Annals of the Child Neurology Society最新文献

筛选
英文 中文
Correction to “Salvage Trans-Sylvian Peri-Insular Hemispherotomy After Embolic Hemispherectomy” 栓塞性脑半球切除术后经脑岛周围半球切开术的纠正
Annals of the Child Neurology Society Pub Date : 2026-06-16 Epub Date: 2026-05-11 DOI: 10.1002/cns3.70072
{"title":"Correction to “Salvage Trans-Sylvian Peri-Insular Hemispherotomy After Embolic Hemispherectomy”","authors":"","doi":"10.1002/cns3.70072","DOIUrl":"https://doi.org/10.1002/cns3.70072","url":null,"abstract":"<p>Baumgartner, M.E., Kessler, S., Galligan, K., Baumgartner, J.E. and Kennedy, B.C. (2025), Salvage Trans-Sylvian Peri-Insular Hemispherotomy After Embolic Hemispherectomy. Annals of the Child Neurology Society, 3: 110-114. https://doi.org/10.1002/cns3.70003.</p><p>“A Case Report and Review of the Literature” is added as the article's subtitle.</p><p>We apologize for this error.</p>","PeriodicalId":72232,"journal":{"name":"Annals of the Child Neurology Society","volume":"4 2","pages":""},"PeriodicalIF":0.0,"publicationDate":"2026-06-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/cns3.70072","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148269055","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Robert Zeller, MD (1938–2025) 罗伯特·泽勒,医学博士(1938-2025)
Annals of the Child Neurology Society Pub Date : 2026-06-16 Epub Date: 2026-03-11 DOI: 10.1002/cns3.70063
Gary D. Clark, Phillip L. Pearl
{"title":"Robert Zeller, MD (1938–2025)","authors":"Gary D. Clark,&nbsp;Phillip L. Pearl","doi":"10.1002/cns3.70063","DOIUrl":"https://doi.org/10.1002/cns3.70063","url":null,"abstract":"&lt;p&gt;&lt;/p&gt;&lt;p&gt;Figure: Robert Stuart Zeller, MD. Used with permission from the Child Neurology Society.&lt;/p&gt;&lt;p&gt;A beloved child neurologist known for his clinical acumen, gentle nature, and duck sounds that put children at ease and reduced families' stress levels, Robert “Bob” Zeller was a master clinician who trained scores of pediatric neurology residents at Texas Children's Hospital.&lt;/p&gt;&lt;p&gt;A native of Buffalo, Bob attended Princeton University and received his MD degree in 1963 from the University of Buffalo. He completed his pediatric internship and residency at Baylor College of Medicine (1963−1966) and published his first paper, which was on translocation heterozygosis, that is, structural chromosomal derangements, during his residency (1966). He then served as a captain in the US Air Force, during which he actively practiced and wrote a publication on the complications of ventriculo-arterial shunting for hydrocephalus. This was followed by child neurology training at the renowned program at Columbia University in New York City with the child neurology leaders and luminaries of that era, including Drs. Sidney Carter, Abe Chutorian, and Arnold Gold.&lt;/p&gt;&lt;p&gt;After his training, Bob settled in Houston. He became a charter member of the Child Neurology Society in 1972. He was appointed chief of pediatric neurology at Texas Children's Hospital and served in this position for 5 years, with publications on eastern equine encephalitis and cerebrovascular thrombosis in ketoacidotic coma and clinical trials in Duchenne muscular dystrophy. He collaborated with Baylor neuropathologists Jan Goddard-Finegold and Dawna Armstrong in experimental investigations of intraventricular hemorrhage using a neonatal beagle model.&lt;/p&gt;&lt;p&gt;Dr. Zeller had an innate capacity to support others and bring out their best. He was a walking model of compassion and had extraordinary abilities in diagnosis and communication. He was known for duck-like quacks, Sesame Street puppets, and cowboy boots, all of which raised humor and reduced stress levels for patients and families.&lt;/p&gt;&lt;p&gt;Dr. Zeller was appointed Clinical Professor of Pediatrics at Baylor in 1993 and named chief of the Blue Bird Circle Clinic of Texas Children's Hospital in 2005. He served with extraordinary commitment, expressing the sense that his patients gave more to him than he gave to them. When he learned that a patient with epilepsy was not allowed to enroll in a summer camp, he created, with his own investment and fundraising, “Camp For AII” to serve the needs of children, adolescents, and adults with disabilities. More than 100,000 campers have benefited from this model facility by now.&lt;/p&gt;&lt;p&gt;A devoted family man, Bob was married to the love of his life, Susan Cooper, for 56 years, and they have a close-knit family of three children and eight grandchildren. He enjoyed building and fixing things, from restoring his own 1929 Mercedes SSK to tennis backboards and playhouses. Dr. Zeller was recognized with the Arnold P. ","PeriodicalId":72232,"journal":{"name":"Annals of the Child Neurology Society","volume":"4 2","pages":"108-109"},"PeriodicalIF":0.0,"publicationDate":"2026-06-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/cns3.70063","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148269085","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","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 Amber of This Moment: A Parable of Training Traditions 这一刻的琥珀:训练传统的寓言
Annals of the Child Neurology Society Pub Date : 2026-06-16 Epub Date: 2026-06-04 DOI: 10.1002/cns3.70073
E. Steve Roach
{"title":"The Amber of This Moment: A Parable of Training Traditions","authors":"E. Steve Roach","doi":"10.1002/cns3.70073","DOIUrl":"https://doi.org/10.1002/cns3.70073","url":null,"abstract":"&lt;p&gt;It was your pride and joy, that 8-year-old convertible. It idled like a purring cat but roared like a lion when challenged. Its quaint turquoise-and-white color scheme made it more suited for a tropical movie set than for the school parking lot. It represented the prospect of worldly adventure and independence and suggested a stature and maturity not yet earned. It was cool, and by extension, so were you.&lt;/p&gt;&lt;p&gt;Over time, the car became a mobile museum to the fading memories of youth. Its classic design could never be replaced by the computer-generated sameness of the newest models. Its energy was always a step ahead of the more fuel-efficient present. It came to represent simpler, idyllic times, an icon of an era that may never have actually existed. The notion of trading it for a newer model was an unthinkable betrayal.&lt;/p&gt;&lt;p&gt;Life moved on even though you did not. The aging car became a bit sluggish and picked up a few dings and leaks. There seemed to be less and less of interest on the car's old AM radio. Knowledgeable mechanics and spare parts became difficult to find, and it stranded you a few times while driving to work. Its lack of fuel efficiency became increasingly painful as gasoline prices soared. Its lack of air conditioning in summer and drafty ragtop in winter promoted nearly year-round discomfort. Your grandchildren were not allowed to ride with you because the car lacked seat belts and airbags. But tradition and nostalgia cannot be easily erased. There were still frequent requests for the car to participate in parades, anniversary celebrations, and weddings, increasingly without you as the driver.&lt;/p&gt;&lt;p&gt;The unfortunate thing about traditions is that no matter how desperately you cling to them, they still sometimes leave you behind. The solution to the above dilemma would be to find a way to acknowledge the past while taking better advantage of modern fuel efficiency, comfort features, and safety measures. Perhaps you could give the classic car a place of honor in the garage next to its modern cousin, try to retrofit some of its more antiquated features, or donate it to a museum where you can visit whenever you like, and others can enjoy it as well.&lt;/p&gt;&lt;p&gt;I am a neurologist, and I still see lots of adult patients and help run a neurology department. I can hardly be accused of bias against adult neurology training. We should find ways to embrace our ties to neurology. But how can we in good conscience maintain decades-old training requirements for child neurologists that allocate so much time to adult neurology and general pediatrics and leave so little time for future child neurologists to learn about the remarkable developments in the field? How many adult neurology residency directors would happily embrace an equivalent 5-year adult neurology residency that required 2 preliminary years of internal medicine and a full year of child neurology? Adult neurologists constitute a strong majority of the Residency Review Committee, but","PeriodicalId":72232,"journal":{"name":"Annals of the Child Neurology Society","volume":"4 2","pages":"115-116"},"PeriodicalIF":0.0,"publicationDate":"2026-06-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/cns3.70073","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148269267","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
A. C. Boudewijn Peters (1941–2026) a·c·布德维恩·彼得斯(1941-2026)
Annals of the Child Neurology Society Pub Date : 2026-06-16 Epub Date: 2026-04-02 DOI: 10.1002/cns3.70067
Jurriaan M. Peters, Oebo F. Brouwer
{"title":"A. C. Boudewijn Peters (1941–2026)","authors":"Jurriaan M. Peters,&nbsp;Oebo F. Brouwer","doi":"10.1002/cns3.70067","DOIUrl":"https://doi.org/10.1002/cns3.70067","url":null,"abstract":"&lt;p&gt;Boudewijn Peters, MD, PhD, a foundational figure in Dutch pediatric neurology and one of the second generation of specialists to shape the field in the Netherlands, passed away on January 30, 2026, at the age of 84. His career was defined by clinical excellence, institutional leadership, and a sustained commitment to advancing the scientific understanding of epilepsy in childhood.&lt;/p&gt;&lt;p&gt;Dr. Peters completed his neurology training in Leiden, followed by a fellowship in pediatric neurology in Utrecht under Professor Kobus Willemse. In the mid-1970s, at what was then the Academic Hospital Leiden (now Leiden University Medical Center), he established one of the country's early departments of pediatric neurology. At a time when pediatric neurology was still emerging as a recognized subspecialty, he traveled throughout the Netherlands with a slide projector, presenting to primary care physicians and pediatricians to demonstrate the distinct diagnostic and therapeutic needs of children with neurological disorders. Through these efforts, he materially contributed to the consolidation of pediatric neurology as an autonomous discipline.&lt;/p&gt;&lt;p&gt;Within Leiden, Dr. Peters built a close-knit clinical service centered on a 10-bed pediatric neurology unit. He was widely regarded as an exemplary clinician: measured, attentive, and deeply empathetic. He devoted generous time to children and their families, combining diagnostic rigor with humane presence. As an educator, he was equally distinguished. His lectures—delivered in a calm, deliberate voice—were enriched by carefully selected case material and nuanced clinical reflection. Many trainees credit him not only for imparting knowledge but also for modeling professional integrity and intellectual discipline. He granted junior colleagues significant autonomy while remaining consistently available for counsel, fostering both independence and loyalty.&lt;/p&gt;&lt;p&gt;A major scientific milestone in his career was the initiation, in 1988, of a large prospective cohort study of childhood epilepsy in collaboration with Dr. Willem Frans Arts. Initially known as the South Holland Pediatric Epilepsy Study and later expanded as the Dutch Study of Epilepsy in Childhood, the project enrolled approximately 500 children and generated numerous influential international publications. The study's strength lay in its systematic, longitudinal design and its careful phenotyping—features that anticipated later standards in pediatric epilepsy research. Dr. Peters was particularly admired for his elegant writing style and his ability during manuscript preparation to interpret findings with clarity, balance, and contextual depth.&lt;/p&gt;&lt;p&gt;Beyond his institutional and research contributions, Dr. Peters played a central organizational role in Dutch pediatric neurology. He served as secretary of the Dutch Society of Child Neurology from 1978 to 1981 and as its president from 1984 to 1990. During his presidency, he oversaw the presentation of the fir","PeriodicalId":72232,"journal":{"name":"Annals of the Child Neurology Society","volume":"4 2","pages":"110-111"},"PeriodicalIF":0.0,"publicationDate":"2026-06-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/cns3.70067","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148269041","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Isolated Corticospinal Tract Lesions as an Early Manifestation of Adrenoleukodystrophy in Children 孤立性皮质脊髓束病变是儿童肾上腺脑白质营养不良的早期表现
Annals of the Child Neurology Society Pub Date : 2026-06-16 Epub Date: 2026-03-16 DOI: 10.1002/cns3.70064
Elle Winter, Amanda Nagy, Yedda Li, Otto Rapalino, Catherine L. Becker, Joseph Locascio, Camilo Jaimes, Florian Eichler
{"title":"Isolated Corticospinal Tract Lesions as an Early Manifestation of Adrenoleukodystrophy in Children","authors":"Elle Winter,&nbsp;Amanda Nagy,&nbsp;Yedda Li,&nbsp;Otto Rapalino,&nbsp;Catherine L. Becker,&nbsp;Joseph Locascio,&nbsp;Camilo Jaimes,&nbsp;Florian Eichler","doi":"10.1002/cns3.70064","DOIUrl":"https://doi.org/10.1002/cns3.70064","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Purpose</h3>\u0000 \u0000 <p>X-linked adrenoleukodystrophy (ALD) encompasses a wide range of neurological manifestations, classically described as distinct phenotypes including childhood cerebral adrenoleukodystrophy and adult-onset adrenomyeloneuropathy (AMN). Corticospinal tract (CST) and cerebellar involvement on brain magnetic resonance imaging (MRI) are typically attributed to long-tract axonopathy in AMN, yet the extent to which these features span the ALD spectrum is unclear. To explore whether CST and infratentorial lesions reflect discrete phenotypes or a continuous disease spectrum, we examined their onset and prevalence across the lifespan.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>We retrospectively analyzed 1189 brain MRIs from 218 male patients diagnosed with ALD evaluated at Massachusetts General Hospital from 2005 to 2024. Imaging reports were screened for CST and infratentorial lesion presence, and serial scans were reviewed to determine chronological patterns of involvement. In pediatric patients with isolated CST lesions, treatment data and post-treatment imaging were assessed.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Isolated CST lesions were identified in 8.7% of pediatric and 11.4% of adult patients. All but one adult with cerebellar lesions also demonstrated CST lesions, which preceded or occurred concurrently with the cerebellar abnormalities. Among pediatric patients with isolated CST lesions, 38.5% underwent lentiviral gene therapy, and 23.1% received hematopoietic stem cell transplantation. Imaging post-treatment revealed lesion size reduction in 87.5%.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>CST involvement, including isolated lesions and those preceding cerebellar involvement, occurs across age groups and may represent a continuous ALD disease spectrum rather than a strictly phenotype-specific feature. These findings support the importance of dedicated CST assessment in routine MRI review and highlight its potential role in earlier diagnosis and therapeutic decision-making.</p>\u0000 </section>\u0000 </div>","PeriodicalId":72232,"journal":{"name":"Annals of the Child Neurology Society","volume":"4 2","pages":"117-124"},"PeriodicalIF":0.0,"publicationDate":"2026-06-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/cns3.70064","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148269045","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Structural and Functional Imaging of Motor Outcomes in Twins With Perinatal Stroke: A Case Report 围产期卒中双胞胎运动结果的结构和功能成像:1例报告
Annals of the Child Neurology Society Pub Date : 2026-06-16 Epub Date: 2026-03-16 DOI: 10.1002/cns3.70058
Prisca Hsu, Trish Domi, Cecilia Jobst, Min Sheng, Amanda Robertson, Erum Syed, Pradeep Krishnan, Douglas Cheyne, Nomazulu Dlamini
{"title":"Structural and Functional Imaging of Motor Outcomes in Twins With Perinatal Stroke: A Case Report","authors":"Prisca Hsu,&nbsp;Trish Domi,&nbsp;Cecilia Jobst,&nbsp;Min Sheng,&nbsp;Amanda Robertson,&nbsp;Erum Syed,&nbsp;Pradeep Krishnan,&nbsp;Douglas Cheyne,&nbsp;Nomazulu Dlamini","doi":"10.1002/cns3.70058","DOIUrl":"https://doi.org/10.1002/cns3.70058","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Perinatal arterial ischemic stroke (AIS) affects 1 in 4000 live births. Dystonia, affecting ~20% of children following AIS, is characterized by involuntary muscle contractions and abnormal movements. Why some develop dystonia post AIS, while others do not, remains unclear. We hypothesized that dystonia after AIS is associated with two key features: lesions involving frontal–basal ganglia network regions and corresponding disruptions in motor oscillatory activity.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>Monozygotic twin brothers aged 11 years were recruited to a study investigating neural correlates of motor control in post-stroke dystonia. Brain magnetic resonance imaging (MRI) assessed lesion localization, volume, and white matter structure. Magnetoencephalography measured timing and localization of neural oscillations during movement execution and inhibition on the “go”/”no-go” task. Clinical history was obtained from charts. Neurological outcome was evaluated with the Pediatric Stroke Outcome Measure (PSOM).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>MRI showed mirrored hemispheric lesions in the twins, with additional basal ganglia involvement in Twin A. Diffusion tensor imaging revealed lower corticospinal tract integrity in Twin A. MEG in Twin A revealed increased frontal theta (4–8 Hz) during correct inhibition. Sensorimotor gamma was reduced for Twin A but upregulated for Twin B during contralesional hand movement. Clinically, Twin A had a PSOM score of 2 (moderate motor and cognitive deficits); Twin B scored 0 (no deficits).</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>Our findings suggest that dystonia following perinatal AIS is associated with altered frontal–basal ganglia network function, with increased frontal recruitment reflecting compensatory disinhibition during motor control.</p>\u0000 </section>\u0000 </div>","PeriodicalId":72232,"journal":{"name":"Annals of the Child Neurology Society","volume":"4 2","pages":"150-158"},"PeriodicalIF":0.0,"publicationDate":"2026-06-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/cns3.70058","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148269044","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Developmental Milestones for Children With Down Syndrome: Revised Estimates Using Moving Average Summaries 唐氏综合症儿童的发展里程碑:使用移动平均摘要的修订估计
Annals of the Child Neurology Society Pub Date : 2026-06-16 Epub Date: 2026-03-25 DOI: 10.1002/cns3.70065
Rahaf Tarawneh, Katherine Pawlowski, Nicole Baumer, Bo Zhang, Maitreyi Mazumdar
{"title":"Developmental Milestones for Children With Down Syndrome: Revised Estimates Using Moving Average Summaries","authors":"Rahaf Tarawneh,&nbsp;Katherine Pawlowski,&nbsp;Nicole Baumer,&nbsp;Bo Zhang,&nbsp;Maitreyi Mazumdar","doi":"10.1002/cns3.70065","DOIUrl":"https://doi.org/10.1002/cns3.70065","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Objective</h3>\u0000 \u0000 <p>Children with Down syndrome follow distinct developmental trajectories that require specialized monitoring and counseling. We aimed to provide updated estimates of developmental milestone attainment using a nonparametric approach and to compare these results with previously reported generalized linear mixed-effects model (GLMM) estimates.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>We reanalyzed developmental data from 842 children with Down syndrome (ages 2 months to 24 years). For each milestone, achievement rates were calculated within overlapping 0.5-year time windows, and shape-constrained additive models were used to derive monotonic regression curves.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Compared with GLMM-based estimates, the nonparametric approach predicted milestone attainment at least 1 year earlier for 17 of 25 milestones at the highest comparable percentile. Early gross motor milestones showed consistent achievement patterns across methods, whereas more complex adaptive, language, and academic skills demonstrated greater variability and were more sensitive to modeling assumptions. Females achieved 17 of 25 milestones at least 1 year earlier than males.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusions</h3>\u0000 \u0000 <p>These findings highlight substantial developmental heterogeneity and support the potential for continued skill acquisition throughout childhood and adolescence in individuals with Down syndrome. The updated estimates provide clinically relevant reference points to guide individualized monitoring, anticipatory guidance, and family counseling.</p>\u0000 </section>\u0000 </div>","PeriodicalId":72232,"journal":{"name":"Annals of the Child Neurology Society","volume":"4 2","pages":"140-144"},"PeriodicalIF":0.0,"publicationDate":"2026-06-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/cns3.70065","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148269173","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Development and Retrospective Application of Novel Outcome Measure: Sturge–Weber Syndrome Acute Crisis (SWAC) Index 新型预后指标:斯特奇-韦伯综合征急性危重症(SWAC)指数的开发与回顾性应用
Annals of the Child Neurology Society Pub Date : 2026-06-16 Epub Date: 2026-03-24 DOI: 10.1002/cns3.70059
Kieran D. McKenney, Luther G. Kalb, Adrienne M. Hammill, Anne M. Comi
{"title":"Development and Retrospective Application of Novel Outcome Measure: Sturge–Weber Syndrome Acute Crisis (SWAC) Index","authors":"Kieran D. McKenney,&nbsp;Luther G. Kalb,&nbsp;Adrienne M. Hammill,&nbsp;Anne M. Comi","doi":"10.1002/cns3.70059","DOIUrl":"https://doi.org/10.1002/cns3.70059","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Objective</h3>\u0000 \u0000 <p>Sturge–Weber syndrome (SWS) symptoms frequently overlap in neurological acute crises (ACs) presenting with seizures, stroke-like episodes (SLE), and headaches. Recent efforts to develop a prospective drug trial highlighted the need for a new outcome measure capable of quantifying these symptoms during an SWS AC. We aimed to develop such a measure and evaluate its practical application.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>The Sturge Weber Acute Crisis (SWAC) index was developed based on clinical care guidelines and experience. We retrospectively applied the SWAC to an open-label prospective drug trial published in 2020; SWAC scores were assigned for every AC during 6-month baseline and treatment phase periods.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Results</h3>\u0000 \u0000 <p>Six of 10 subjects experienced 670 ACs; only 78/670 ACs were included in the primary quantitative analyses (one subject was an extreme outlier) and 71/78 events were single-symptom while 7/78 included multiple symptoms. Three of six subjects experienced improved median seizure total score; all three improved their median SLE total score. Three of five subjects with headache improved their median headache total score. Poisson-log GLMM models demonstrated significant improvements in composite severity and total, SLE duration and total, and headache severity and total index scores.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Interpretation</h3>\u0000 \u0000 <p>The newly developed SWAC index, applied retrospectively, reflected site-PI clinical experience, quantified the severity and duration of SWS ACs ranging from mild to severe, and better enabled comparison of treatment versus baseline responses. These results support additional studies with the SWAC index to further assess clinical utility and use in future treatment trials in SWS.</p>\u0000 </section>\u0000 </div>","PeriodicalId":72232,"journal":{"name":"Annals of the Child Neurology Society","volume":"4 2","pages":"132-139"},"PeriodicalIF":0.0,"publicationDate":"2026-06-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/cns3.70059","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148269340","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Neonatal Seizures in Tuberous Sclerosis Complex: A Case Series 结节性硬化症并发的新生儿癫痫:一个病例系列
Annals of the Child Neurology Society Pub Date : 2026-06-16 Epub Date: 2026-03-09 DOI: 10.1002/cns3.70061
Kristina Jülich, Kristen Arredondo
{"title":"Neonatal Seizures in Tuberous Sclerosis Complex: A Case Series","authors":"Kristina Jülich,&nbsp;Kristen Arredondo","doi":"10.1002/cns3.70061","DOIUrl":"https://doi.org/10.1002/cns3.70061","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Background</h3>\u0000 \u0000 <p>Epilepsy affects up to 90% of patients with tuberous sclerosis complex (TSC); earlier seizure onset is associated with worse neurocognitive outcomes. The incidence of neonatal seizures in TSC is unknown, although in a recent multicenter trial 23% of infants with TSC were excluded prior to randomization because of pre-existing seizures prior to age 4 months, suggesting that neonatal or early infantile seizures may be a common occurrence.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Aims</h3>\u0000 \u0000 <p>We aimed to determine the rate of neonatal seizures in our cohort of TSC patients.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Methods</h3>\u0000 \u0000 <p>We performed a single-center medical records review of patients with TSC who were seen between 2020 and 2025 to identify patients with neonatal seizure onset.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Summary and Conclusions</h3>\u0000 \u0000 <p>Of six patients with data from the neonatal period, four (67%) had neonatal seizures. Two had confirmed electrographic seizures in the first week of life, and another two had clinical seizure onset during the neonatal period. All patients with neonatal seizures had TSC2 and developed drug-resistant epilepsy, and two of four developed infantile spasms despite intensive early medical management, including vigabatrin. The two patients without neonatal seizures had TSC1 and mosaic TSC2, respectively. They have not developed epilepsy and have had milder neurodevelopmental impairment.</p>\u0000 \u0000 <p>In conclusion, data from our center suggest that neonatal seizures may be common in TSC, especially in high-risk infants with TSC2 with highly epileptogenic lesions. With recent preliminary data about safety and efficacy of mTOR inhibitor treatment in infants with TSC, early EEG monitoring should be considered.</p>\u0000 </section>\u0000 </div>","PeriodicalId":72232,"journal":{"name":"Annals of the Child Neurology Society","volume":"4 2","pages":"145-149"},"PeriodicalIF":0.0,"publicationDate":"2026-06-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/cns3.70061","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148268933","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Steroids for Drug-Resistant Seizures and Prolonged Stroke-Like Episode in a Patient With Sturge–Weber Syndrome: A Case Report 类固醇治疗斯特奇-韦伯综合征患者的耐药癫痫发作和延长卒中样发作:一例报告
Annals of the Child Neurology Society Pub Date : 2026-06-16 Epub Date: 2026-05-26 DOI: 10.1002/cns3.70068
Brenna N. Keam, Fabiola A. Morales-Vias, Siddharth S. Gupta, Doris D. Lin, Anne M. Comi
{"title":"Steroids for Drug-Resistant Seizures and Prolonged Stroke-Like Episode in a Patient With Sturge–Weber Syndrome: A Case Report","authors":"Brenna N. Keam,&nbsp;Fabiola A. Morales-Vias,&nbsp;Siddharth S. Gupta,&nbsp;Doris D. Lin,&nbsp;Anne M. Comi","doi":"10.1002/cns3.70068","DOIUrl":"https://doi.org/10.1002/cns3.70068","url":null,"abstract":"<div>\u0000 \u0000 \u0000 <section>\u0000 \u0000 <h3> Introduction</h3>\u0000 \u0000 <p>Sturge–Weber syndrome (SWS) brain involvement has been associated with impairments in the blood–brain barrier (BBB) and microglial activation within involved cortical regions. Acute neurological crises, including seizures, stroke-like episodes, and/or significant headaches, are common in these patients. This report describes in detail an adolescent with SWS and acute drug-resistant status epilepticus, headache, and stroke-like episode who improved clinically when treated with high-dose steroids. Review of medical records identified two other patients previously treated with steroids for acute neurological symptoms.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Case Presentation</h3>\u0000 \u0000 <p>A 13-year-old boy with SWS brain involvement presented with fever, headache, seizures, and right-sided weakness. Brain magnetic resonance imaging (MRI) revealed characteristic findings of SWS. The patient was placed on continuous electroencephalogram that showed findings consistent with electrographic status epilepticus. Seizures were refractory to multiple anti-seizure medications as well as to an intravenous midazolam drip. A repeat MRI of the brain documented a significant increase in leptomeningeal enhancement with associated gyral edema and sulcal effacement. Steroid therapy with methylprednisolone and prednisone improved his status epilepticus and stroke-like symptoms, including resolution of seizures and marked improvement in his hemiparesis.</p>\u0000 </section>\u0000 \u0000 <section>\u0000 \u0000 <h3> Conclusion</h3>\u0000 \u0000 <p>This patient suggests a potential role for inflammation-targeted therapies in individuals with SWS brain involvement who present with prolonged neurological crises. Two other patients with SWS brain involvement who were treated with high-dose steroids in the context of acute neurological episodes also support the need for future clinical and preclinical research to validate this approach.</p>\u0000 </section>\u0000 </div>","PeriodicalId":72232,"journal":{"name":"Annals of the Child Neurology Society","volume":"4 2","pages":"159-166"},"PeriodicalIF":0.0,"publicationDate":"2026-06-16","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://onlinelibrary.wiley.com/doi/epdf/10.1002/cns3.70068","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"148268916","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","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:604180095
Book学术官方微信
小红书