AIMS:Supratentorial ependymoma with ZFTA fusion represents a molecularly defined entity with characteristic but heterogeneous histopathological features. In routine diagnostic practice, morphologic diversity and limitations of individual molecular assays may lead to diagnostic uncertainty. This study aimed to systematically characterize the clinicopathological spectrum of pediatric supratentorial ZFTA fusion-positive ependymomas and to highlight potential diagnostic pitfalls. METHODS:Seventeen pediatric cases with confirmed ZFTA fusion were retrospectively analyzed. Clinical, radiological, histopathological, immunohistochemical (including L1CAM, p65/RELA, etc.), and molecular findings (FISH, NGS, DNA methylation profiling), along with follow-up data, were evaluated. RESULTS:The median patient age was 7 years (range, 1-14 years). Non-classical morphologic patterns (embryonal-like, sieve-like, ependymoblastic-like) occurred in 8/17 cases (47.1%), mostly in WHO grade 3 tumors. Crucially, all 8 cases retained focal conventional ependymal features upon thorough sampling and maintained diffuse L1CAM positivity, nuclear p65/RELA, and dot-like EMA positivity in deceptive areas. Molecularly, FISH yielded a 28.6% (4/14) false-negative rate in high-grade cases, and NGS showed 1 false-negative result (7.1%), rescued by DNA methylation profiling. Overall median follow-up was 48 months (range, 6-68). WHO grade 2 tumors had zero recurrence (median follow-up, 60 months), whereas WHO grade 3 tumors showed median time to recurrence of 6 months (range, 3-12). CONCLUSIONS:Pediatric ZFTA fusion-positive ependymomas exhibit morphologic divergence and false-negative molecular risks. Extensive tissue sampling, integrated IHC surrogate panels (p65/L1CAM/GFAP/EMA), and complementary molecular platforms are essential for accurate diagnosis.

BACKGROUND:Surgical intervention has become an established treatment option for epilepsy, but its traditional indications are limited to drug resistant cases. This study compares outcomes of early surgery (predrug resistance) with traditional surgery (postdrug resistance) in focal cortical dysplasia (FCD)-related epilepsy, providing clinical evidence for early surgical intervention. METHODS:Medical records of FCD-related epilepsy children who underwent 1.5 T or 3T brain magnetic resonance imaging at our center (Jan 2008-Dec 2022) were reviewed. Children were divided into early surgery and traditional surgery groups based on treatment pathway. Postoperative seizure outcomes, antiseizure medication (ASM) outcomes, seizure duration, and ASM usage were compared. RESULTS:Of the 195 children with magnetic resonance imaging-confirmed FCD who met the inclusion criteria, 167 (85.6%) were diagnosed with FCD-related epilepsy. Median follow-up duration was 61 months. Twenty-eight (16.7%) achieved seizure freedom with the first ASM, while 5.6% and 5.4% achieved seizure freedom with the second and third or more ASMs, respectively. Ninety-nine children received surgical treatment, with 45 undergoing early surgery and 54 receiving traditional surgery. At the last follow-up, significantly more children in the early surgery group (88.9%) achieved seizure freedom compared to the traditional surgery group (74.1%) (P = 0.033). CONCLUSIONS:Early surgical evaluation can help identify candidates who may benefit from early surgery while maintaining comparable perioperative risks to traditional surgery. Therefore, it is necessary to refine the timing and criteria for epilepsy surgery evaluation. For patients with noneloquent cortical brain lesions, early surgery is recommended to reduce the duration of living with seizure.

BackgroundDifferentiating sellar region germ cell tumors (GCTs) from Langerhans cell histiocytosis (LCH) is challenging due to highly similar MRI features, especially in tumor marker-negative patients. In this study, we aimed to develop and validate a radiomics model to distinguish tumor marker-negative sellar GCTs from LCH.MethodsThis retrospective study enrolled a total of 93 patients diagnosed pathologically or by therapeutic diagnosis at our single institution between April 2012 and April 2024, including 40 cases of LCH and 53 cases of GCTs. Radiomics features extracted from multiparametric MRI, including T1-weighted imaging (T1WI) and T2-weighted imaging (T2WI). We manually segmented the regions of interests (ROIs) of tumors. Feature selection was subsequently performed using LASSO regression with five-fold cross-validation. We have chosen three machine learning classifiers-Support Vector Machine (SVM), Random Forest (RF), and Logistic Regression (LR) to construct models based on the 7 features which were retained. Additionally, by integrating clinically significant features and imaging semantic features, classification models based on radiomics features, imaging semantic features, and clinical features were developed separately. There are 7 models in total. Furthermore, combined prediction models were constructed based on different fusion feature sets, respectively. The performance of the diagnostic model was evaluated using the receiver operating characteristic (ROC) curve. The mean area under the curve (AUC), sensitivity, specificity, accuracy, and F1-score were calculated for both the development set and the test set. Differences in AUC between models were assessed using DeLong's test, and the resulting P-values were adjusted using the Bonferroni false discovery rate (FDR) correction method. Code available upon request.ResultsThe best diagnostic performance was achieved by the combined model of radiomics with clinical features and imaging semantic features using the RF classifier, with an AUC value of 0.81. A statistically significant difference (p < 0.05) was confirmed by the DeLong test, indicating robust diagnostic capability.ConclusionRadiomics-based machine learning is a promising, non-invasive approach to distinguish tumor marker-negative sellar GCTs from LCH, which has good predictive performance and may help with treatment decision-making.

Background: There are no apparent distinctions in clinical presentation or conventional imaging findings between brainstem gliomas and embryonal tumors occurring in the brainstem. Our aim was to study the role of diffusion tensor imaging in differentiating embryonal tumors from gliomas of the brainstem. Methods: Three cases of embryonal tumors occurring in the brainstem and 19 cases of brainstem gliomas were analyzed retrospectively. Result: The most common brainstem gliomas are diffuse intrinsic pontine gliomas. On the fiber tracking images, brainstem gliomas were associated with relatively intact projection fibers that continuously traversed the tumor and followed the trajectory of normal neural fibers, whereas embryonal tumors were associated with disruption of projection fibers. The close cellularity created tissues with significant directional properties in embryonal tumors, restricting the diffusion of water molecules. As a result, there were areas of high anisotropy within the embryonal tumors. Additionally, we observed that the apparent diffusion coefficient value of embryonal tumors occurring in the brainstem was lower than that of brainstem gliomas and the difference was statistically significant (P < 0.05). Conclusion: Disruption of projection fibers within the tumor on diffusion tensor imaging may help differentiate embryonal pathology from glial. (c) 2024 Elsevier Inc. All rights are reserved, including those for text and data mining, AI training, and similar technologies.

OBJECTIVE:To investigate the association of treatment approaches on the clinical outcomes and health-related quality of life (HRQL) of pediatric intracranial aneurysms (PIAs) at our institution. METHODS:A retrospective analysis of 37 children with PIAs treated from 2015 to 2022. Clinical outcomes were evaluated using the modified Rankin score and Lovett scale. Additionally, the HRQL was assessed using the Pediatric Quality of Life Inventory 4.0 questionnaire. RESULTS:Thirty-seven children were admitted with 41 aneurysms. Of these, 15 patients underwent surgical treatment, 16 received endovascular therapy, and 6 were managed conservatively. After an average follow-up period of 2.8 years, the median modified Rankin score was 0 for endovascular therapy against 1.5 for surgical treatment (P = 0.443). Similarly, limb muscle strength was higher in the endovascular group (5 vs. 4, P = 0.433). Pediatric Quality of Life Inventory 4.0 assessments demonstrated excellent reliability (α = 0.907), with no significant difference between the endovascular and surgical groups in overall HRQL (P = 0.390, 95% confidence interval: -6.892 to 16.978). However, the endovascular group showed a trend toward improved quality of life, including physical, emotional, and social functioning. CONCLUSIONS:Active treatments yield favorable outcomes for children with PIAs. Our study is the first to demonstrate that endovascular approach may offer a beneficial long-term improvement in HRQL for children with PIAs.

OBJECTIVE:This study aims to investigate the alterations in structural and functional connectivity networks (SCN and FCN) in children with hypothalamic syndrome (HS) following craniopharyngioma resection and to explore the relationship between these network changes and clinical manifestations. MATERIALS AND METHODS:We performed graph theory analysis on SCN and FCN derived from 36 patients with HS and 36 age- and sex-matched healthy controls (HC), with an age range of 6 to 13 years. We evaluated characteristics, nodal properties, and the coupling between SCN and FCN across 90 brain nodes. Partial correlation analyses examined relationships between graph theory properties and clinical scales, including the Wechsler Intelligence Scale for Children (WISC), the Wechsler Memory Scale (WMS), and the Attention Deficit Hyperactivity Disorder (ADHD) scale. RESULTS:The SCN in the HS group exhibited abnormal global properties, including increased characteristic path length (Lp), decreased global efficiency (Eg), and local efficiency (ELOC), alongside notable reductions in nodal properties, such as degree centrality (Dc) and nodal efficiency (Ne) across multiple nodes. The FCN in the HS group also displayed abnormal global attributes, with elevated Lp and reduced Eg, alongside decreased Dc at the median cingulate and paracingulate gyri (DCG.L) node. However, no statistically significant differences were found in structural-functional connectivity (SC-FC) coupling between groups. Correlation analysis revealed significant links between WISC, WMS, and ADHD scales and various graph-theoretic properties in the HS group. CONCLUSION:In patients with HS following craniopharyngioma resection, alterations in SCN and FCN characteristics have been observed. These neural changes are associated with cognitive developmental impairments related to HS, providing neuroimaging evidence elucidating the mechanisms underlying cognitive deficits in HS patients.

Corynoline is a bioactive compound extracted from Corydalis bungeana Turcz, which has shown significant potential in mitigating inflammatory responses and treating tumours. However, to the best of our knowledge, whether corynoline has anti-glioma activity has not been reported. In the present study, the activity of corynoline against glioblastoma (GBM) was evaluated, and its underlying mechanisms was explored. Corynoline was found to significantly inhibit the proliferation and promote the apoptosis of GBM cell lines U87 and LN229. Mechanistically, corynoline decreased the level of STAT3 phosphorylation, downregulated the expression of the anti-apoptotic protein Bcl2, and upregulated the expression of the pro-apoptotic proteins Bax, Bak, cleaved caspase-9, and cleaved caspase-3. Importantly, the STAT3 activator colivelin TFA markedly attenuated the effects of corynoline on GBM cells and inhibited the STAT3/Bcl2 signalling axis. Furthermore, corynoline treatment significantly inhibited the growth of GBM in vivo and was shown to modulate the STAT3/Bcl-2 signalling pathway. These results suggest that corynoline exerts antitumour activity against GBM by inhibiting the STAT3/Bcl2 pathway, indicating that corynoline might be a promising agent for the treatment of GBM.

BACKGROUND:Suprasellar hypothalamic-opticochiasmatic glioma (HOCG) and craniopharyngioma (CP) have similar appearances on conventional magnetic resonance imaging (MRI) and are difficult to distinguish. Moreover, these tumors are situated near vital structures like the optic chiasm and hypothalamus, rendering conventional surgery susceptible to significant complications. We mainly discussed the surgical application value and diagnostic value of diffusion tensor imaging (DTI) in HOCG and CP. METHODS:The retrospective analysis of 13 cases of HOCG and 16 cases of CP was conducted. All patients underwent conventional MRI and DTI prior to surgery, and were pathologically diagnosed postoperatively. RESULTS:Both CP and HOCG appeared as heterogeneous mixed signal masses on conventional MRI. For HOCGs, fiber tractography revealed 2 different growth patterns of the tumor: infiltrative type and inflated type. The surgical approach and risk levels differ between these growth patterns. Additionally, fiber tractography demonstrates significant differences compared to CPs. The surgical approach and extent of resection for all cases of these 2 tumors were guided by DTI. CONCLUSIONS:DTI enhances the accuracy of HOCG and CP differentiation. Furthermore, patterns of tractography described in this study assist neurosurgeons in delineating the surgical pathway and tumor resection range without damaging important fiber bundles, thereby avoiding permanent neurological deficits and improving survival quality for patients.

Background:Among germ cell tumours, germinomas are extremely sensitive to radiotherapy and chemotherapy. Histological diagnosis is important for clinical treatment decisions. This study aimed to identify germinomas and non-germinomatous germ cell tumours (NGGCTs) using radiomics-based machine learning (ML). Methods:The present retrospective study comprised 141 patients diagnosed with intracranial germ cell tumours (ICGCTs), 71 germinomas, and 70 NGGCTs. Radiomics features were quantitatively extracted from magnetic resonance imaging (MRI) sequences, including T1-weighted imaging (T1WI), T2-weighted imaging (T2WI), T2 Fluid-Attenuated Inversion Recovery (T2-FLAIR), diffusion weighted imaging (DWI) (b=1,000), apparent diffusion coefficient (ADC) images, and contrast-enhanced T1WI. Based on the combination of three feature selection methods and three classification methods, the optimal model was screened out from the internal test set. A combined model of clinical-multi-sequence radiomics was ultimately created by combining with statistically significant clinical features. The performance of the models was evaluated using the area under the curve (AUC), accuracy, sensitivity, specificity, and F1-score. Results:The combination of the least absolute shrinkage and selection operator (LASSO) and logistic regression (LR) yielded the optimal diagnostic performance in the multi-sequence radiomics model, as evidenced by an AUC value of 0.823 in the internal and 0.804 in the external test set. In the combined model, the AUC values of the internal and external tests were 0.838 and 0.809, respectively. The DeLong test revealed no significant difference between multi-sequence radiomics and the combined model, indicating that the inclusion of clinical characteristics did not significantly improve diagnostic accuracy. Conclusions:ML based on radiomics may provide a non-invasive approach for the clinical differentiation of intracranial germinomas and NGGCTs.

Objective People with epilepsy desire to acquire accurate information about epilepsy and actively engage in its management throughout the long journey of living with seizures. ChatGPT is a large language model and we aimed to assess the accuracy and consistency of ChatGPT in responding to the common concerns of people with epilepsy and to evaluate its ability to provide emotional support. Methods Questions were collected from the International League against Epilepsy and the China Association against Epilepsy. The responses were independently assessed by two board-certified epileptologists from the China Association against Epilepsy, and a third reviewer resolved disagreements. The reviewers assessed its ability to provide emotional support subjectively. Results A total of 378 questions related to epilepsy and 5 questions related to emotional support were included. ChatGPT provided "correct and comprehensive" answers to 68.4% of the questions. The model provided reproducible answers for 82.3% questions. The model performed poorly in answering prognostic questions, with only 46.8% of the answers rated as comprehensive. When faced with questions requiring emotional support, the model can generate natural and understandable responses. Significance ChatGPT provides accurate and reliable answers to patients with epilepsy and is a valuable source of information. It also provides partial emotional support, potentially assisting those experiencing emotional distress. However, ChatGPT may provide incorrect responses, leading users to inadvertently accept incorrect and potentially dangerous advice. Therefore, the direct use of ChatGPT for medical guidance is not recommended and its primary use at present is in patients education.

Infectious intracranial aneurysm (IIA) and embolic cerebral infarction are well-known devastating complications of children suffering infective endocarditis. In this report, we describe a successfully embolized IIA concurrent with bilateral middle cerebral artery (MCA) occlusion. Unfortunately, a newly formed IIA located in the contralateral MCA bifurcation ruptured at the seventh day following embolization. A 6-month-old female child was admitted to hospital 3 days following acute right limb mobility disorder. An interventional surgery history of congenital heart disease was confirmed. She was immediately started on antibiotic therapy and the computed tomography agiography (CTA) scan showed occlusion of the upper branch of the left MCA. Unfortunately an IIA was located in the distal artery region (DAR) of the ipsilateral anterior cerebral artery. Angiography (digital subtraction angiography) was performed and the DAR IIA was embolized by OnyX-18 with Magic 1.2 Fr. microcatheter. On the sixth day, magnetic resonance imaging during the hospital stay showed reduced infarction area with no other special sign. Desperately, a major seizure with opisthotonos attacked the baby on the seventh day after embolization. An immediate CTA scan showed massive hematoma in the right basal ganglia and a ruptured bifurcate aneurysm of the right MCA. The parents refused positive treatment and discharged in considering the critical situation. It should be noted that IIA can be fast formed anywhere in cerebral artery and dynamic angio-image should be performed as supervision.

Background Observational studies have indicated that psychiatric disorders are the most common comorbidities in pediatric epilepsy. However, the existence and direction of a causal relationship between the two remains controversial. This study aims to investigate the association between common childhood psychiatric disorders and epilepsy using a two-sample, bidirectional Mendelian randomization (MR) approach. Methods Genetic instruments were obtained from the most recent and largest genome-wide association studies (GWAS), including datasets for epilepsy ( N _case = 29,994, N _control = 52,538), attention deficit hyperactivity disorder (ADHD) ( N _case = 38,691, N _control = 186,843), autism spectrum disorder (ASD) ( N _case = 18,381, N _control = 27,969), and Tourette syndrome (TS) ( N _case = 4,819, N _control = 9488). MR analyses were conducted using the inverse variance weighted (IVW) method, weighted median method, and MR-Egger regression. Results No reliable evidence was found to suggest a causal effect of ADHD, ASD, or TS on epilepsy, nor was there any reliable evidence indicating that epilepsy increases the risk of these three psychiatric disorders. These findings remained consistent across various sensitivity analyses. Conclusion Although observational studies have highlighted a high comorbidity rate between pediatric epilepsy and psychiatric disorders like ADHD and ASD, the MR analysis did not confirm a causal relationship between them. This suggests that previous studies might have been influenced by confounding biases or other biases, potentially overestimating the true relationship. A deeper understanding of the mechanisms underlying these comorbidities is crucial for refining the treatment of pediatric epilepsy.

Objective The study aimed to explore the association between the site of interictal epileptic discharges (IEDs) on postoperative electroencephalogram (EEG) and seizure recurrence after antiepileptic drug (AED) withdrawal. The study hypothesizes that the concordance of IED sites with surgical sites indicates incomplete resection of epileptic focus, while non-concordance of IED sites with surgical sites indicates postoperative changes or cortical stimulation. The former has a higher risk of seizure recurrence.Methods We retrospectively analyzed the postoperative EEG pattern of 182 consecutive children who underwent resection surgery. To identify the risk factors for seizure recurrence, we compared the attributes of seizure recurred and seizure-free groups by univariate and multivariate analyses. AED tapering was standardized, involving a 25% reduction in the dose of a single type of AED every 2 weeks, independent of the presurgical AED load.Results We attempted AED withdrawal in 116 (63.7%) children. Twenty-eight (24.1%) children experienced seizure recurrence during or after AED withdrawal. A greater number of AEDs used at the time of surgery (p=0.005), incomplete resection (p=0.001), and presence of IED on postoperative EEG (p=0.011) are predictors of seizure recurrence. The completeness of resection and seizure recurrence after AED withdrawal were related to the presence of IED on the EEG, but not to the concordance of IED with surgical sites.Conclusion For children with abnormal EEG, the decision to discontinue AED should be made more cautiously, regardless of the relative location of the discharge site and the surgical site.

BACKGROUND Intracranial pial arteriovenous fistulas (PAVFs) are uncommon neurovascular anomalies that primarily affect the pediatric population. PAVFs are often linked to hereditary hemorrhagic telangiectasia, yet the specific genetic mutations remain unidentified. While endovascular embolization is the preferred treatment for PAVFs, complications like hydrocephalus and sinus thrombosis pose challenges in management. OBSERVATIONS The authors present a rare case of PAVF in a 6-month-old male neonate with a hereditary GDF2 mutation, where the fistula was supplied by the posterior inferior cerebellar artery and drained directly into the sigmoid sinus. The PAVF was effectively treated with endovascular embolization using coils and Onyx. Furthermore, the authors describe the successful use of rivaroxaban in managing subsequent sinus thrombosis after the embolization of PAVFs. Additionally, the authors review treatment strategies and complications following fistula disconnection. LESSONS Endovascular embolization is the primary treatment choice for the majority of pediatric PAVFs, while a hereditary GDF2 mutation is considered a potential contributing factor to the formation of these malformations in children. Rivaroxaban has shown promise as an effective therapeutic option for pediatric sinus thrombosis, supported by its established safety profile. https://thejns.org/doi/10.3171/CASE24182

The crucial role of ribonucleotide reductase M2 (RRM2) enzyme in cancer occurrence and progression has been well-established, but its specific function and significance in medulloblastoma (MB) remains largely unknown. First, we conducted a bioinformatics analysis of public genomic databases and observed highly expressed RRM2 in MB and an association of high RRM2 expression with adverse outcomes. In addition, by collecting clinical MB specimens for polymerase chain reaction (PCR), western blotting (WB), and immunohistochemistry (IHC), RRM2 was confirmed to be highly expressed in tumor tissues. Furthermore, immunohistochemical analysis linked adverse prognosis to high RRM2 expression. Moreover, knocking down RRM2 significantly inhibited MB cell proliferation, migration, and invasion in vitro. This report is the first to demonstrate the oncogenic role of RRM2 in MB, associated with adverse patient outcomes. Knocking down RRM2 contributes to weakened proliferating, migrating, and invading potentials of MB cells. RRM2 is expected to be a novel prognostic biomarker and therapeutic target for MB.

IntroductionMolecular subgroups influence the vascular architecture within medulloblastomas, particularly the wingless (WNT) subgroup, which contributes to its propensity for primary tumor hemorrhage. Whether this mechanism affects intraoperative blood loss remains unknown. This study aimed to assess the association between WNT medulloblastoma and the predisposition for blood loss.MethodsThis was a retrospective observational study using data from a neuro-oncology center comprising molecular data on patients treated between December 31, 2014, and April 30, 2023. Differences between WNT and other subgroups in the risk of primary outcome-intraoperative blood loss were assessed using multivariable-adjusted linear regression.ResultsOf the 148 patients included in the analysis, 18 patients (12.2%) had WNT, 42 (28.4%) had sonic hedgehog (SHH) TP53-wildtype, 7 (4.7%) had SHH TP53-mutant, and 81 (54.7%) were non-WNT/ non-SHH. The WNT subgroup more frequently underwent primary intratumoral hemorrhage (22% vs. 3.8%; p = 0.011). The median intraoperative blood loss was 400.00 (interquartile range [IQR] 250, 500) mL for WNT and 300.00 [200, 400] mL for the other subgroups (p = 0.136), with an adjusted β of 135.264 (95% confidence intervals [CI], 11.701–258.827; p = 0.032). Similar results were observed in both midline and noninfiltrative margin medulloblastoma.DiscussionWNT medulloblastoma is typically associated with primary intratumoral hemorrhage and intraoperative blood loss. The validity of determining the surgical approach based on predicted molecular subtypes from imaging data is questionable. However, attempting to engage in risk communication with patients in a molecular-specific way is worthwhile to validate.

BACKGROUND:Neonatal posthemorrhagic hydrocephalus remains a common complication in preterm infants, with high rates of mortality and morbidity, placing parents at high risk of anxiety and depression. We sought to investigate the neurodevelopmental outcomes of infants with posthemorrhagic hydrocephalus who underwent surgery and the psychological effect on their parents.METHODS:We retrospectively analysed all infants with posthemorrhagic hydrocephalus born between 2014 and 2020 in the Children's Hospital of Chongqing Medical University, China. The neurodevelopmental outcomes of 28 patients were evaluated by the Pediatric Stroke Outcome Measure score, and the psychological states of the parents of survivors were assessed by the Hospital Anxiety and Depression Scale.RESULTS:The families of the 28 patients were followed up for a median duration of 3 years; 6 (21.4%) patients died within 6 months after discharge, 12 (42.9%) patients had moderate to severe dysfunction, and only 10 (35.7%) patients had good outcomes. Regarding the 22 parents of the survivors, 5 (22.7%) and 4 (18.2%) had borderline anxiety and depression symptoms, respectively. Two (9.1%) caregivers had exact anxiety and depression symptoms. Leukomalacia after intraventricular haemorrhage was associated with adverse neurological outcomes. The infants' histories of epileptic seizures during the neonatal period were associated with the anxiety of their parents.CONCLUSION:The overall outcome of posthemorrhagic hydrocephalus patients is unsatisfactory, and children with leukomalacia after haemorrhage tend to have poor outcomes. A history of epileptic seizures during the course of the disease may exacerbate the anxiety of the caregivers.

目的 探讨可调压抗虹吸分流装置在颅内生殖细胞肿瘤(IGCTs)所致脑积水治疗中的意义及远期移除分流装置的可行性.方法 重庆医科大学附属儿童医院神经外科2013年1月—2017年12月间42例拟诊为IGCTs伴梗阻性脑积水患儿,均采用可调压抗虹吸分流装置行脑室腹腔分流术(VPS),术后予以行诊断性化疗;回顾性分析其中20例在肿瘤达到完全缓解后行分流装置移除术的诊疗过程.结果 行VPS术后,20例患儿的高颅压症状均获得显著缓解,无症状加重者.术后复查CT或MRI显示脑室系统正常;眼底检查未见明显损害情况,术前视力模糊者术后视力逐渐恢复正常,现视力与同龄人相仿.治疗过程中未见分流装置堵塞、感染病例,予以诊断性化疗,肿瘤获得完全缓解,6～12个月后行分流装置夹闭或移除术,随访36～92个月,20例患者均未出现颅内高压症状.至随访结束时均已入学,生活质量未见明显影响.结论 IGCTs所致梗阻性脑积水行VPS早期可以快速降低颅压,缓解颅内高压症状,降低颅内压持续升高而发生脑疝的风险.术后经诊断性化疗,在肿瘤获得完全缓解后,可行分流装置移除术,降低分流装置堵塞、感染、断裂等风险,避免患儿终生留置体内分流装置,可有效提高患儿生活质量.

Background Artery of Percheron (AOP) as main feeder artery of arteriovenous malformation (AVM) is extremely rare. Two cases of thalamic AVM fed by AOP have been reported to date and only one AVM been removed by microsurgery when attempt of intervention embolization failed. Midbrain AVM fed by AOP has not been reported yet. Case presentation Here, we presented the first successful embolized case of midbrain AVM supplied by the AOP in a 10-year-old boy, who suffered dual oculomotor nerve palsy and secondary hemorrhage before embolization. During endovascular embolization, selective angiography by 1.2 Fr. Magic microcatheter showed an intranidal aneurysm located on the distal AOP. Two injections of a 1:4 ratio mixture of NBCA-MS completely occlude the nidus and intranidal aneurysm with no complications occurred. The child recovered well and the oculomotor deficits improved. Conclusion This case highlighted that AOP is a clinically significant branch associated with AVM in midbrain and thalamus. Moreover, intervention embolization of midbrain AVM fed by AOP is a considerable therapeutic strategy.

AIM:To construct a prediction nomogram model for the postoperative seizure outcomes in children with focal cortical dysplasia (FCD). MATERIAL AND METHODS:We retrospectively reviewed the clinical data of 97 children with epilepsy secondary to FCD who had undergone resection surgery at Children's Hospital of Chongqing Medical University from June 2013 to September 2019. Univariate and multivariate Cox proportional hazards regression were used to explore the predictors of postoperative persistent seizure, and a nomogram prediction model for postoperative seizure outcome was developed. The C-index was chosen to evaluate the discriminability of the nomogram with internal validation. Calibration curves and decision curve analysis were used to evaluate consistency and clinical efficacy, respectively. RESULTS:The complete resection of epileptogenic focus and the pathological type of FCD were independent predictors of persistent seizure in children with epilepsy secondary to FCD after surgery. Based on multivariate Cox proportional hazard regression, a predictive nomogram for epilepsy outcome was established and validated via the bootstrap method with 1000 resamples. The nomogram showed superior prediction accuracy (C-index = 0.883); by drawing and reviewing the calibration curve and decision curve, the nomogram presented good consistency and clinical efficacy. CONCLUSION:A nomogram prediction model of postsurgery seizure outcome in children with epilepsy secondary to FCD was constructed based on four variables, providing a reliable and convenient tool for individual seizure outcome prediction.