A Retrospective Analysis of the Therapeutic Outcomes of 117 Neuroblastoma Patients Treated at a Single Pediatric Oncology Center in China.

OBJECTIVE: Recent therapeutic advances have greatly enhanced the survival rates of patients with neuroblastoma (NB). However, the outcomes of neuroblastoma patients in China, particularly those with high-risk (HR) NB, remain limited. METHOD: We retrospectively analyzed the clinical data and outcomes of NB patients who were treated at a tertiary pediatric cancer facility in China between January 2013 and October 2021. RESULTS: A total of 117 NB patients were recruited. Patients with very low-risk (VLR), low-risk (LR), intermediate-risk (IR), and HR-NB patients made up 4%, 27%, 15%, and 54% of total patient population, respectively. Patients diagnosed between 2013 and 2018 were treated according to the protocol of Sun Yat-Sen University Cancer Center and those diagnosed between 2019 and 2021 were treated according to the COG ANBL0531 or ANBL0532 protocol with or without autologous stem cell transplantation (ASCT). The 5-year EFS and OS of all risk groups of patients were 67.29% and 77.90%, respectively. EFS and OS were significantly decreased in patients with higher risk classifications (EFS: VLR/LR vs IR vs HR: 97.22% vs 67.28% vs 51.83%; ***P = .001; OS: VLR/LR vs IR vs HR: 97.06% vs 94.12% vs 64.38%; *P = .046). In HR-NB patients treated according to the COG protocol between 2019 and 2021, the 3-year OS of patients who received tandem ASCT was significantly greater than those who did not receive ASCT (93.33% % vs 47.41%; *P = .046; log-rank test). EFS was not significantly different between patients with and without ASCT (72.16% vs 60.32%). CONCLUSION: Our findings show that patients with lower risk classification have a positive prognosis for survival. The prognosis of patients with HR-NB remains in need of improvement. ASCT may enhance OS in HR-NB patients; however, protocol adjustment may be necessary to increase EFS in these patients.

Long-term follow-up in a Chinese child with congenital lipoid adrenal hyperplasia due to a StAR gene mutation.

BACKGROUND: Congenital lipoid adrenal hyperplasia (CLAH) is an extremely rare and the most severe form of congenital adrenal hyperplasia. Typical features include disorder of sex development, early-onset adrenal crisis and enlarged adrenal glands with fatty accumulation. CASE PRESENTATION: We report a case of CLAH caused by mutations in the steroidogenic acute regulatory protein (StAR) gene. The patient had typical early-onset adrenal crisis at 2 months of age. She had normal-appearing female genitalia and a karyotype of 46, XY. The serum cortisol and adrenal steroids levels were always nearly undetectable, but the adrenocorticotropic hormone levels were extremely high. Genetic analysis revealed compound heterozygous mutations at c. 229C > T (p.Q77X) in exon 3 and c. 722C > T (p.Q258X) in exon 7 of the StAR gene. The former mutation was previously detected in only two other Chinese CLAH patients. Both mutations cause truncation of the StAR protein. The case reported here appears to be a classic example of CLAH with very small adrenal glands and is the second reported CLAH case with small adrenal glands thus far. In a 15-year follow-up, the patient's height was approximately average for females before age 4 and fell to - 1 SDS at 10 years of age. Her bone age was similar to her chronological age from age 4 to age 15 years. CONCLUSIONS: In conclusion, this is a classic case of CLAH with exceptionally small adrenal glands. Q77X mutation seems to be more common in Chinese CLAH patients. Additionally, this is the first report of the growth pattern associated with CLAH after a 15-year follow-up.

MRI signal intensity differentiation of brainstem encephalitis induced by Enterovirus 71: a classification approach for acute and convalescence stages.

BACKGROUND: The objective of this study is to assess standardized histograms of signal intensities of T1 signal and T2 signal on sagittal view without enhancement during (1) acute stage, and (2) convalescence stage of pediatric patients with Enterovirus 71 related brainstem encephalitis (BE), and with respect to (3) healthy normal. METHODS: Our subjects were hospitalized between March 2010 and October 2012, and underwent pre- and post-contrast MRI studies. The research question to be answered is whether the comparison of the MRI image intensity histograms and relevant statistical quantification can add new knowledge to the diagnosis of BE patients. So, both 25 cases in acute stage with prolonged T1 and T2 signal, without enhancement, and 13 cases in convalescence stage were introduced. In additional, a healthy group with 25 cases was recruited for comparison. RESULTS: MRI signal intensity histogram changes of the lesions were compared at the acute and convalescence stages of the disease. Our preliminary results suggest that standardized histograms of signal intensities and their statistical properties are able to provide diagnostic information for the clinical assessment of the disease. Different stages pertaining to the histogram plots comparison showed that overall T1 signal intensity values increase as we traverse from the acute stage to the convalescence stage. And then for the healthy subjects, the T2 signal intensity values changed their magnitudes in a reverse direction. However, exceptions of this can happen in four cases where the primary lesions occurred in the brainstem that developed encephalomalacia resulting in a lower signal in T1WI and higher signal in T2WI. Statistical analysis revealed there was significant difference of T1 signal intensity among the three groups; and also, the T2 signal intensity was lower than other two groups. CONCLUSIONS: Standardized histogram of T1 and T2 intensity provide valuable and useful information for disease diagnosis and evaluation, which can potentially help medical doctors to save the lives of children.

Association of Glymphatic and White Matter Impairment With the Postoperative Outcome of Pediatric Hydrocephalus.

BACKGROUND AND OBJECTIVES: Assessment of postoperative outcomes on pediatric hydrocephalus is critical for adjusting treatment strategies. The aim of this work was to investigate the ability of MRI metrics to predict postoperative outcomes. METHODS: A total of 55 children with hydrocephalus who underwent MRI and ventriculoperitoneal shunt surgery were prospectively enrolled. MRI was also performed at 6 months postoperatively in 33 of the 55 children. A total of 92 controls matched for age and sex were enrolled and divided into preoperative and postoperative control groups. We calculated the diffusion tensor imaging along the perivascular space (DTI-ALPS) index, Evans index, and diffusion tensor imaging metrics. The ability of various metrics to predict postoperative outcomes was assessed using receiver operating characteristic curve analysis. RESULTS: The DTI-ALPS index was significantly lower in patients with hydrocephalus than in controls. The abnormal DTI-ALPS index trended toward the normal range after surgery. Patients with lower preoperative DTI-ALPS index, lower fractional anisotropy (FA), and higher radial diffusivity in association fibers had less favorable short-term outcomes. Patients with worse long-term outcomes had lower postoperative DTI-ALPS index, higher postoperative Evans index, and lower FA and higher radial diffusivity in association fibers. Predictive performance was better when the DTI-ALPS index and FA in association fibers were used in combination than when either of these metrics was used alone. CONCLUSION: The DTI-ALPS index and FA in association fibers provided complementary information for prognostic assessment after the ventriculoperitoneal shunt surgery on pediatric hydrocephalus. A combination of DTI-ALPS index and FA would improve our ability to predict postoperative outcomes in these patients.

Clinical and MRI features of neurological complications after influenza A (H1N1) infection in critically ill children.

BACKGROUND: Influenza A (H1N1) can cause severe neurological complications. OBJECTIVE: The purpose of this study was to analyze clinical and MRI features of neurological complications after H1N1 infection in critically ill children. MATERIALS AND METHODS: We retrospectively analyzed clinical and neuroimaging findings in 17 children who were hospitalized in an intensive care unit with severe neurological complications after H1N1 infection in South China between September 2009 and December 2011. All children underwent pre- and post-contrast-enhanced brain MRI. Postmortem studies were performed in two children. RESULTS: Six children died, five because of acute necrotizing encephalopathy (ANE) and one because of intracranial fungal infection. Eleven recovered; their manifestations of H1N1 were meningitis (3), encephalitis (1) and influenza encephalopathy (7). MRI features of ANE included multiple symmetrical brain lesions demonstrating prolonged T1 and T2 signal in the thalami, internal capsule, lenticular nucleus and pontine tegmentum. Postmortem MRI in two children with acute necrotizing encephalopathy showed diffuse prolonged T1 and T2 signal in the bilateral thalami, brainstem deformation and tonsillar herniation. CONCLUSION: Fatal neurological complications in children after H1N1 infection include ANE and opportunistic fungal infection. MRI is essential for identification of neurological complications and for clinical evaluation.

MRI and associated clinical characteristics of EV71-induced brainstem encephalitis in children with hand-foot-mouth disease.

INTRODUCTION: This study was conducted to investigate MRI and associated clinical characteristics of brainstem encephalitis induced by enterovirus 71 (EV71) in children with hand-foot-mouth disease (HFMD). METHODS: We analyzed clinical and imaging data from 42 HFMD cases with EV71-induced brainstem encephalitis. All patients underwent plain and enhanced MRI cranial scans and were placed into one of two groups according to MRI enhancement results, an enhanced group or a nonenhanced group. RESULTS: Thirty-two cases were positive on MRI exam. The primary location of the lesion for brainstem encephalitis was the dorsal pons and medulla oblongata (32 cases), followed by the cerebellar dentate nucleus (8 cases), midbrain (5 cases), and thalamus (2 cases). Plain T1-weighted images showed isointense or hypointense signals, and T2-weighted images showed isointense and hyperintense signals. Enhanced MRI scans showed that 12 cases had slight to moderate enhancement; 4 of these were normal on plain scan. The time from MRI examination to disease onset was statistically different between the enhanced (n = 12) and nonenhanced (n = 21) groups with a mean of 7.67 days (SD = 1.07) vs 11.95 days (SD = 5.33), respectively. The most common neurological symptoms for brainstem encephalitis were myoclonus and tremor. The greater the area of affected brain, the more severe the clinical symptoms were. CONCLUSION: The locations of EV71-induced HFMD-associated brainstem encephalitis lesions are relatively specific. Enhanced MRI scans could also identify the lesions missed by early plain scans. MRI scans can provide important information for clinical evaluation and treatment.

Whole-Body MRI Is an Effective Imaging Modality for Hematological Malignancy Treatment Response Assessment: A Systematic Review and Meta-Analysis.

OBJECTIVES: To evaluate the diagnostic accuracy of whole-body MRI (WB-MRI) for assessment of hematological malignancies' therapeutic response. METHODS: PubMed, Embase, and Web of Science were searched up to August 2021 to identify studies reporting the diagnostic performance of WB-MRI for the assessment of hematological malignancies' treatment response. A bivariate random-effects model was applied for the generation of the pooled diagnostic performance. RESULTS: Fourteen studies with 457 patients with lymphoma, multiple myeloma, and sarcoma (very small proportion) were analyzed. Overall pooled sensitivity and specificity of WB-MRI were 0.88 (95% CI: 0.73-0.95) and 0.86 (95% CI: 0.73-0.93), respectively. Studies using whole-body diffusion-weighted imaging (WB-DWI) showed higher sensitivity than those that did not (0.94 vs. 0.55, p = 0.02). The pooled concordance rate of WB-MRI to assess hematological malignancies' treatment response with reference standard was 0.78 (95% CI: 0.59-0.96). WB-MRI and PET/CT showed similar diagnostic performance (sensitivity [0.83 vs. 0.92, p = 0.11] and specificity [0.87 vs. 0.76, p = 0.73]). CONCLUSION: WB-MRI has high diagnostic performance for hematological malignancies' treatment response assessment. The adding of WB-DWI is strongly associated with increased sensitivity.

Role of SNAP-25 MnlI variant in impaired working memory and brain functions in attention deficit/hyperactivity disorder.

INTRODUCTION: Attention deficit/hyperactivity disorder (ADHD) is a hereditary neurodevelopmental disorder characterized by working memory (WM) deficits. The MnlI variant (rs3746544) of the synaptosomal-associated protein 25 (SNAP-25) gene is associated with ADHD. In this study, we investigated the role and underlying mechanism of SNAP-25 MnlI variant in cognitive impairment and brain functions in boys with ADHD. METHOD: We performed WM capacity tests using the fourth version of the Wechsler Intelligence Scale for Children (WISC-IV) and regional homogeneity (ReHo) analysis for the resting-state functional magnetic resonance imaging data of 56 boys with ADHD divided into two genotypic groups (TT homozygotes and G-allele carriers). Next, Spearman's rank correlation analysis between the obtained ReHo values and the WM index (WMI) calculated for each participant. RESULTS: Compared with G-allele carrier group, there were higher ReHo values for the left medial prefrontal cortex (mPFC) and higher WM capacity in TT homozygote group. Contrary to TT homozygote group, the WM capacity was negatively correlated with the peak ReHo value for the left mPFC in G-allele carrier group. CONCLUSION: These findings suggest that SNAP-25 MnlI variant may underlie cognitive and brain function impairments in boys with ADHD, thus suggesting its potential as a new target for ADHD treatment.

Functional Connectivity Underlying Symptoms in Preschool Boys With Autism: A Resting-State Functional Magnetic Resonance Imaging Study.

Background: Single-sex children have been regarded as one of the best subjects to understand the abnormal development patterns of autism spectrum disorders (ASDs). However, the functional connectivity (FC) behind their symptoms is still unknown. Methods: Based on FC analysis, the acquired resting-state functional magnetic resonance imaging (rs-fMRI) data sets, including 86 boys with ASD and 54 normal controls (NC), were used to detect the neural synchronous activity between brain regions. Pearson correlation analysis was used to evaluate the relationship between the abnormal FC value and clinical features. Results: Individuals with ASD showed enhanced FC between the right calcarine and the right lingual gyrus (LG). The right medial orbital frontal cortex also showed increased FC with bilateral inferior temporal gyrus (ITG) [two-tailed, voxel-level p < 0.001, gaussian random field (GRF) correction, cluster-level p < 0.05]. We did not find a correlation between the abnormal FC value and clinical scales. Conclusion: Our study reveals a possible relationship between atypical visual attention and poor learning ability in subjects with ASD, and delayed social language development may be a secondary symptom to ASD.

Abnormal Degree Centrality in Children with Low-Function Autism Spectrum Disorders: A Sleeping-State Functional Magnetic Resonance Imaging Study.

Purpose: This study used the graph-theory approach, degree centrality (DC) to analyze whole-brain functional networks at the voxel level in children with ASD, and investigated whether DC changes were correlated with any clinical variables in ASD children. Methods: The current study included 86 children with ASD and 54 matched healthy subjects Aged 2-5.5 years. Next, chloral hydrate induced sleeping-state functional magnetic resonance imaging (ss-fMRI) datasets were acquired from these ASD and healthy subjects. For a given voxel, the DC was calculated by calculating the number of functional connections with significantly positive correlations at the individual level. Group differences were tested using two-sample t-tests (p < 0.01, AlphaSim corrected). Finally, relationships between abnormal DCs and clinical variables were investigated via Pearson's correlation analysis. Results: Children with ASD exhibited low DC values in the right middle frontal gyrus (MFG) (p < 0.01, AlphaSim corrected). Furthermore, significantly negative correlations were established between the decreased average DC values within the right MFG in ASD children and the total ABC scores, as well as with two ABC subscales measuring highly relevant impairments in ASD (ie, stereotypes and object-use behaviors and difficulties in language). Conclusion: Taken together, the results of our ss-fMRI study suggest that abnormal DC may represent an important contribution to elucidation of the neuropathophysiological mechanisms of preschoolers with ASD.

A 4-day-old newborn with an isolated sternal cleft: A rare case and literature review.

An isolated sternal cleft is an orphan congenital defect of the sternum that presents from birth to adulthood. We report the case of a 4-day-old newborn with an isolated congenital cleft sternum. We also reviewed the related literature and operative options.

[The relationship between chest CT findings and viral load in patients with novel influenza A (H1N1)].

OBJECTIVE: To evaluate the value of chest CT findings and dynamic changes of viral load in patients with novel influenza A (H1N1) infection in clinical diagnosis, differential diagnosis and treatment. METHODS: Fifty-one patients with confirmed novel influenza A (H1N1) according to the diagnostic criteria of the Ministry of Health, received chest X-ray, CT scans (HRCT) and viral load tests in our hospital from May to December of 2009. Based on whether there were signs of pneumonia in CT imaging, the patients were divided into a pneumonia group (n = 31) and a non-pneumonia group (n = 20). The relationship between chest CT changes and viral load was observed and analyzed statistically using SPSS 10.5 software. RESULTS: Patchy consolidations of lungs were the main findings in pneumonia group with influenza A (H1N1) infection, and ground-glass opacities were the main CT findings at acute and convalescent phases. Lobular and segmental shadows of the lungs were diffusely distributed, mostly found in lower lungs, especially the left lung. In some cases, the lung diseases were accompanied with mediastinal lymphadenopathy. Co-existence of pulmonary parenchymal, interstitial and pleural diseases was observed. Peak viral load occurred at the early phase of illness, with the mean initial viral load being 7.7 copies/ml and 4.2 copies/ml in the pneumonia and the non-pneumonia groups respectively. The viral nucleic acid became negative 4 days after antiviral treatment (course of 6 days). Dynamic observation of 3 patients with novel influenza A (H1N1) pneumonia showed that, the viral clearance period preceded the absorption of lung lesions in 2 cases, but viral clearance period of a young patient was significantly prolonged. CONCLUSION: In patients with the novel influenza A (H1N1) infection, the viral load in the pneumonia group was significantly higher than that in the group with normal chest imaging. Dynamic observation on chest imaging and viral load may be beneficial for clinicians to start prompt and effective treatment.

Accuracy of multi-echo Dixon sequence in quantification of hepatic steatosis in Chinese children and adolescents.

BACKGROUND: Nonalcoholic fatty liver disease (NAFLD) is currently the outstanding cause of chronic liver disease in children and adolescents, especially in overweight and obese groups. Liver biopsy is the reference standard to diagnose NAFLD but invasive, thus it is not the best choice in clinical diagnosis and follow-up. Magnetic resonance (MR) is widely used in clinical trials to noninvasively quantify liver fat content in adults and children in foreign countries. While currently, it is rarely used in Chinese children and adolescents. We postulated that quantifying hepatic steatosis by MR could be extended to children and adolescents in China. AIM: To investigate the accuracy of MR imaging (MRI) in quantifying liver fat with MR spectroscopy (MRS) as a reference. A secondary goal was to assess the prevalence of NAFLD in overweight and obese Chinese children and adolescents. METHODS: There were 86 children and adolescents enrolled in this study, including 65 overweight and obese children and 21 healthy children. The participants underwent MRI and MRS. MRI and MRS were performed using multi-echo Dixon and HISTO sequences, respectively, to calculate hepatic proton density fat fraction (PDFF). Hepatic steatosis was diagnosed using MRS-PDFF > 5% as the threshold. Spearman's analysis was used to evaluate the correlation between MRI and MRS. The agreement between these two methods was assessed by Bland-Altman analysis. RESULTS: The MRI-PDFF in the MRS region of interest and the entire liver was 9.9% ± 10.3% with a range of 0.3%-39.9%, and 10.6% ± 9.4% with a range of 1.9%-38.9%, respectively. The MRS-PDFF was 9.1% ± 10.0%, with a range of 0.5%-37.8%. The incidence of hepatic steatosis detected by MRS-PDFF was 46.5% (40/86) of all participants, all of whom belonged to the overweight and obese group. Spearman's analysis indicated an excellent correlation between multi-echo Dixon and MRS (r > 0.9, P < 0.01). Bland-Altman analysis also demonstrated a good agreement between these two methods. CONCLUSION: Multi-echo Dixon shows an excellent correlation and agreement with MRS in quantifying liver fat content and could be a potential tool to detect hepatic steatosis in Chinese children and adolescents.

Altered Functional Connectivity in Children With Low-Function Autism Spectrum Disorders.

Neuroimaging studies have shown that autism spectrum disorders (ASDs) may be associated with abnormalities in brain structures and functions at rest as well as during cognitive tasks. However, it remains unclear if functional connectivity (FC) of all brain neural networks is also changed in these subjects. In this study, we acquired functional magnetic resonance imaging scans from 93 children with ASD and 79 matched healthy subjects. Group independent component analysis was executed for all of the participants to estimate FC. One-sample t-tests were then performed to obtain the networks for each group. Group differences in the different brain networks were tested using two-sample t-tests. Finally, relationships between abnormal FC and clinical variables were investigated with Pearson's correlation analysis. The results from one-sample t-tests revealed nine networks with similar spatial patterns in these two groups. When compared with the controls, children with ASD showed increased connectivity in the right dorsolateral superior frontal gyrus and left middle frontal gyrus (MFG) within the occipital pole network. Children with ASD also showed decreased connectivity in the left gyrus rectus, left middle occipital gyrus, right angular gyrus, right MFG and right inferior frontal gyrus (IFG), orbital part within the lateral visual network (LVN), the left IFG, right precuneus, and right angular gyrus within the left frontoparietal (cognition) network. Furthermore, the mean FC values within the LVN showed significant positive correlations with total score of the Childhood Autism Rating Scale. Our findings indicate that abnormal FC extensively exists within some networks in children with ASD. This abnormal FC may constitute a biomarker of ASD. Our results are an important contribution to the study of neuropathophysiological mechanisms in children with ASD.

The Safety and Effectiveness of Intravenous Contrast-Enhanced Sonography in Chinese Children-A Single Center and Prospective Study in China.

Background and Objective: Intravenous contrast-enhanced ultrasound (CEUS), using the second-generation ultrasound contrast agent SonoVue®, has been widely used in adults. In 2016, it was approved for pediatric applications by the American Food and Drug Administration (FDA). However, it has not been approved by the Chinese Food and Drug Administration (CFDA). The objective of the study was to evaluate the safety and effectiveness of CEUS in children prospectively at a single center in China. Methods: A total of 312 cases of Chinese children were enrolled in clinical trials. Contrast agent was given intravenously with two different doses, including 2.4 ml/time and 0.03 ml/kg. All CEUS was performed for evaluating adverse effect and the diagnostic accuracy compared with the pathology and enhanced CT. Results: All 312 subjects underwent CEUS successfully. The dose of contrast agent for CEUS was 2.4 ml between November 2015 and June 2016, which was modified to 0.03 ml/kg between July 2016 and April 2019, according to the recommendation of the FDA. With the two different doses of the contrast agent, the heart rate, respiration rate, oxygen saturation, and blood pressure of the participants had no statistically significant difference (P > 0.05) before and after administration. The blood pressure had been significantly decreased in participants who received combined anesthetic administration. Following 600 intravenous injections of the CEUS, there were three cases of transient rash and three cases of hypotension (n = 6, 1.92%). The six recovered quickly after receiving intravenous methylprednisolone and epinephrine. Most of the studies were performed for evaluating renal microcirculation and assisting renal biopsy (192/312 [61.5%]), which together had a 98.9% effectiveness in the identification of pathology in the specimens. Some studies were conducted to identify a mass in the liver, retroperitoneum, abdominal cavity, kidneys, testicles, thyroid, and so on (99/312 [31.4%]), which had a 97.6% accuracy. The other studies were conducted to identify trauma, vascular malformation, infection, hemorrhage, and so on (21/312 [6.73%]), which had a similar accuracy to enhanced CT. Conclusion: The adverse effects of CEUS in children are similar to that in adults. The results indicate that it is safe to use SonoVue® for CEUS in pediatric patients.

Both Hypo-Connectivity and Hyper-Connectivity of the Insular Subregions Associated With Severity in Children With Autism Spectrum Disorders.

Some studies identified hypo-connectivity, while others showed hyper-connectivity of the insula in the autism spectrum disorders (ASD). These contradictory findings leave open the question of whether and to what extent functional connectivity of the insula is altered and how functional connectivity of the insula is associated with the severity of ASD. A newly emerging insular atlas that comprises multiple functionally differentiated subregions provides a new framework to interpret the functional significance of insular findings and uncover the mechanisms underlying the severity of ASD. Using the new insular atlas, the present study aimed to investigate the distinct functional connectivity of the insular subregions and their associations with ASD severity in a cohort of 49 children with ASD and 33 typically developing (TD) subjects. We found that compared with TD group, the ASD group showed different connectivity patterns in the left ventral agranular insula, right ventral dysgranular and granular insula, and dorsal dysgranular insula, characterized by significant hyper-connectivity and/or hypo-connectivity with special brain regions. Furthermore, both the hypo-connectivity and hyper-connectivity patterns of the insular subregions were significantly associated with the severity of ASD symptoms. Our research demonstrated distinct functional connectivity patterns of the insular subregions and emphasized the importance of the subdivisions within the insula to the potential impact of functional difference in children with ASD. Moreover, these results might help us to better understand the mechanisms underlying the symptoms in children with ASD and might elucidate potential biomarkers for clinical applications.

Alterations in Spontaneous Brain Activity and Functional Network Reorganization following Surgery in Children with Medically Refractory Epilepsy: A Resting-State Functional Magnetic Resonance Imaging Study.

For some patients with medically refractory epilepsy (MRE), surgery is a safe and effective treatment for controlling epilepsy. However, the functional consequences of such surgery on brain activity and connectivity in children remain unknown. In the present study, we carried out a longitudinal study using resting-state functional magnetic resonance imaging in 10 children with MRE before and again at a mean of 79 days after surgery, as well as in a group of 28 healthy controls. Compared with the controls, children with epilepsy exhibited abnormalities in intrinsic activity in the thalamus, putamen, pallidum, insula, hippocampus, cerebellum, and cingulate gyrus both before and after surgery. Longitudinal analyses showed that the amplitude of low frequency fluctuations (ALFF) increased in the parietal-frontal cortex and decreased in the deep nuclei from pre- to post-surgery. The percentage changes in ALFF values in the deep nuclei were positively correlated with the age of epilepsy onset. Functional connectivity (FC) analyses demonstrated a reorganization of FC architecture after surgery. These changes in brain activity and FC after surgery might indicate that the previously disrupted functional interactions were reorganized after surgery. All these results provide preliminary evidence that the age of epilepsy onset may have some potential to predict the outcome of brain functional reorganization after surgery in children with MRE.

Diffusion tensor imaging detects early brain microstructure changes before and after ventriculoperitoneal shunt in children with high intracranial pressure hydrocephalus.

To explore the use of diffusion tensor imaging (DTI) parameters in the quantitative assessment of early brain microstructure changes before and after ventriculoperitoneal shunt in children with high intracranial pressure hydrocephalus.Ten patients with communicating hydrocephalus (age: 2-36 months) and 14 age-/gender-matched controls (age: 2-36 months) were enrolled in this study. All patients underwent the ventriculoperitoneal shunt procedure. The imaging data were collected before and 3 months after the operation. Regions of interests (ROIs) included the white matter near the frontal horn of the lateral ventricles (FHLV), the occipital horn of the lateral ventricles (OHLV), occipital subcortical (OS) area, frontal subcortical (FS) area, and thalamus. Fractional anisotropies (FA) and apparent diffusion coefficients (ADC) of the ROIs before and after ventriculoperitoneal shunt were compared between the patients and the controls.Three months after surgery, the patients recovered from the surgery with ameliorated intracranial pressure and slight improvement of clinical intelligence scale and motor scale. Before ventriculoperitoneal shunt, the FA values (except the right FHLV) were significantly decreased and the ADC values were significantly increased in the patients with hydrocephalus, compared with the controls. After the ventriculoperitoneal shunt, the FA values in the FHLV and OHLV of the patients were similar to the controls, but the FA values in other ROIs were still significantly lower than controls. The ADC values in the FS and OS white matter areas of the patients were similar to the controls; however, the ADC values in other ROIs were still significantly higher in patients.The increase of FA and the reduction in ADC in the ROIs preceded the clinical function improvement in patients with high intracranial pressure hydrocephalus and reflected the early changes in brain tissue microstructure, such as the compression of the white matter areas in the ROIs.

New Findings, Classification and Long-Term Follow-Up Study Based on MRI Characterization of Brainstem Encephalitis Induced by Enterovirus 71.

BACKGROUND: To report the diversity of MRI features of brainstem encephalitis (BE) induced by Enterovirus 71. This is supported by implementation and testing of our new classification scheme in order to improve the diagnostic level on this specific disease. METHODS: Neuroimaging of 91 pediatric patients who got EV71 related BE were hospitalized between March, 2010 to October, 2012, were analyzed retrospectively. All patients underwent pre- and post-contrast MRI scan. Thereafter, 31 patients were randomly called back for follow-up MRI study during December 2013 to August 2014. The MRI signal patterns of BE primary lesion were analyzed and classified according to MR signal alteration at various disease stages. Findings in fatal and non-fatal cases were compared, and according to the MRI scan time point during the course of this disease, the patients' conditions were classified as 1) acute stage, 2) convalescence stage, 3) post mortem stage, and 4) long term follow-up study. RESULTS: 103 patients were identified. 11 patients did not undergo MRI, as they died within 48 hours. One patient died on 14th day without MR imaging. 2 patients had postmortem MRI. Medical records and imaging were reviewed in the 91 patients, aged 4 months to 12 years, and two cadavers who have had MRI scan. At acute stage: the most frequent pattern (40 patients) was foci of prolonged T1 and T2 signal, with (15) or without (25) contrast enhancement. We observed a novel pattern in 4 patients having foci of low signal intensity on T2WI, with contrast enhancement. Another pattern in 10 patients having foci of contrast enhancement without abnormalities in T1WI or T2WI weighted images. Based on 2 cases, the entire medulla and pons had prolonged T1 and T2 signal, and 2 of our postmortem cases demonstrated the same pattern. At convalescence stage, the pattern observed in 4 patients was foci of prolonged T1 and T2 signal without contrast enhancement. Follow-up MR study of 31 cases showed normal in 26 cases, and demonstrated foci of prolonged T1 and T2 signal with hyper-intensity on FLAIR in 3 cases, or of prolonged T1 and T2 signal with hypo-intensity on FLAIR in 2 cases. Most importantly, MR findings of each case were thoroughly investigated and classified according to phases and MRI signal alteration. CONCLUSIONS: This study has provided enhanced and useful information for the MRI features of BE induced by EV71, apart from common practice established by previous reports. In addition, a classification scheme that summarizes all types of features based on the MRI signal at the four different stages of the disease would be helpful to improve the diagnostic level.