005","url":null,"abstract":"","PeriodicalId":50481,"journal":{"name":"European Journal of Paediatric Neurology","volume":"50 ","pages":"Pages v-vi"},"PeriodicalIF":3.1,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140960658","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}

{"title":"Clinical correlation between disease progression and central vein sign in pediatric onset multiple sclerosis: A binational study","authors":"Shay Menascu ,&nbsp;Simona Halusková ,&nbsp;Amir Pollak ,&nbsp;Pavel Ryska ,&nbsp;Francesco Angelucci ,&nbsp;David Magalashvili ,&nbsp;Diana Guber ,&nbsp;Arthur Yosef ,&nbsp;Alon Kalron ,&nbsp;Martin Valis ,&nbsp;Michael Gurevich","doi":"10.1016/j.ejpn.2024.04.007","DOIUrl":"10.1016/j.ejpn.2024.04.007","url":null,"abstract":"<div><h3>Background</h3><p>The central vein sign (CVS) has been proposed as a novel MRI biomarker to improve diagnosis of pediatric-onset MS (POMS). However, the role of CVS in POMS progression has yet to be discovered.</p></div><div><h3>Objectives</h3><p>To investigate the appearance of CVS and its correlation with POMS disease progression.</p></div><div><h3>Methods</h3><p>One hundred fifty-six POMS from two MS centers in Israel and Czech Republic MS centers were followed for five years. Patient assessment was performed by the Expanded Disability Status Scale (EDSS) and Annual Relapse Rate (ARR). Patients in whom at least 40 % of brain MRI lesions had CVS (“rule of 40”) were determined as CVS-positive.</p></div><div><h3>Results</h3><p>The total group of POMS consisted of 96 CVS-negative (61.5 %), aged 14.6 ± 1.9 years, EDSS 2.0, 75 % Interquartile Range (IQR) 1.0–3.0, disease duration (DD) 6.28 ± 0.38 years, and 60 CVS-positive (38.5 %), aged 15.1 ± 0.3 years, EDSS 2.0, IQR 1.5–3.0, DD 5.62 ± 0.13 years, were analyzed. After a three and five-year follow-up, the CVS-positive patients had higher EDSS scores than those who were CVS-negative, 2.0, IQR 1.0–2.5, vs 1.0, IQR 1.0–2.0, (p = 0.009) and 2.0, IQR 1.0–3.25 vs 1.0, IQR 1.0–2.0, (p = 0.0003), respectively. Patients with CVS-positive POMS were characterized by a significantly higher ARR (0.78 ± 0.08 vs 0.57 ± 0.04, p = 0.002). These results were confirmed in subgroups of Disease Modifying Treatments (DMT) untreated and treated patients.</p></div><div><h3>Conclusion</h3><p>CVS-positive POMS is characterized by higher disability progression than CVS-negative, indicating the importance of CVS in disease pathogenesis.</p></div>","PeriodicalId":50481,"journal":{"name":"European Journal of Paediatric Neurology","volume":"50 ","pages":"Pages 81-85"},"PeriodicalIF":3.1,"publicationDate":"2024-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140770979","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}