Frontiers in Neurology最新文献

Objective: This real-world study evaluated the efficacy and safety of telitacicept, a dual BAFF/APRIL inhibitor, in patients with generalized myasthenia gravis (MG).

Methods: In this retrospective study, 17 myasthenia gravis patients on stable background therapy received weekly subcutaneous telitacicept (160-240 mg). Efficacy was evaluated at 12/24 weeks using a primary composite endpoint (≥2-point MG-ADL and ≥3-point QMG reduction). A pre-specified Bayesian analysis, updating a conservative prior with observed data, was employed to determine the posterior probability of treatment success and its 95% credible interval. Safety and steroid-sparing effects were also assessed.

Results: Of the 15 patients evaluable for efficacy (≥12 weeks treatment), 12 (80.0%) met the composite efficacy endpoint. Significant improvements were observed: mean MG-ADL decreased from 8.0 ± 4.4 to 4.2 ± 3.1 (p < 0.01); mean QMG decreased from 13.8 ± 5.5 to 7.6 ± 4.3 (p < 0.01). A robust steroid-sparing effect was demonstrated: the mean daily prednisone dose decreased by 76.0% (10.41 ± 7.30 mg to 2.50 ± 3.21 mg, p < 0.05), with 3 patients achieving complete withdrawal. Five patients attained Minimal Symptom Expression. Bayesian analysis yielded a posterior mean efficacy rate of 66.67% (95% CrI: 49.99-81.43%), with a probability (P) of exceeding 50% efficacy at 97.49%. Treatment was well-tolerated: only mild, transient AEs occurred (one injection-site reaction, one gastrointestinal event leading to withdrawal), and no serious adverse events (SAEs) were reported.

Conclusion: This real-world study utilizing Bayesian analysis provides evidence supporting a high probability of efficacy for telitacicept in AChR-Ab-positive MG, demonstrating significant symptom improvement, substantial steroid-sparing effects, and favorable safety. These findings complement prior randomized controlled trial data and support the use of telitacicept in clinical practice. Study limitations include retrospective design and small sample size.

Objective: To assess the efficacy, tolerability, and safety of vagus nerve stimulation (VNS) in pediatric refractory epilepsy with myoclonic seizures.

Methods: We conducted a retrospective monocentric study at a pediatric center specializing in myoclonic seizures. This study included 19 children (13 males, 6 females; mean age 5.8 years, range: 2-14 years) who underwent VNS implantation between January 2019 and July 2025. Myoclonic seizures were confirmed by video electroencephalogram (v-EEG). The median number of Anti-seizure Medications (ASMs) at implantation was 3.1 (IQR: 2-4). The mean follow-up duration was 31 months (range: 12-56 months).

Results: Patients exhibited various seizure types, including infantile spasms, myoclonic, myoclonic-tonic, generalized tonic-clonic, generalized tonic, and focal seizures. At the last follow-up, 10 patients (52.6%) achieved ≥50% seizure reduction, and 4 (21.1%) attained seizure freedom. The seizure freedom rate was 31.6% for myoclonic seizures.

Significance: VNS demonstrates promise as a safe and effective treatment for pediatric refractory epilepsy (PRE). The seizure freedom rate for myoclonic seizures was particularly noteworthy. These findings suggest that VNS should be considered an early intervention to optimize myoclonic seizure control outcomes.

Objective: This study aims to retrieve, evaluate, and summarize the existing evidence regarding the prevention of deep vein thrombosis (DVT) in patients with cerebral hemorrhage. The findings will provide a solid foundation for clinical nursing practice.

Design: This study presents a comprehensive evidence summary conducted in accordance with the standards set forth by the Evidence-Based Nursing Center at Fudan University. The adherence to these standards ensures the rigor and reliability of the findings presented herein.

Methods: Based on the '5S' evidence pyramid model, various evidence-based resources for the prevention of deep vein thrombosis in patients with cerebral hemorrhage were systematically retrieved. These resources include clinical decisions, best practices, guidelines, expert consensus, systematic reviews, and evidence summaries. The search period spans from January 2011 to April 2025.

Results: This study included a total of 16 high-quality articles, comprising 2 clinical decisions, 7 guidelines, 4 expert consensuses, 2 systematic reviews, and 1 summary of evidence. In total, 38 pieces of evidence were synthesized across six dimensions: risk assessment, basic prevention, mechanical prevention, pharmacological prevention, nursing documentation, and informed consent.

Conclusion: This study summarizes the 38 most compelling pieces of evidence for the prevention of DVT in patients with cerebral hemorrhage, providing an evidence-based foundation for clinical medical staff. It is recommended that healthcare professionals implement these evidence-based practices in clinical settings to effectively reduce the incidence of DVT among patients suffering from cerebral hemorrhage.

Systematic review registration: http://ebn.nursing.fudan.edu.cn/registerResources, identifier ES2025786.

Objective: To investigate the connectivity and formation mechanism of the whole brain resting-state network in cingulate gyrus epilepsy and to identify biological markers and potential neuromodulation targets for this condition.

Methods: Fifteen patients with cingulate gyrus epilepsy and 15 healthy controls underwent resting-state magnetoencephalography (MEG). To compute functional network connectivity at the source level, we used MEG Processor software. Twenty regions of interest (ROI) were selected from both cerebral hemispheres, and connectivity was assessed across four frequency bands: theta (4-7.5 Hz), alpha (8-13 Hz), beta (14-30 Hz), and gamma (31-80 Hz).

Results: The number of neocortical-related functional connectivity differences increased with the frequency band, being smallest in the theta (θ) band and largest in the gamma (γ) band. The connections between the angular gyrus (AG) and the occipital gyrus (OG) and between the OG and the superior temporal gyrus (STG) were the most influential in terms of functional connectivity within the neocortex. The connectivity between the anterior cingulate cortex (ACC) and the inferior frontal gyrus (IFG) showed the most pronounced differences in the α, β, and γ bands. Among the functional connectivities to the posterior cingulate gyrus (PCC), those involving the AG-PCC and STG-PCC were the most significant. The hippocampal-related functional connectivity differed from neocortex-related functional connectivity, and the number of differential functional connections was greater in the θ-band than in the α-band.

Conclusion: Enhanced functional connectivity (AG-OG and OG-STG) of the neocortical surface may be characteristic features of the resting-state network in cingulate gyrus epilepsy and could serve as potential biological markers for this condition. The IFG's close relationship with the ACC suggests it may be a candidate target for neuromodulation therapy in anterior cingulate gyrus epilepsy. Similarly, the AG and STG's connections with the PCC make them potential candidates for neuromodulation therapy in posterior cingulate gyrus epilepsy for future investigation.

Background: Depressive symptoms are very common in the acute phase of stroke; however, its impact on distinct functional recovery trajectories in acute ischemic stroke (AIS) patients remains unclear. Our study aimed to depict the functional recovery trajectories within 6 months after stroke and explore the association of early depressive symptoms with these recovery patterns among AIS patients.

Methods: A total of 219 eligible patients were enrolled at the stroke centers of two tertiary hospitals in Xuzhou, China from April 2023 to June 2024. The Center for Epidemiologic Studies Depression Scale (CES-D) was used to assess depressive symptoms during the acute hospitalization. The Group-based trajectory model was conducted to identify distinct trajectories of functional recovery, as measured by modified Rankin Scale (mRS) and Barthel Index (BI) at baseline, 3 months, and 6 months. A series of multinomial logistic regression models were performed to examine the relationship between early depressive symptoms and dynamic recovery patterns.

Results: We identified 3 mRS trajectories (mild, moderate, and severe) and 5 BI trajectories (low-rapid rise, moderate low-stable, moderate-progressive rise, moderate high-rapid decline, and high-stable), respectively. After full adjustments, patients with early depressive symptoms were at increased likelihood of being in the moderate (OR 8.22, 95% CI 2.77-24.39) and severe (OR 24.41, 95% CI 5.33-111.90) trajectory group for mRS trajectories, and of the moderate high-rapid decline (OR 12.93, 95% CI 1.49-112.42) trajectory group for BI trajectories ( p < 0.05).

Conclusion: Early depressive symptoms were associated with unfavorable functional recovery trajectories within 6 months following acute stroke in AIS patients.

Objective: This study was designed to clarify the clinical characteristics of myelin oligodendrocyte glycoprotein (MOG) antibody-positive anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis.

Methods: This was a single-center retrospective study. Patients with anti-NMDAR encephalitis hospitalized at Tianjin Huanhu Hospital were included in the study. The observation group consisted of patients with anti-NMDAR encephalitis who tested positive for serum MOG-antibodies [MOG-Ab (+)], while the reference group included patients who tested negative for serum MOG-antibodies [MOG-Ab (-)]. Clinical data were collected from both groups and statistical methods were employed to analyze the differences between the two groups.

Results: This study enrolled 48 patients (n = 48) with anti-NMDAR encephalitis, comprising eight cases (n = 8, 16.67%) in the MOG-Ab (+) group and 40 cases (n = 40, 83.33%) in the MOG-Ab (-) group. The proportion of male patients in the MOG-Ab (+) group was significantly higher than that in the MOG-Ab (-) group (87.50 vs. 40.00%, χ2 = 4.274, P = 0.039). Patients in the MOG-Ab (+) group frequently experienced headaches, which occurred more often than in the MOG-Ab (-) group (75.00 vs. 25.00%, χ2 = 5.419, P = 0.020). The median white blood cell (WBC) count in the cerebrospinal fluid (CSF) of the MOG-Ab (+) group was 125.00 (65.00, 155.00) × 10⁶/L, representing a rate that is 12.5 times higher than that of the MOG-Ab (-) group, indicating more pronounced immune inflammatory response in the CSF of the MOG-Ab (+) group (Z = -3.320, P = 0.000). Additionally, the MOG-Ab (+) group exhibited a higher proportion of leptomeningeal enhancement (37.50 vs. 2.50%, P = 0.012) and cortical lesions (87.50 vs. 40.00%, χ2 = 4.274, P = 0.039) on MRI. Recurrence occurred in 50.0% of patients in the MOG-Ab (+) group within 1 year of discharge follow-up, compared to only 12.50% in the MOG-Ab (-) group (χ2 = 3.938, P = 0.047).

Conclusion: For patients with anti-NMDAR encephalitis presenting with headaches, if there is a significant increase in CSF-WBC count, coupled with abnormal MRI signals in the leptomeninges or cortex, it is recommended to test for MOG antibodies. This is particularly crucial for male patients. For those with positive double antibodies, more aggressive long-term immunosuppressive therapy may be necessary to prevent recurrence.

Epilepsy is a frequent complication observed among stroke survivors. Post-stroke epilepsy (PSE) is defined as the occurrence of at least two unprovoked seizures beyond 2 weeks of an acute stroke that are not due to any other identifiable cause. PSE constitutes a significant clinical concern in pediatric stroke patients, adversely affecting both short-term and long-term management outcomes. This systematic review aimed to identify patient-related, stroke-related, and seizure-related risk factors associated with the development of PSE in the pediatric population. We included all clinical studies that compared these variables between pediatric stroke patients who developed PSE and those who did not. Studies reporting potential predictors of PSE among children with stroke were incorporated into the analysis. A total of 16 studies comprising 3,198 patients were included. The pooled risk of PSE was 27.6%, with a 95% confidence interval ranging from 19.8 to 37.2% (p < 0.001). Statistically significant associations were observed between younger age at stroke onset (OR 0.838; 95% CI 0.796-0.883; p < 0.001), cortical involvement (OR 3.151; 95% CI 1.132-8.772; p = 0.028), middle cerebral artery involvement (OR 3.541; 95% CI 1.068-11.738; p = 0.039), and increased risk of PSE. Additionally, patients presenting with acute symptomatic seizures (HR 3.924; 95% CI 2.580-5.967; p < 0.001) and those experiencing prolonged acute symptomatic seizures (OR 4.7; 95% CI 2.286-9.662; p < 0.001) demonstrated a higher likelihood of developing PSE. Pediatric patients who are younger at stroke onset and exhibit cortical or middle cerebral artery involvement are at a substantially elevated risk for PSE. Furthermore, the presence of acute symptomatic seizures at stroke onset, particularly when prolonged, markedly increases the probability of subsequent PSE development.

CRISPR-Cas genome-editing technologies have emerged as powerful tools for precise DNA and RNA modulation, offering promising therapeutic strategies for neurodegenerative disorders such as Alzheimer's disease (AD), Parkinson's disease (PD), Huntington's disease (HD), and amyotrophic lateral sclerosis (ALS). This review critically evaluates current CRISPR/Cas applications in neurodegeneration, with emphasis on mechanistic insights, therapeutic outcomes, and translational feasibility. Preclinical and early translational studies demonstrate that CRISPR-Cas platforms can correct pathogenic mutations, suppress toxic gene expression, and restore neuronal function. Advanced modalities, including base and prime editing, CRISPRi/a, and RNA-targeting Cas systems, improve precision and reduce genomic damage, which is particularly advantageous in post-mitotic neurons. Emerging CRISPR-based diagnostics (e.g., SHERLOCK and DETECTR), AI-assisted sgRNA design, and machine-learning approaches for predicting off-target effects further enhance the safety, stratification, and monitoring of CRISPR therapeutics. In parallel, patient-derived brain organoids and assembloids provide scalable human-relevant platforms for mechanistic studies and preclinical validation. Despite this progress, major challenges remain, including efficient delivery across the blood-brain barrier, immune responses, long-term safety, and ethical and regulatory considerations. Overall, CRISPR-Cas technologies hold strong potential as disease-modifying interventions for neurodegenerative disorders, provided that advances in delivery systems, artificial intelligence integration, and regulatory oversight continue to evolve toward clinical translation.

Objective: This study aims to assess changes in the cross-sectional area of the tibial nerve in the disabled elderly.

Materials and methods: The study sample included 124 tibial nerves in 62 participants; 16 of which were disabled elderly patients (13 males, 3 females), with a mean age of 66.6, a mean height of 167.1 cm, a mean weight of 83.9 kg, and a mean BMI of 29.9. Twenty-three young controls (6 males, 17 females), a mean age of 48.4, a mean height of 154.9 cm, a mean weight of 79.8 kg, a mean BMI of 32.7, and 23 elderly non-disabled participants (13 males, 10 females), a mean age of 63.6, a mean height of 161.9 cm, a mean weight of 80.56 kg, and a mean BMI of 30.88.

Results: The mean CSA of the TN in the elderly disabled group was 28.5 mm². The mean CSA of the young control group's TN was 20.45 mm². The mean CSA of the TN in the elderly non-disabled group (both diabetic and non-diabetic) was 27.6 mm². The mean CSA of the TN in the elderly diabetic non-disabled group was 29.9 mm². The mean CSA of the TN in the elderly (non-diabetic) non-disabled group was 24.6 mm².

Conclusion: In conclusion, although our study suggests that nerve ultrasound could be a helpful tool for assessment of the tibial nerve in disabled elderly patients, disability status was not an independent predictor of tibial nerve CSA. Future studies with a larger sample size and a homogenous group are suggested.