The global research hotspots and future trends of infantile epileptic spasms syndrome: A bibliometric analysis of trends and themes.

PurposeIodine-125 (125-I) seed implantation is a widely used brachytherapy technique for treating various solid tumors. This study aims to provide a bibliometric analysis of the research trends, key contributors, and emerging hotspots in this field.Materials and methodsA comprehensive search was conducted using the Web of Science Core Collection, covering publications from January, 1960 to August 20, 2024. Bibliometric analysis was performed with VOSviewer, CiteSpace, and the R package “bibliometrix” to examine trends in publications, countries, institutions, journals, authors, and keywords.ResultsThe analysis included 2212 publications, showing a steady increase in research output, with USA, China, and Japan leading in publication volume. The University of California System and Keio University were the most productive institutions. Brachytherapy and the International Journal of Radiation Oncology, Biology, and Physics emerged as the most influential journals in this field. Yorozu Atsunori and Wang Junjie were identified as key authors. Keyword co-occurrence analysis highlighted “cancer,” “brachytherapy,” and “radiotherapy” as core themes. Keyword burst analysis revealed evolving research hotspots, such as “hepatocellular carcinoma,” “efficacy,” “safety,” and “transarterial chemoembolization,” emphasizing concerns about long-term outcomes, safety, and treatment strategies for 125-I implantation therapy across multiple cancers.ConclusionThis bibliometric analysis underscores that research on 125-I seed implantation is primarily focused on optimizing dosimetry, improving implantation techniques, and addressing long-term outcomes and safety. The findings emphasize the need for standardized treatment protocols to ensure consistent and effective clinical practice.

A case-control evaluation of Spasm control and Tolerability of the Modified Atkins diet versus classic ketogenic diet in Chinese Children with infantile epileptic spasms syndrome

Safety, Efficacy, and Tolerability of Ketogenic Diet Versus Adrenocorticotropic Hormone in Infantile Epileptic Spasms Syndrome: A Randomized Controlled Trial Mahesan A, Gulati S, Sondhi V, Agarwala A, Gupta KL, Kamila G, Jauhari P, Chakrabarty B, Pandey RM. Pediatr Neurol. 2025;169:196-203. doi:10.1016/j.pediatrneurol.2025.05.027. Background: Ketogenic diet (KD) is efficacious in adrenocorticotrophic hormone (ACTH)-refractory infantile epileptic spasms syndrome (IESS) with lesser side effects. Upfront treatment with KD in IESS is the least explored. This study aims to compare the efficacy of upfront treatment with KD compared with ACTH in IESS. Methods: In this open-label randomized controlled trial, infants aged 6 to 24 months with an electroclinical diagnosis of IESS were enrolled and randomly assigned to either the KD or the ACTH arm. The primary efficacy endpoint was to compare spasm reduction at 6 weeks of therapy from the baseline between the two arms. Secondary end points were electroclinical spasm cessation rates at 6 weeks, relapse after initial clinical response, adverse effects, and developmental progress at 24 weeks of follow-up. Results: A total of 178 children were screened; 87 children were randomized, of which 41 received ACTH and 42 received KD. At 6 weeks of therapy, the median (interquartile range) percentage spasm reduction was comparable (66.7% [24.5-100] for ACTH vs 76.6% [56.3-100] for KD [P=.39]). The electroclinical spasm cessation rates were similar. There were higher relapses in the ACTH arm in those with initial clinical spasm cessation (38.9% ACTH vs 0 KD, P =.004) at 24 weeks' follow-up. The proportion of children having at least one side effect was higher in the ACTH arm (95.1% ACTH vs 59.5% KD; relative risk, 1.6 [1.23-2.1]). Conclusions: KD may not be inferior to ACTH as first-line therapy for IESS with a better side effect profile and fewer relapses compared with ACTH.

Developmental arrest or regression that results in intellectual disability (ID) is a common neurological sequela of infantile epileptic spasms syndrome. Spindles are the hallmark of non-rapid eye movement sleep, and their density correlates with cognitive performance. Recent evidence suggests that ictal and interictal epileptic activities hijack the thalamocortical network. Therefore, we aimed to investigate whether thalamocortical dysfunction, as represented by reduced spindle density, is related to cognitive outcomes. We retrospectively recruited patients diagnosed with infantile epileptic spasms syndrome who were treated at Saitama Children's Hospital. Specifically, we included patients with an unknown etiology to minimize the effects of preceding thalamocortical dysfunction induced by various etiologies before the onset of infantile epileptic spasms syndrome. These cohorts were separated into two groups based on the presence or absence of ID, defined as an intelligence quotient (IQ) or developmental quotient (DQ) of <70, during follow-up. Information concerning patients' background characteristics, treatment response (defined as seizure cessation for at least 3 months), and cognitive performance was extracted. Pre- and posttreatment spindle features were derived from quantitative assessment of spindle features performed using automated algorithms, which were validated against expert human annotations. Additionally, logistic regression was used to validate the independent predictors of cognitive outcomes. Overall, 45 patients were included (23 and 22 patients in the ID and non-ID groups, respectively). Posttreatment spindle density was significantly higher in the non-ID group than in the ID group. Moreover, spindle density was positively correlated with DQ/IQ values across the entire cohort. Finally, a logistic regression model revealed that spindle density and treatment response were independent predictors of cognitive outcomes. Reduced posttreatment spindle density was correlated with cognitive outcome in infantile epileptic spasms syndrome. The thalamocortical network may be a potential treatment target to achieve favorable cognitive outcomes.

ABSTRACTBackgroundNeurodegenerative diseases are characterized by a progressive decline in neuronal function, posing a major challenge to understanding their molecular mechanisms. The role of DNA methylation in these diseases remains an area of research focus.MethodsA bibliometric analysis was performed using the Web of Science Core Collection (WoSCC) database, covering 3284 publications from January 1, 2000 to November 18, 2024. The search focused on articles related to methylation in neurodegenerative diseases, with queries limited to the “title” field. Only original articles and reviews in English were included. Data were analyzed using VOSviewer, CiteSpace, and Bibliometrix R packages to visualize trends, identify collaborative networks, and identify key topics, such as DNA methylation and Alzheimer's disease.ResultsThe analysis showed a steady increase in research output, with major contributions coming from the United States, China, and the United Kingdom. The University of California system, Harvard University, and University College London were leading institutions. Key journals such as the International Journal of Molecular Sciences, Nature, and Proceedings of the National Academy of Sciences were identified as having impact. Keyword analysis highlighted DNA methylation and Alzheimer's disease as prominent research topics.ConclusionDespite limitations such as the lack of experimental validation and clinical evidence, this study highlights the growing interest in methylation research on neurodegenerative diseases and underscores the need for innovative efforts to identify novel therapeutic targets and biomarkers.

Background and objectivesMetabolic Dysfunction-Associated Steatotic Liver Disease (MASLD) is one of the leading causes of hepatocellular carcinoma (HCC). Over the years, significant progress has been made in diagnosing and treating MASLD. However, there is a lack of bibliometric analysis exploring the relationship between MASLD and HCC. This study aims to comprehensively analyze the knowledge structure and research status of MASLD-related HCC using bibliometric methods.MethodsThe study was conducted using the Web of Science Core Collection (WoSCC) database, retrieving literature related to MASLD and HCC from 2020 to 2024. VOSviewer, CiteSpace, and the R package “bibliometrics” were used for data analysis.ResultsThe study included 2,576 articles from 97 countries. The results showed that the number of publications related to MASLD and HCC increased steadily until 2023, after which there was a slight decline. China (The People’s Republic of China) is the country with the highest volume of research output, while the United States is the country with the highest number of citations. The University of California System, the Chinese University of Hong Kong, and the Egyptian Knowledge Bank were the leading contributing institutions. The International Journal of Molecular Sciences, Cancers, and Liver International were the most influential journals in this field, with the International Journal of Molecular Sciences being the most co-cited journal, and the Journal of Hepatology had the highest local impact in the field, as evaluated by the H-index. The publications were authored by 13,851 researchers, with Vincent Wai-Sun Wong, Masato Yoneda, and Rohit Loomba being the most influential authors in the field of MASLD and HCC. Rohit Loomba and Hashem B. El-Serag were the most cited authors. Trend analysis indicated that “tissue”, “element-binding protein”, and “contributes” were the most frequently studied topics in 2024, suggesting emerging research trends.ConclusionThis study provides a comprehensive summary of the research trends and developments in HCC related to MASLD over the past five years. The findings reveal the latest research frontiers and hotspots in this field, offering valuable insights for future research. However, it should be noted that although the WoSCC is a widely recognized and authoritative database, it may have limitations related to regional bias, language coverage, and metadata accuracy, which could potentially affect the comprehensiveness of our analysis.

Approximately half of all cases of Infantile Epileptic Spasms Syndrome (IESS) do not respond to vigabatrin and hormonal therapies. There is no clear consensus as to the second-line therapy for IESS. Ketogenic diet (KD) has emerged as an effective treatment for certain drug-resistant epilepsies and in many cases of IESS. Understanding the mechanism of action of the KD in IESS will allow for harnessing the power of the KD and discovering novel therapeutics for IESS. In this review, we will summarize the current state of knowledge of the action of the KD in IESS derived from animal models. We emphasize the importance of the KD in altering respiration to cause brain acidosis. In addition, we review recent data implicating altered gut microbiome and the tryptophan-serotonin-kynurenine pathway in KD animals with infantile epileptic spasms syndrome. PLAIN LANGUAGE SUMMARY: Infantile Epileptic Spasms Syndrome is a serious seizure condition in babies, often resistant to standard drugs, failing in half of cases. Animal studies helped unravel multiple mechanisms through which a high-fat, low-carb ketogenic diet can control seizures, including altering gut bacteria, reducing inflammation, balancing brain chemicals, boosting mitochondrial function, or adjusting breathing to slightly acidify the brain. These findings could lead to new, targeted therapies that are simpler to use and more accessible for families facing this challenging condition.

Safety, Efficacy, and Tolerability of Ketogenic Diet Versus Adrenocorticotropic Hormone in Infantile Epileptic Spasms Syndrome: A Randomized Controlled Trial.

Infantile epileptic spasms syndrome is an early-onset epileptic encephalopathy. Its association with inborn errors of metabolism is underrecognized, and the treatment can be complicated by the biochemical vulnerability. This report showed individualized treatment in 2 patients with inborn errors of metabolism. The first patient was found to have propionic acidemia in the setting of encephalopathy by third day of life. Infantile epileptic spasms syndrome was diagnosed at 10 months. Levetiracetam was started, and vigabatrin was introduced following liver transplant, which resulted in seizure freedom. The second patient presented at 6 months with hyperammonemia and elevated orotic acid consistent with ornithine transcarbamylase deficiency. Infantile epileptic spasms syndrome was diagnosed 3 weeks later. Treatment with vigabatrin, adjunctive to levetiracetam, led to a brief period of seizure cessation. The patient was later treated with two 4-week courses of enteral prednisolone, leading to the cessation of spasm. These cases emphasize the complexity in managing infantile epileptic spasms syndrome in patients at high risk for metabolic decompensation.

The potential benefits of the ketogenic diet (KD) on ageing are currently receiving increasing attention. Although there are various studies on this subject in the existing literature, there is a lack of systematic review and bibliometric analysis. This study aimed to present a bibliometric overview and visualization analysis of the existing studies examining the relationship between KD and ageing, identify trends in this field, and provide a basis for future research and sustainable development goals. This study involved a systematic review of the literature. In this study, Scopus (Elsevier) and Web of Science Core Collection (WoSCC) databases were used for bibliometric analysis. In the study, all articles, reviews, and other types of publications on KD and ageing published between 1995 and 2024 were analysed. Studies covering the years 1995-2025 and including the keywords 'ketogenic diet OR ketogenic diets OR ketone diet AND aging AND PUB YEAR > 1995 AND PUBYEAR < 2025' in the title were included. The VOSviewer software (VOSviewer v.1.6.10) was utilized to visualize the data. The data obtained were evaluated by bibliometric methods, such as keyword analysis and cluster analysis. A significant increase in the number of studies on KD and ageing was observed. In the study, when the data obtained from WoSCC and Scopus databases and VOSviewer analysis results were evaluated together, a total of 10,170 scientific documents in the Scopus database and a total of 168 scientific documents were identified in the Web of Science database between 1995-2025 worldwide. The author publishing the most on the subject was found to be Cunnane, S.C. The country contributing the most to the field was found to be the United States of America (USA). The institution that produced the most documents was Harvard Medical School. In a total of 10,170 records, the most preferred type of publication was articles. Nutrients journal was the journal with the highest number of publications. According to the results of keyword analysis, the words "ketogenic diet" and "aging" were the most frequently used and most strongly related words. The results of this study showed a significant increase in the number of studies investigating the effects of KD on ageing. More high-quality, randomised controlled clinical trials are needed in this field. In particular, there is a lack of studies examining the effects of KD on age-related diseases at the molecular level.

Infantile and epileptic spasms syndrome (IESS) is a childhood epilepsy syndrome characterized by infantile or late-onset spasms, abnormal neonatal EEG, and epilepsy. Few treatments exist for IESS, clinical outcomes are poor, and the molecular and circuit-level etiologies of IESS are not well understood. Multiple human IESS risk genes are linked to Wnt/β-catenin signaling, a pathway that controls developmental transcriptional programs and promotes glutamatergic excitation via β-catenin's role as a synaptic scaffold. We previously showed that deleting adenomatous polyposis coli (APC), a component of the β-catenin destruction complex, in excitatory neurons (APC cKO mice, APCfl/fl x CaMKIIαCre) increased β-catenin levels in developing glutamatergic neurons and led to infantile behavioral spasms, abnormal neonatal EEG, and adult epilepsy. Here, we tested the hypothesis that the development of GABAergic interneurons (INs) is disrupted in APC cKO male and female mice. IN dysfunction is implicated in human IESS, is a feature of other rodent models of IESS, and may contribute to the manifestation of spasms and seizures. We found that parvalbumin-positive INs (PV+ INs), an important source of cortical inhibition, were decreased in number, underwent disproportionate developmental apoptosis, and had altered dendrite morphology at P9, the peak of behavioral spasms. PV+ INs received excessive excitatory input, and their intrinsic ability to fire action potentials was reduced at all time points examined (P9, P14, P60). Subsequently, GABAergic transmission onto pyramidal neurons was uniquely altered in the somatosensory cortex of APC cKO mice at all ages, with both decreased IPSC input at P14 and enhanced IPSC input at P9 and P60. These results indicate that inhibitory circuit dysfunction occurs in APC cKOs and, along with known changes in excitation, may contribute to IESS-related phenotypes.SIGNIFICANCE STATEMENT Infantile and epileptic spasms syndrome (IESS) is a devastating epilepsy with limited treatment options and poor clinical outcomes. The molecular, cellular, and circuit disruptions that cause infantile spasms and seizures are largely unknown, but inhibitory GABAergic interneuron dysfunction has been implicated in rodent models of IESS and may contribute to human IESS. Here, we use a rodent model of IESS, the APC cKO mouse, in which β-catenin signaling is increased in excitatory neurons. This results in altered parvalbumin-positive GABAergic interneuron development and GABAergic synaptic dysfunction throughout life, showing that pathology arising in excitatory neurons can initiate long-term interneuron dysfunction. Our findings further implicate GABAergic dysfunction in IESS, even when pathology is initiated in other neuronal types.

This study aimed to investigate the effectiveness, tolerability, and retention of the ketogenic diet (KD) in patients with infantile epileptic spasms syndrome (IESS). In this single-center prospective cohort study, baseline data were collected from the Children's Hospital Affiliated to Shandong University. Follow-up assessments were conducted at 3, 6, and 12 months after initiating KD. Outcomes included seizure frequency, adverse reactions, and retention rates. Survival analysis was performed to examine the association between retention rates and follow-up duration. A total of 74 patients with IESS were admitted. The seizure response rates were 56.8% (42/74), 41.9% (31/74), and 25.7% (19/74) at 3, 6, and 12 months, respectively. The corresponding seizure-free rates were 13.5% (10/74), 13.5% (10/74), and 8.1% (8/74), respectively. Retention rates at 3, 6, and 12 months were 70.2% (52/74), 43.2% (32/74), and 25.7% (19/74), respectively. Survival analysis indicated that retention rates decreased over time, most markedly within the first 3 months. Responders to the KD exhibited significantly higher retention rates than non-responders throughout the 12 months (HR = 0.35, 95% CI: 0.19-0.64; p < 0.001). Adverse reactions were reported in 20.3% of patients, with gastrointestinal symptoms being the most common (16.2%), including constipation (6.8%), diarrhea (5.4%), and vomiting (4.0%). The KD demonstrated favorable effectiveness and an acceptable safety profile in patients with IESS, particularly in those who did not respond to first-line antiseizure medications (ASMs). The higher retention rates among responders supports its utility in children with IESS. Therefore, early initiation of the KD should be considered for IESS patients who do not respond to first-line ASMs.

ABSTRACTPurposeImaging diagnosis of mild cognitive impairment (MCI) has garnered increasing attention due to its critical role in the early detection of Alzheimer's disease (AD) and other dementias. This study presents a bibliometric analysis to elucidate global research trends, key contributors, thematic clusters, and emerging topics within the field of MCI imaging diagnosis.MethodEnglish‐language publications related to MCI imaging were retrieved from the Web of Science Core Collection (WoSCC) from January 1999 to December 2024. Bibliometric analyses were performed using VOSviewer, CiteSpace, and R‐bibliometrix to evaluate co‐authorship networks, institutional collaborations, journal impact, keyword co‐occurrence, and burst trends.FindingA total of 7,568 articles showed an average annual growth of 22.27%, with output surging after 2007 and peaking in 2024 (n = 762). The United States led in productivity and impact, ahead of China and Italy. Leading institutions were the University of California System, Vrije Universiteit Amsterdam, and the University of London, with key authors including Clifford R. Jack Jr., Ronald C. Petersen, and Philip Scheltens. Core journals were Neurology, Neuroimage, and Brain. Cluster analysis revealed four themes: functional and cognitive networks, biomarkers and pathology, structural imaging and computational diagnostics, and guidelines. Recent trendsc (AI) (e.g., machine learning, deep learning), while citation bursts indicate an evolution from early biomarker and imaging research toward current AI and multimodal imaging for improved diagnosis and risk prediction.ConclusionThis bibliometric analysis provides a comprehensive overview of the evolving research landscape in MCI imaging diagnosis. The integration of advanced computational methodologies, particularly AI‐powered tools, is driving precision diagnostics and personalized medicine. These advancements hold significant potential to improve early detection, stratify risk, and inform therapeutic interventions, ultimately contributing to better outcomes for individuals with MCI.

Despite the growing interest in stem cell therapy for kidney disease research, there remains a lack of comprehensive and systematic bibliometric analyses in this field. This study aimed to summarize the progress in stem cell therapy for kidney disease through bibliometric analysis, providing insights into the development and direction of the field. Publications related to kidney disease and stem cell therapy in the last 17years were retrieved from the Web of Science Core Collection (WOSCC) database on July 15, 2025. In conducting a comprehensive bibliometric analysis, a variety of bibliographic elements were meticulously collected to map the landscape of research within a specific field. Between January 1, 2009, and July 15, 2025, a total of 3,914 articles on stem cell therapy research in kidney disease were published across 186 academic journals by 19,195 authors from 3906 institutions in 90 countries. The volume of publications has shown a steady increase over this period. The United States and China lead in both publication and citation counts. Harvard University and University of California System are the most active institutions in this research domain. Lilach O. Lerman from the Mayo Clinic College of Medicine and Science was noted for the highest number of significant publications and received the most co-citations. The analysis revealed key research themes, including pluripotent stem cells, exosomes, and extracellular vesicles. This study provided a comprehensive bibliometric and knowledge mapping analysis of stem cell therapy in kidney disease research landscape, offering valuable insights into the trends, key contributors, and thematic focus areas within the field. By delineating the evolution of this research, this study aims to guide future scholarly endeavors and enhance our understanding of stem cell therapeutic potential.

Background: Infantile epileptic spasms syndrome is an epileptic encephalopathy, characterized by spasms, hypsarrhythmia, and developmental regression. Appropriately selected patients with infantile epileptic spasms syndrome may be candidates for epilepsy surgery. Methods: This is a single-center retrospective case series of children 0-18 years with a current or previous diagnosis of infantile epileptic spasms syndrome with a lesion on magnetic resonance imaging (MRI) and/or positron emission tomography scan who underwent epilepsy surgery at The Hospital for Sick Children (HSC) in Toronto, Canada. The records of 223 patients seen in the infantile epileptic spasms syndrome clinic were reviewed. Results: Nineteen patients met inclusion criteria. The etiology of infantile epileptic spasms syndrome was encephalomalacia in 6 patients (32%), malformations of cortical development in 12 patients (63%), and atypical hypoglycemic injury in 1 patient (5%). Nine patients (47%) underwent hemispherectomy, and 10 patients (53%) underwent lobectomy/lesionectomy. Three patients (16%) underwent a second epilepsy surgery. Fifteen patients (79%) were considered ILAE seizure outcome class 1 (completely seizure free; no auras) at their most recent follow-up visit. The percentage of patients who were ILAE class 1 at most recent follow-up decreased with increasing duration of epilepsy prior to surgery. Developmental outcome after surgery was improved in 14 of 19 (74%) and stable in 5 of 19 (26%) patients. Conclusions: Our study found excellent seizure freedom rates and improved developmental outcomes following epilepsy surgery in patients with a history of infantile epileptic spasms syndrome with a structural lesion detected on MRI brain. Patients who undergo surgery earlier have improved seizure freedom rates and improved developmental outcomes.