PURPOSE:To characterise clinical features of Rasmussen encephalitis (RE) and identify factors associated with preoperative neurodevelopmental status in affected children. METHODS:This retrospective single-centre study included 51 paediatric patients diagnosed with RE who underwent surgical treatment. Demographic characteristics, seizure features, video-electroencephalography (VEEG) findings, magnetic resonance imaging (MRI) features, and neurodevelopmental assessments were systematically collected. Associations between full-scale developmental quotient/intelligence quotient (FSDQ/IQ) and clinical variables, including age at onset, disease duration, electroencephalography findings, MRI characteristics, and number of antiseizure medications (ASMs), were analysed. RESULTS:All patients underwent hemispherotomy or hemispherectomy and were seizure-free at the latest follow-up, although one experienced postoperative seizures from the contralateral hemisphere. Epilepsia partialis continua occurred in 54.9% of patients, and 52.9% had contralateral independent epileptiform discharges. Early MRI showed hemispheric or focal atrophy in 49%. Preoperative neurodevelopmental assessment was completed in 45 patients, with a mean FSDQ/IQ of 70.5. Overall, 55.6% demonstrated normal or borderline development, and 26.7% had mild developmental delay. Motor function was the most affected domain, whereas cognitive and non-motor domains were generally borderline. Patients with contralateral epileptiform discharges had lower FSDQ/IQ than those without (p = 0.001), and this association remained significant after adjustment for age at seizure onset, disease duration, and number of ASMs. CONCLUSION:Preoperative neurodevelopmental status in children with RE was heterogeneous, with overall scores in the borderline range. Contralateral independent epileptiform discharges were independently associated with lower FSDQ/IQ, although the underlying mechanisms and clinical significance of this association remain uncertain. These findings support close developmental monitoring and individualised preoperative assessment in RE.

OBJECTIVE:Invasive presurgical evaluation plays a key role in pediatric epilepsy surgery, particularly in magnetic resonance imaging (MRI)-negative cases, by guiding resective, disconnective, or ablative procedures. This International League Against Epilepsy (ILAE) Pediatric Epilepsy Surgery Taskforce study provides an updated global overview of current invasive evaluation practices. METHODS:Group-level data were collected from 61 epilepsy surgery programs (49 pediatric-only) in 29 countries across six continents. Included were children and adolescents who underwent presurgical evaluation and epilepsy surgery in 2023. The study was designed to enable comparison with the similar ILAE survey conducted in 2004. RESULTS:A total of 2427 patients were included. Invasive evaluations were performed in 21.1% of cases, most frequently in North America (33.7%, higher than Europe: 18.0%, p = .003). Among invasive cases, 32.3% had no detectable MRI abnormalities. The main indication for invasive evaluation was seizure onset localization (88.1%), followed by motor or sensory mapping (17.2%) and language mapping (14.2%). Stereoelectroencephalography (SEEG) was the predominant technique (19.8% overall, 93.6% of invasive cases), more common in North America (30.0%, p = .021) and less common in South America (7.4%, p < .001). Subdural electrodes were used in only 3.2% of invasive cases, and combined depth and subdural approaches in 3.2%. SEEG-guided radiofrequency thermocoagulation (RF-TC) was performed in 40.9% of SEEG cases, most commonly in Asia (63.8%). In 16.2% of invasive evaluations, patients did not proceed to resection, disconnection, or ablation, with the highest rate in Europe (28.5%). SIGNIFICANCE:This global survey provides the first broad overview of invasive evaluation practices in pediatric epilepsy surgery across participating centers worldwide. It highlights the widespread adoption of SEEG, declining use of subdural electrodes, and increasing application of SEEG-guided RF-TC. The high proportion of MRI-negative cases and the considerable proportion of patients not proceeding to resection, disconnection, or ablation underscore the complexity of contemporary surgical candidates and the need for further refinement of selection strategies.

OBJECTIVE:Pediatric epilepsy surgery is well established, but contemporary global data on referral and presurgical evaluation practices are lacking. This International League Against Epilepsy (ILAE) Pediatric Epilepsy Surgery Task Force study provides an updated overview of current trends and regional differences. METHODS:Group-level data were collected from 61 epilepsy surgery programs (49 pediatric-only) across 29 countries and six continents, identified through ILAE networks, and included all children and adolescents treated in 2023 who underwent presurgical evaluation/epilepsy surgery. RESULTS:Group-level data were available for 2427 patients. Mean age at surgery was 9.1 ± 4.9 years; mean epilepsy duration was 5.3. At surgery, 3.2% were <1 year old (highest in Oceania: 5.1%), and 6.1% were nonpharmacoresistant (highest in Europe: 15.0%). Prior neurosurgery was reported in 14.2% (highest in North America: 28.8%), including 8.0% resections (6.1% for epilepsy, 1.5% for tumors; highest in Oceania: 16.5%), 2.3% disconnections (1.3% corpus callosotomy; highest in South America: 4.7%), and 4.2% neuromodulation (3.7% vagal nerve stimulation, .2% responsive neurostimulation, one deep brain stimulation; highest in North America: 12.2%). Developmental and epileptic encephalopathies (DEEs) at surgery included Lennox-Gastaut syndrome (7.4%), infantile epileptic spasms syndrome (5.1%), and DEE with spike-wave activation in sleep (1.5%). Presurgical investigations included fluorodeoxyglucose positron emission tomography (52.6%; highest in Oceania: 79.7%), genetic testing (46.8%; highest in Asia: 54.3%), magnetic resonance imaging (MRI) postprocessing (32.4%; highest in South America: 53.0%), functional MRI (fMRI; 15.2%; highest in North America: 40.3%), magnetoencephalography (11.9%; highest in North America: 39.3%), single photon emission computed tomography (9.6%; highest in North America: 22.2%), high-density electroencephalography (EEG; 1.9%; highest in Europe: 4.7%), source localization (1.6%; highest in Oceania: 7.6%), Wada test (1.2%; highest in North America: 3.5%), and EEG-fMRI (.5%; highest in Europe: 1.1%). SIGNIFICANCE:Despite some early surgeries, including in infancy and before pharmacoresistance, mean epilepsy duration before surgery remains >5 years. Reoperations are common, with resection more frequent than neuromodulation. Genetic testing in nearly half of patients reflects its growing relevance, and the high rate of DEEs underscores the complexity of surgical candidates.

OBJECTIVE:The glymphatic system (GS) facilitates perivascular clearance of interstitial solutes and is modulated in part by neuronal activity. However, its relationship to cortical excitability in epilepsy remains unclear. We aim to clarify the mechanistic link between GS function and cortical excitation-inhibition (E-I) balance in patients with epilepsy. METHODS:We investigated this coupling in patients with Rasmussen encephalitis (RE), a rare epileptic disorder with unilateral cortical pathology. Using a hemispheric within-subject design (N = 20), we compared the affected hemisphere (AH) and unaffected hemisphere (UH) within each patient, assessing glymphatic function via diffusion MRI (diffusion tensor imaging-analysis along the perivascular space [DTI-ALPS] index) and E-I dynamics via resting-state EEG spectral decomposition. RESULTS:The AH exhibited reduced DTI-ALPS indices, elevated aperiodic exponents, and increased delta-theta oscillatory power. Across individuals, lower ALPS values in the AH correlated with higher aperiodic exponents (r = -.496, p = .026), but not with periodic EEG features. Principal component analysis (PCA) of channel-level aperiodic asymmetry (exponent-informed PCA) revealed a spatial pattern localized to atrophic cortical regions, which also showed strong correspondence with interhemispheric ALPS asymmetry (r = -.570, p = .009). SIGNIFICANCE:These findings demonstrate a spatially convergent link between glymphatic dysfunction, cortical activity with predominant inhibitory tone, and focal atrophy in the AH. Our results indicate that impaired perivascular clearance is correlated with altered cortical excitability, highlighting the potential role of the GS in maintaining neural stability in the epilepsy.

OBJECTIVE:Rasmussen encephalitis (RE) is a rare progressive disorder causing drug-resistant epilepsy. Hemispheric surgery is an established treatment, but comprehensive data on postoperative seizure, motor, and cognitive outcomes are limited. We aimed to evaluate these outcomes and identify associated prognostic factors. METHODS:This dual-center retrospective study included RE patients who underwent hemispheric surgery at two tertiary epilepsy centers in China. Seizure outcomes were classified by Engel class, with Engel class I regarded as seizure-free. Motor outcomes were evaluated using the Motricity Index (MI), Gross Motor Function Classification System (GMFCS), and Manual Ability Classification System (MACS), with stable outcomes defined as improvement or no change between baseline and follow-up. Cognitive function was evaluated using standardized scales. Multivariable Cox regression identified factors associated with seizure and motor outcomes. RESULTS:Eighty-five patients (52 female, 63%) with median follow-up of 2.75 years were included. At last follow-up, 79% were seizure-free after initial hemispheric surgery. Hemispheric disconnection showed better seizure control (92%) than anatomic (67%) or functional hemispherectomy (57%), with shorter surgery and less blood loss. Stable gross motor function was achieved in 94%, whereas 64% experienced worsening fine motor skills. Generalized seizures (hazard ratio [HR] 5.48, 95% confidence interval [CI] 1.63-18.42), contralateral magnetic resonance imaging (MRI) abnormalities (HR 6.43, 95% CI 1.14-36.28), contralateral interictal electroencephalography (EEG) discharges (HR 4.61, 95% CI 1.57-13.51), and type of hemispheric surgery (HR 5.25, 95% CI 1.57-13.51) were associated with seizure persistence. Postoperative seizure-free (HR 4.43, 95% CI 1.61-12.17) and baseline MI (HR .94, 95% CI .91-.96) predicted overall motor stability, whereas fine motor stability was related to epilepsia partialis continua (EPC) duration (HR .50, 95% CI .25-.98), preoperative immunotherapy (HR 3.30, 95% CI 1.13-9.59), and baseline MACS (HR 2.45, 95% CI 1.68-3.57). SIGNIFICANCE:This study suggests that hemispheric surgery is effective in achieving seizure-free and favorable gross motor recovery in RE. Early surgery, attention to contralateral abnormalities, and preoperative immunotherapy may further improve outcomes.

Unraveling the cellular and molecular characteristics of human prefrontal cortex (PFC) development is crucial for understanding human cognitive abilities and vulnerability to neurological and neuropsychiatric disorders. Here, in this study, we created a comparative repository for gene expression, chromatin accessibility and spatial transcriptomics of human and macaque postnatal PFC development at single-cell resolution. Integrative analyses outlined species-specific dynamic trajectories of different cell types, highlighting key windows and gene regulatory networks for processes such as synaptogenesis, synaptic pruning and gliogenesis. We identified regulatory correlates of the prolonged development of human PFC relative to macaques. Glial progenitors showed higher proliferation capability in humans compared to macaques, associated with distinct gene expression profiles. Furthermore, we uncovered cell types and lineages most susceptible to neurodevelopmental and neuropsychiatric disorders, focusing on transcription factors with human-specific expression features. In summary, our discoveries shed light on human-specific regulatory programs extending postnatal cortical maturation through coordinated neuronal and glial development, with implications for cognition and neurodevelopmental disorders.

Mild malformation of cortical development with oligodendroglial hyperplasia in epilepsy (MOGHE) is a new histopathological entity identified in the surgically resected brain tissue of patients with drug-resistant epilepsy. Somatic variants in SLC35A2 have been increasingly identified in MOGHE brain resections. SLC35A2 protein transports uridine 5'-diphosphogalactose (UDP-Gal) into the Golgi lumen, playing a crucial role in the process of N-glycosylation. Currently, research on the pathogenic mechanism of SLC35A2 variants in MOGHE is limited. Here we conducted genetic testing on brain samples and paired blood samples from 28 pediatric patients pathologically diagnosed with MOGHE. We performed an in-depth functional analysis of somatic variants identified in SLC35A2, integrating glycan labeling and intact glycopeptide profiling to assess N-glycosylation defects. With whole-exome sequencing and validation with ultra-deep amplicon sequencing, we identified 101 potentially pathogenic somatic variants (PPSVs) across 87 genes. Nine PPSVs in SLC35A2 were found in 10 samples. The 9 identified variants of SLC35A2, characterized by various mutation types (4 frameshift, 3 missense and 2 nonsense variants), were all confirmed to be loss-of-function via altered glycan chains. Intact glycopeptide analysis at the cellular level indicated an increase in truncated N-glycan glycoforms. Analysis of brain tissue revealed N-glycosylated proteins and glycosites modified with agalactosylated glycoforms, and glycoproteins bearing agalactosylated N-glycans were significantly enriched in cell adhesion and axon guidance-related pathways. Additionally, chemoenzymatic glycan labeling in lesions demonstrated N-glycan damage of heterotopic neurons, suggesting a potential diagnostic approach for MOGHE. Our findings provide a comprehensive somatic landscape of MOGHE and a rich resource of somatic SLC35A2 variant-related glycoform and glycoprotein abnormalities, thereby unveiling valuable insights into compromised N-glycosylation and MOGHE formation.

BackgroundEvidence regarding the efficacy of vagus nerve stimulation (VNS) in treating developmental and epileptic encephalopathy/ epileptic encephalopathy with spike-and-wave activation in sleep (DEE/EE-SWAS), particularly its impact on the SWAS remains limited. We present a boy with EE-SWAS who was treated with VNS at 4.8 years of age.Case presentationA male patient developed seizures at 2.3 years of age. At 2.8 years of age, electroencephalography (EEG) showed SWAS, leading to regression in cognitive, motor, and language functions. Administration of multiple anti-seizure medications (ASMs) achieved poor efficacy, and repeated corticosteroids resulted in only transient improvement. He was treated with VNS at 4.8 years of age. Seizure freedom was achieved at 1.3 years postoperatively. The SWAS pattern was not observed on follow-up EEG 2 years after implantation. Concurrently, his neurodevelopment improved. No new ASMs or corticosteroids were added during this period. VNS was interrupted due to pulse generator battery depletion at 5.6 years after implantation. Increased SWI was showed on the EEG 6 months after the interruption of stimulation.ConclusionEarly VNS intervention should be considered in addition to conventional medication for young children with SWAS who have greater distance from the self-limited age.

OBJECTIVE:This study aimed to evaluate the efficiency of an RF-TC Target Planning Software in enhancing outcomes of SEEG-guided RF-TC for pediatric drug-resistant epilepsy. METHODS:A retrospective analysis of 68 cases at Peking University First Hospital from May 2018 and November 2023 was conducted. Of these, software-assisted planning and manual planning were utilized in 27 and 41 patients, respectively. Surgical parameters, primarily the number of contact pairs, planning time, and postoperative results were analyzed. Statistical analysis compared seizure-free rates and other key metrics across the two planning groups. RESULTS:A larger group with software-assisted planning was associated with a significantly higher number of contact pairs for RF-TC and lower planning time. The software-assisted group's seizure-free rate was 77.78 % after 23 months, compared to 29.41 % over 57.97 months for the manual planning group. Transient contralateral limb weakness was observed in 11.11 % of software-assisted cases, but full recovery was eventually noted for all patients. INTERPRETATION:The RF-TC Target Planning Software enhances the effectiveness and efficiency of SEEG-guided RF-TC by improving electrode contact pair choice, reducing planning time, and increasing postoperative seizure-free rates. In complex situations, such as hypothalamic hamartomas, the software displays its value by reducing the risk of missed electrode pairings. Notwithstanding its advantages, the software-assisted approach may raise transient postoperative neurological deficits slightly. Additional studies with expanded cohorts and extended follow-ups are necessary for a comprehensive assessment of this technique's long-term effectiveness.

Alzheimer's disease (AD) is a predominant neurodegenerative disorder worldwide, with epileptic seizures being a common comorbidity that can exacerbate cognitive deterioration in affected individuals, thus highlighting the importance of early therapeutic intervention. It is determined that deletion of Ms4a4a, an AD-associated gene, exacerbates seizures in amyloid β (Aβ)-driven AD mouse model. MS4A4A is significantly upregulated in brain lesions in patients with epilepsy. Single-cell sequencing reveals that MS4A4A is highly expressed in microglia within these lesions, linked to enhanced phagocytic activity. Mechanistic investigation delineates that deletion of Ms4a4a impairs microglial phagocytosis, accompanied by diminished calcium influx and disruptions in mitochondrial metabolic fitness. The cytosolic fragment of Ms4a4a is anchored to the cytoskeletal components, supporting its critical role in mediating phagocytosis. Induction of Ms4a4a through central delivery of LNP-Il4 alleviates seizure conditions. Collectively, these findings identify Ms4a4a as a potential therapeutic target for managing seizures in AD treatment.

OBJECTIVE:To compare the efficacy and safety of vagus nerve stimulation using different modes in patients with drug-resistant epilepsy (DRE). METHODS:Children with DRE who underwent vagus nerve stimulator implantation between March 2019 and December 2022 were prospectively enrolled at the Peking University First Hospital. Ninety-four children were randomly divided into the high pulse amplitude [pulse amplitude was gradually increased to maximum tolerance value (≤2.8 mA) and duty cycle was maintained at 10%] and high duty cycle groups [pulse amplitude was maintained at 1.5 mA and duty cycle was subsequently increased to maximum tolerance value (≤37%)]. At the 55-week follow-up, the responder rate, seizure-free rate, and adverse effects were compared between the two groups. RESULTS:Ninety-four children with DRE were included (47 patients each in the high pulse amplitude group and the high duty cycle group). For epileptic seizures, the responder rate of the high pulse amplitude group was 53.2% (25/47), while that of the high duty cycle group was 38.3% (18/47). High pulse amplitude had a higher responder rate of focal seizures (60.7% in the high pulse amplitude group vs. 29.2% in the high duty cycle group; chi-squared test, p = 0.023). Among children with a ≥ 75% reduction in epileptic spasms, the high duty cycle group had a higher responder rate than the high pulse amplitude group (54.5% vs. 16.7%; Fisher's exact test, p = 0.022). In addition, in the high duty cycle group, the responder rate in children with epileptic spasms was higher than that in children without epileptic spasms (59.1% vs. 20.0%; chi-square test, p = 0.011). Among children with implantation age <6 years, the responder rate of the high pulse amplitude group was higher (chi-square test, p = 0.024). The incidence of adverse effects in the two groups was not statistically different. SIGNIFICANCE:The overall responder rates in the two modes were similar. The high pulse amplitude mode may be more effective for focal seizures, whereas the high duty cycle mode may be more effective for epileptic spasms. Patients aged <6 years with DRE and VNS implantation may require a higher pulse amplitude to achieve better efficacy. PLAIN LANGUAGE SUMMARY:This study compared the anti-seizure efficacy of two different vagus nerve stimulation (VNS) treatments. We found that the overall efficacy of the two treatments was similar. The high pulse amplitude mode may be more effective for focal seizures, whereas the high duty cycle mode may be more effective for epileptic spasms. Patients aged <6 years with drug-resistant epilepsy (DRE) and VNS treatment may require a higher pulse amplitude to achieve better efficacy.

OBJECTIVE:Conventional multimodal imaging, including MRI and fluorodeoxyglucose positron emission tomography (FDG-PET), has difficulty in accurately detecting subtle or blurred focal cortical dysplasia (FCD) lesions. Morphometric maps assist localization by highlighting abnormal regions, whereas wavelet-filtered images emphasize texture and edge details. Therefore, we propose a three-branch feature enhancement and fusion network (TBFEF-Net) that integrates conventional multimodal imaging, morphometric maps, and wavelet-filtered images to enhance the accuracy of FCD localization. METHODS:The proposed TBFEF-Net comprises a semantic segmentation backbone, a cross-branch feature enhancement (CFE) module, and a multi-feature fusion (MFF) module. In the semantic segmentation backbone, three UNet-based branches separately extract semantic features from conventional multimodal imaging, morphometric maps, and wavelet-filtered images. In the encoding stage, the CFE incorporates a residual-based convolutional block attention module (CBAM) to aggregate features from all branches, enhancing the feature representation of FCD lesions. While in the decoding stage, the MFF integrates edge detail features from the wavelet-filtered imaging branch into the conventional multimodal imaging branch, enhancing the ability to capture lesion edges. As a result, this approach enables more precise segmentation. RESULTS:Experimental results show that TBFEF-Net surpasses several state-of-the-art methods in FCD segmentation. In the primary cohort, the Dice and sensitivity reached 59.73 % and 67.13 %, respectively, while in the open cohort, the Dice and sensitivity were 54.67 % and 54.81 %, respectively. SIGNIFICANCE:We introduced wavelet-filtered images for the first time in FCD segmentation, offering a novel approach and perspective for FCD lesions localization.

IntroductionFocal cortical dysplasia (FCD) is one of the common causes of refractory epilepsy. The subtle and indistinct edge of FCD lesions pose considerable challenges for accurate lesion localization. Therefore, we propose an edge guided segmentation network based on Laplacian pyramid to improve the localization performance of FCD lesions.MethodsThis is a retrospective study evaluated on two independent datasets. The proposed Laplacian Edge Mix UNet (LEM-UNet) builds upon the MedNeXt baseline and incorporates the Laplacian Edge Attention (LEA) block and the Multi-strategy Feature Fusion (MFF) block. LEA block captures lesion details and edge information during the encoding phase by integrating Laplacian pyramid feature maps with an attention mechanism, while MFF block fuses edge features with high level features during the decoding phase.ResultsThe model's performance was assessed through 5-fold cross-validation across both Open and Private Datasets, demonstrating superior performance. The average Dice Coefficient achieved 0.452 and 0.597 on the Open and Private Datasets, respectively, representing improvements of 2.40% and 2.90% compared to the baseline model.DiscussionThe results demonstrate the importance of focusing on lesion edge in the FCD segmentation task. The integration of the Laplacian pyramid enhances the mode's ability to capture lesions with blurred edge and subtle features. LEM-UNet exhibits significant advantages over current FCD segmentation algorithms. The source code and pre trained model weights are available at https://github.com/simplify403/LEM-UNet.

OBJECTIVE:Peri-Rolandic epilepsy in pediatric patients presents unique surgical challenges due to the involvement of eloquent cortical regions, where resective surgery carries risks of permanent neurological deficits. This study investigates lesion distribution, surgical outcomes, and the relationship between lesion location and postoperative motor function in children undergoing surgery for drug-resistant epilepsy in the peri-Rolandic area. METHODS:This retrospective study included 152 pediatric patients who underwent craniotomy for peri-Rolandic epilepsy between September 2014 and January 2023. Patients met the criteria of drug-resistant epilepsy, peri-Rolandic surgical resection, and a minimum follow-up of 6 months. Preoperative evaluations included video electroencephalography (VEEG), magnetic resonance imaging, and positron emission tomography, with invasive monitoring in select cases. Motor function and seizure outcomes were assessed using Engel classification and multivariate logistic regression to examine correlations between lesion location, motor deficits, and seizure prognosis. RESULTS:Lesions were most commonly found in the central operculum, affecting 66% of patients. Postoperative seizure freedom (Engel class I) was achieved in 80% of cases. Among patients with preoperative motor deficits (28%), 39.5% fully recovered after surgery, whereas 27.9% experienced permanent severe impairments. Resecting lesions in the precentral gyrus, paracentral lobule, and premotor cortex was significantly associated with long-term motor dysfunction. There was no significant association between the location of the lesion and postoperative seizure control. SIGNIFICANCE:Surgery for peri-Rolandic epilepsy in children is effective in achieving seizure control, although it carries risks of motor dysfunction. Lesion location should be carefully considered to optimize surgical outcomes, balancing seizure control with the preservation of motor function.

Preoperative pathological information of focal cortical dysplasia (FCD) is critical for surgical planning, but non-invasive diagnostic methods are currently unavailable. This study aimed to identify potential biomarkers for FCD subtyping by screening microRNAs (miRNAs) in plasma extracellular vesicles (EVs). We identified the most representative pathological subtypes based on the clinical characteristics of FCD Type I and II in 439 pediatric FCD patients. Differential expression analysis of miRNA was performed in plasma EVs and brain tissues from samples of representative pathological subtypes. Potential biomarkers and downstream target genes were identified by integrating brain tissue transcriptome data. Target genes underwent enrichment and protein-protein interaction analyses, with immunohistochemical validation in brain tissue. FCD Ia and IIb represent the clinical characteristics between FCD Type I and II, respectively. Eight differentially expressed miRNAs common to plasma EVs and brain tissue were identified, which had 241 target genes in brain tissue. These target genes were enriched in immune-related functions, cytokine-cytokine receptor interaction, p53, and NF-κB signaling pathways. Among nine core proteins, CDKN1A and CD274 were confirmed to be elevated in FCD IIb lesions compared to FCD Ia. Differentially expressed miRNAs in extracellular vesicles may serve as potential non-invasive biomarkers for FCD subtyping.

Objective: To investigate the alterations in cerebral white matter diffusion tensor metrics in individuals with Rasmussen’s encephalitis (RE). Methods: This retrospective study utilized diffusion tensor imaging (DTI) to assess cerebral white matter integrity. We included ten patients diagnosed with RE and ten healthy control subjects, matched for sex, age, and handedness. Employing tract-based spatial statistics (TBSS), we conducted a comprehensive analysis of DTI parameter indices in the cerebral white matter of all participants. The parameters measured included fractional anisotropy (FA), mean diffusivity (MD), axial diffusivity (AD), and radial diffusivity (RD). Results: Compared to the control group, patients with RE exhibited significantly elevated MD and RD values in several white matter tracts. Specifically, these differences were observed in the anterior thalamic radiation, corticospinal tract, cingulate gyrus, forceps minor, inferior fronto-occipital fasciculus, inferior longitudinal fasciculus, uncinate fasciculus, and the temporal part of the superior longitudinal fasciculus within the ipsilesional hemisphere. Additionally, increased MD and AD values were found in the anterior thalamic radiation, cingulate gyrus, inferior fronto-occipital fasciculus, and uncinate fasciculus in the contralateral hemisphere. Conclusion: Our findings indicate that RE is not confined to the cortical regions of the affected hemisphere. There is evidence of extensive white matter integrity compromise in patients with RE, which could potentially serve as the structural foundation for the emergence of affective disorders, cognitive impairments, and motor dysfunctions.

Abstract We retrospectively analyzed the clinical characteristics of three ARE cases with delayed‐onset seizures treated at Peking University First Hospital and Sanbo Brain Hospital from May 2021 to January 2023. We also reviewed previously reported atypical cases of Rasmussen's encephalitis (RE) in the literature, summarizing onset symptoms, seizure symptomatology, imaging findings, electroencephalogram (EEG) results, treatment course, and prognosis. The onset age of the three cases ranged from 1 year and 9 months to 7 years and 5 months. All three initially presented with limb motor disorders, which progressively worsened. Two cases developed focal seizures within 1 month of onset, whereas the third case had no seizures over 3 years. Brain MRIs revealed progressive unilateral hemispheric atrophy with multifocal abnormal signals, and PET‐CT showed decreased metabolism in the affected hemisphere. EEGs exhibited asymmetric background rhythms with slow waves in the affected hemisphere. In the two children with seizures, epileptiform discharges from the affected hemisphere were recorded, including one case of sustained partial epilepsy. One child was initially diagnosed with autoimmune encephalitis, whereas two were suspected of having RE at onset. The two children with seizures were treated with immunotherapy and various antiseizure medications. Both underwent hemispherectomy because neither seizures nor limb motor disorders were effectively controlled. Post‐surgery, neither experienced seizures during 2 years of follow‐up, and both showed cognitive and motor improvements. The child without seizures received intermittent steroids and immunoglobulin therapy over 3 years. During 18 months of follow‐up, the patient's motor function improved, and no seizures occurred. Seizures are common initial symptoms of RE. Such cases are often misdiagnosed or missed, leading to delays in optimal treatment. If symptoms are predominantly unilateral and EEG and imaging findings show laterality, the possibility of RE should be considered. Early diagnosis and treatment can reduce unnecessary investigations and improve prognosis. Plain Language Summary Rasmussen's encephalitis (RE) is a rare disease that typically begins with seizures and generally has a poor prognosis. However, over the past 20 years, there have been reports of RE cases where the initial symptoms are not seizures. Our center has diagnosed and treated three such cases in the past 5 years. We aim to provide an overview of these atypical RE patients, focusing on clinical features, electroencephalographic (EEG) findings, and imaging characteristics to inspire early detection and diagnosis of RE, thus improving treatment timing and outcomes for RE patients.

Malformations of cortical development(MCDs)represent one of the most common causes of childhood-onset epilepsy,and are often refractory to antiseizure medications(Guerrini,2006;Guerrini and Dobyns,2014;Severino et al.,2020).Mild malforma-tion of cortical development with oligodendroglial hyperplasia in ep-ilepsy(MOGHE)is a newly recognized subtype of Focal Cortical Dysplasia(FCD),which was officially added to the FCD classifica-tion in 2022 by the International League Against Epilepsy(ILAE)(Najm et al.,2022).

AIMS:This study aims to evaluate the role of stereo-electroencephalography (SEEG) in managing pediatric patients with drug-resistant epilepsy. We further explore prognostic factors influencing surgical outcomes following SEEG-guided resective or disconnective surgery. METHODS:A retrospective review was conducted on pediatric patients who underwent SEEG at the Pediatric Epilepsy Center, Peking University First Hospital, between July 2017 and July 2022. Univariate and multivariate analyses identified key predictors for SEEG-guided surgery. Kaplan-Meier survival analysis was employed to estimate the seizure-free rate, and further statistical tests were applied to evaluate factors associated with seizure outcomes. RESULTS:Among the 148 children included in this study, 102 underwent SEEG-guided resective/disconnective surgery. Multivariate regression identified age at surgery (p < 0.05, 95% CI 0.190-0.997) as an independent predictor for selecting resective/disconnective surgery. The seizure-free rate in patients who underwent SEEG-guided surgery was 69.6%. Multivariate regression confirmed that total resection with lesional MRI (p < 0.05, 95% CI 0.012-0.186) and FCD type II (p < 0.05, 95% CI 0.051-0.851) were strong predictors of seizure freedom. CONCLUSIONS:SEEG plays a crucial role in pediatric epilepsy surgery, particularly in children under 6 years old. Total resection with lesional MRI and FCD type II was the most favorable prognostic predictor for achieving seizure freedom in children undergoing SEEG-guided surgery.

Medical imaging is now a widely used test for the preoperative evaluation of focal cortical dysplasia (FCD). Deep learning-based methods can learn lesion features from image data to automatically recognize and segment FCD in epilepsy treatment. However, the existing FCD segmentation networks lack the ability to fully extract the FCD lesion information and automatically aggregate the salient features of the lesions, the segmentation accuracy needs to be improved. To this end, we propose an end-to-end 3D Convolutional Neural Network segmentation model, Multi Attention Two-Path Residual UNet (MATPR-UNet). Specifically, we propose two modules: (1) Two-Path Residual Attention module, which can extract both local and global information, and suppress invalid information by fully fusing the features in channel and space; (2) Spatially Gated Attention module, which enables the model to automatically focus on the FCD lesion region, highlighting its salient features. We combine the proposed two modules with the 3D UNet to construct the MATPR-UNet. Extensive experiments on the private FCD dataset and the public EPISURG dataset demonstrate that our method outperforms other state-of-the-art methods, and is robust.