Developmental Medicine and Child Neurology最新文献

{"title":"Early developmental trajectories of the impaired hand in infants with unilateral cerebral palsy","authors":"","doi":"10.1111/dmcn.16266","DOIUrl":"10.1111/dmcn.16266","url":null,"abstract":"<p>We looked at the developmental trajectories (which describes the progression of a given behaviour as individuals age) of impaired hand function in 63 infants with unilateral cerebral palsy. We assessed hand function using the Hand Assessment for Infants either two or three times between 3- and 15-months corrected age. The Hand Assessment for Infants measures the degree and quality of goal-directed actions performed with each hand separately and both hands together.</p><p>We used group-based trajectory modelling which has been developed to determine subgroups within a given population. We found there were three distinctly different trajectories for low, moderate, and high functioning infants.</p><p>There were 23 infants (35%) in the moderate group and 22 (36%) in the high functioning group. Eighteen infants (29%) were in the low functioning group and showed no appreciable change in hand function between 3- and 15-months corrected age, despite all receiving early targeted upper limb training, either modified constraint-induced movement therapy or bimanual therapy. Infants born closer to term equivalent age were at a higher risk of being in the low compared to high functioning group.</p>","PeriodicalId":50587,"journal":{"name":"Developmental Medicine and Child Neurology","volume":"67 4","pages":"e83"},"PeriodicalIF":3.8,"publicationDate":"2025-02-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/dmcn.16266","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143384085","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Polymicrogyria in infants with symptomatic congenital cytomegalovirus at birth is associated with epilepsy: A retrospective, descriptive cohort study","authors":"","doi":"10.1111/dmcn.16263","DOIUrl":"10.1111/dmcn.16263","url":null,"abstract":"<p>Cytomegalovirus (CMV) is the most common virus that is passed from mother to infant during pregnancy. Some infants with congenital CMV (cCMV) show symptoms at birth, like hearing problems, small head size, or growth problems. These infants are at higher risk of long-term problems like epilepsy, resulting in seizures (fits). This study aimed to find out if magnetic resonance imaging (MRI) features at birth could predict which children are more likely to develop epilepsy.</p><p>The study looked at 90 children with cCMV. Seventy-two children had symptoms at birth, and 18 did not have symptoms. Seven children developed epilepsy, which was 7.8% of all children with cCMV and 9.7% of those who had symptoms at birth. None of the children without symptoms at birth developed epilepsy. The study found that specific brain changes on MRI, particularly a condition called polymicrogyria (atypical brain development with too many small folds in the brain), were closely linked to epilepsy. Infants with polymicrogyria were 35 times more likely to develop epilepsy. Other features, like smaller head size at birth, and white matter damage on MRI, were also more common in children who developed epilepsy.</p><p>All seven children with epilepsy required medications to reduce their seizures, and only three achieved seizure control. Four children needed more than two medications and still had frequent seizures.</p>","PeriodicalId":50587,"journal":{"name":"Developmental Medicine and Child Neurology","volume":"67 4","pages":"e80"},"PeriodicalIF":3.8,"publicationDate":"2025-02-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/dmcn.16263","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143384091","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Cerebral palsy and perinatal mortality in children born in Norway to immigrant mothers","authors":"","doi":"10.1111/dmcn.16271","DOIUrl":"10.1111/dmcn.16271","url":null,"abstract":"<p>This study aimed to compare the birth prevalence and clinical characteristics of cerebral palsy (CP) between children born to non-immigrant mothers and those born to immigrant mothers from high-income countries (HICs) and low- and middle-income countries (LMICs), as well as perinatal mortality rates. Data were collected from the Medical Birth Registry of Norway and the Norwegian Quality and Surveillance Registry for Cerebral Palsy, covering live births and perinatal deaths from 2000 to 2016.</p><p>Results showed that the prevalence of CP was higher among children of non-immigrant mothers, with a decreasing trend over time. Children of immigrant mothers from HICs had lower odds of CP, while those from LMICs also had lower odds despite higher rates of consanguinity and lower folate use during pregnancy. Perinatal mortality was higher among mothers from LMICs. Clinical characteristics of CP were similar across groups, but children from LMICs had a higher incidence of intellectual disabilities and brain grey matter injuries.</p>","PeriodicalId":50587,"journal":{"name":"Developmental Medicine and Child Neurology","volume":"67 4","pages":"e89"},"PeriodicalIF":3.8,"publicationDate":"2025-02-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/dmcn.16271","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143384083","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Adaptive functioning in children and young adults with monogenic neurodevelopmental disorders","authors":"","doi":"10.1111/dmcn.16264","DOIUrl":"10.1111/dmcn.16264","url":null,"abstract":"<p>This study looked at the adaptive behaviour profiles of children and young adults with different neurodevelopmental disorders (e.g. autism, intellectual disability), with known genetic causes. Adaptive behaviours are the practical skills that an individual needs to function independently in everyday life (e.g. interacting with other people, shopping, personal grooming, etc.). Understanding these behaviours within individual neurodevelopmental disorders, as well as comparing these behaviours across different disorders can help us to identify and develop suitable interventions for these conditions.</p><p>The study included 243 children and young adults aged 1 to 25 years with one of eight genetic neurodevelopmental disorders (<i>CDK13</i>, <i>DYRK1A</i>, <i>FOXP2</i>, <i>KAT6A</i>, <i>KANSL1</i>, <i>SETBP1</i>, <i>BRPF1</i>, and <i>DDX3X</i>). Parents completed the standardized interview of the Vineland Adaptive Behavior Scales, Third Edition, a common measure of adaptive behaviour that assesses communication, daily living, socialization, and motor skills.</p>","PeriodicalId":50587,"journal":{"name":"Developmental Medicine and Child Neurology","volume":"67 4","pages":"e81"},"PeriodicalIF":3.8,"publicationDate":"2025-02-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/dmcn.16264","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143384080","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Glycopyrronium 320 μg/mL in children and adolescents with severe sialorrhoea and neurodisabilities: An open-label study extension of the SALIVA trial","authors":"","doi":"10.1111/dmcn.16268","DOIUrl":"10.1111/dmcn.16268","url":null,"abstract":"<p>Drooling (sialorrhoea) is common in children with neurodisabilities, such as cerebral palsy. Severe drooling can cause irritated skin, dehydration, retention of urine, and chest infections. Drooling may also affect the child's quality of life, self-esteem, and social interactions. In addition, it can add to the burden of parents and caregivers, for example, leading to frequent changes of bibs and clothing. The SALIVA (<span>S</span>ialanar plus or<span>A</span>l rehabi<span>L</span>itation against placebo plus oral rehabilitation for ch<span>I</span>ldren and adolescents with se<span>V</span>ere sialorrhoe<span>A</span> and neurodisabilities) trial investigated 320 μg/mL glycopyrronium, a medicine that has been specifically designed to meet the needs of children.</p><p>An open label (or non-blinded) study is one in which both the healthcare providers and the patients are aware of the drug or treatment being given.</p><p>In the first part of the trial, 87 children (aged 3–17 years) with neurodisabilities and severe drooling were randomly allocated to receive either 320 μg/mL glycopyrronium or placebo for 12 weeks. Treatment with 320 μg/mL glycopyrronium significantly reduced drooling versus placebo and the impact of drooling on quality of life was decreased, with no unexpected side effects.</p><p>In the second part of the trial, all children received 320 μg/mL glycopyrronium for 24 weeks. Improvements in drooling seen in the first part were sustained with continued 320 μg/mL glycopyrronium. Around 80% of children responded to treatment and 70% showed a good response. In addition, reductions in drooling's impact on quality of life were sustained with continued 320 μg/mL glycopyrronium.</p><p>Overall, the side effects seen with 320 μg/mL glycopyrronium were typical of this type of medicine, most commonly constipation and dry mouth. Side effects were more frequent during the first 4 weeks, when the dose was being adjusted, with the frequency of side effects subsequently reducing.</p>","PeriodicalId":50587,"journal":{"name":"Developmental Medicine and Child Neurology","volume":"67 4","pages":"e85"},"PeriodicalIF":3.8,"publicationDate":"2025-02-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/dmcn.16268","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143384086","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Involving people with lived experience when setting cerebral palsy research priorities: A scoping review","authors":"","doi":"10.1111/dmcn.16269","DOIUrl":"10.1111/dmcn.16269","url":null,"abstract":"<p>Scoping reviews identify key concepts within a particular research area and types of evidence currently available. This scoping review examines how people with lived experience of cerebral palsy (CP) have contributed to setting priorities for CP research in order to characterize how the current body of literature addresses the high priority needs of those directly affected. This review specifically focused on characteristics of the participants who were involved, methods used, and research priorities identified. Five projects were identified that involved people with lived experience of CP in setting research priorities, conducted in North America and Australia.</p><p>Most participants with lived experience were caregivers, with fewer individuals with CP making up the total number of participants. The methods varied, including surveys, workshops, and webinars. The most commonly identified areas of research were related to optimizing interventions. Four of the five projects actively involved participants with lived experience of CP as research partners, influencing decisions at every stage of the research.</p><p>This review highlighted significant gaps in representation of the CP community. People with CP, particularly from low- and middle-income countries were underrepresented. Similarly, perspectives from children and young adults with CP and individuals with more complex needs, such as those using augmentative communication devices to supplement or replace speech or writing, were rarely included.</p><p>The findings emphasize the importance of including a broader range of voices in future research priority-setting projects. This can ensure that the unique needs and perspectives of all individuals with CP, regardless of background or condition severity, are reflected in the literature. Employing rigorous methods and collaborating with people with lived experience as research partners is also necessary to improve transparency and reliability of new research.</p>","PeriodicalId":50587,"journal":{"name":"Developmental Medicine and Child Neurology","volume":"67 4","pages":"e86"},"PeriodicalIF":3.8,"publicationDate":"2025-02-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/dmcn.16269","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143384089","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Safety and effectiveness of stiripentol in patients with Dravet syndrome: A prospective, 3-year, postmarketing surveillance study.","authors":"Yuki Kitamura, Hiroaki Ohyabu, Tatsuo Miura, Naomi Takei-Masuda, Daisuke Matsui, Yushi Inoue, Yoko Ohtsuka","doi":"10.1111/dmcn.16252","DOIUrl":"https://doi.org/10.1111/dmcn.16252","url":null,"abstract":"<p><strong>Aim: </strong>To conduct a postmarketing surveillance study of patients with Dravet syndrome in Japan to investigate the safety and effectiveness of long-term, real-world, clinical use of stiripentol (STP).</p><p><strong>Method: </strong>This prospective study was conducted over 156 weeks in all patients with Dravet syndrome who started STP treatment from its launch in Japan in November 2012 until August 2017. Adverse drug reactions (ADRs) were investigated by degree of seriousness. Effectiveness was determined based on a comprehensive assessment by the physician in charge as well as on the percentage change in the number of seizures from the pretreatment period.</p><p><strong>Results: </strong>In total, 520 patients (266 males, 254 females; mean age [SD] 10 years 6 months [9 years 10 months]; age range 0-50 years) were included in the safety analysis set, and 515 patients in the effectiveness analysis set. ADRs occurred in 69.2%, including somnolence, decreased appetite, dizziness, in order of frequency. Twelve deaths occurred, the rate of which was not higher than the reported rates. No new safety concerns were identified. The rate of overall improvement (marked or moderate) after 156 weeks or at treatment discontinuation was 37.7%. Decreases in the number of all seizure types over the long term were confirmed.</p><p><strong>Interpretation: </strong>In real-world clinical settings, long-term STP treatment can be safe and effective in patients with Dravet syndrome.</p>","PeriodicalId":50587,"journal":{"name":"Developmental Medicine and Child Neurology","volume":" ","pages":""},"PeriodicalIF":3.8,"publicationDate":"2025-02-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143384097","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Wearable sensors in paediatric neurology","authors":"","doi":"10.1111/dmcn.16267","DOIUrl":"10.1111/dmcn.16267","url":null,"abstract":"<p>Wearable sensors are small, non-invasive devices that can easily be attached to the body to monitor signals such as movement, heart rate, breathing, and temperature. These sensors allow data to be collected in familiar environments, such as at home or during school, and capture real-world patterns and behaviours that may not be evident during traditional clinical assessments. This approach reduces the stress of being examined, minimizes bias, and overcomes poor cooperation, especially in younger patients.</p><p>Wearable sensors have been studied for various paediatric neurological conditions, including cerebral palsy, epilepsy, autism spectrum disorder, attention-deficit/hyperactivity disorder, Rett syndrome, Down syndrome, Angelman syndrome, Prader–Willi syndrome, and neuromuscular disorders such as Duchenne muscular dystrophy and spinal muscular atrophy. Other areas of application are ataxia, Gaucher disease, headaches, and sleep disorders.</p><p>The data collected by wearable sensors can be used to detect early signs of neurological disorders and monitor changes over time. For example, subtle changes in walking patterns that might go unnoticed through conventional clinical assessments can be detected using these devices. This information could enable earlier interventions, more accurate diagnoses, and personalized treatment plans tailored to each child's specific needs.</p>","PeriodicalId":50587,"journal":{"name":"Developmental Medicine and Child Neurology","volume":"67 4","pages":"e84"},"PeriodicalIF":3.8,"publicationDate":"2025-02-09","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1111/dmcn.16267","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143383293","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Developmental trajectories of impaired hand function in unilateral cerebral palsy: Clinical insights and future directions.","authors":"Nava Gelkop, Hanoch Cassuto","doi":"10.1111/dmcn.16257","DOIUrl":"https://doi.org/10.1111/dmcn.16257","url":null,"abstract":"","PeriodicalId":50587,"journal":{"name":"Developmental Medicine and Child Neurology","volume":" ","pages":""},"PeriodicalIF":3.8,"publicationDate":"2025-02-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143191246","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Glycopyrronium 320 μg/mL in children and adolescents with severe sialorrhoea and neurodisabilities: An open-label study extension of the SALIVA trial.","authors":"Pierre Fayoux, Mickael Dinomais, Helen Shaw, Frédéric Villain, Déborah Schwartz, Vincent Gautheron, Guy Letellier, Stéphane Auvin","doi":"10.1111/dmcn.16251","DOIUrl":"https://doi.org/10.1111/dmcn.16251","url":null,"abstract":"<p><strong>Aims: </strong>To test the long-term efficacy, safety, and impact on quality of life (QoL) of an oral paediatric formulation of 320 μg/mL glycopyrronium in the 36-week SALIVA (Sialanar plus orAl rehabiLitation against placebo plus oral rehabilitation for chIldren and adolescents with seVere sialorrhoeA and neurodisabilities) trial.</p><p><strong>Method: </strong>In the initial 12-week blinded period, 87 children with neurodisabilities and severe sialorrhoea were randomized to 320 μg/mL glycopyrronium versus placebo. In the subsequent 24-week open-label study extension, 74 children received 320 μg/mL glycopyrronium (37 continued glycopyrronium, 37 switched from placebo).</p><p><strong>Results: </strong>The open-label study extension population included 39 males and 35 females. The median age was 10 years 2 months (quartile 1, quartile 3: 7 years 5 months, 14 years 7 months; range: 3 years 5 months-17 years 8 months). Over 36 weeks, continued 320 μg/mL glycopyrronium resulted in a median 39-point reduction in Drooling Impact Scale (DIS) score from baseline (quartile 1, quartile 3: -51, -21; p < 0.001), with an 81.1% response rate (DIS improvement ≥ 13.6 points) and a 70.3% good response rate (≥ 28 points). Improvements in the impact of drooling on QoL seen in the blinded period were sustained with continued glycopyrronium. Treatment-related adverse events occurred most frequently during titration (0-4 weeks: 40.9%; 5-20 weeks: 32.4% in those who switched). Constipation was the most common adverse event.</p><p><strong>Interpretation: </strong>Long-term treatment with 320 μg/mL glycopyrronium resulted in significant sustained improvements in drooling and QoL, with fewer adverse events after initial titration and overall good tolerability.</p>","PeriodicalId":50587,"journal":{"name":"Developmental Medicine and Child Neurology","volume":" ","pages":""},"PeriodicalIF":3.8,"publicationDate":"2025-01-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"143070804","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}