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BackgroundPatients with intraventricular hemorrhage (IVH) are at a higher risk of developing hydrocephalus and often require external ventricular drainage or long-term ventriculoperitoneal shunt surgery.ObjectiveTo investigate whether cerebrospinal fluid drainage in patients with IVH due to aneurysmal subarachnoid hemorrhage (aSAH) reduces the incidence of chronic hydrocephalus.MethodA retrospective analysis was conducted on patients with aSAH treated at our hospital between January 2020 and December 2022. The first analysis compared patients with and without IVH, while the second analysis compared IVH patients with and without chronic hydrocephalus. The third analysis compared IVH patients who underwent in different drainage methods which is lumbar drainage (LD) or external ventricular drainage (EVD). The primary outcome measure was the incidence of chronic hydrocephalus.ResultOf the 296 patients hospitalized with aSAH, 108 (36.5%) had IVH, which was associated with a significantly higher incidence of chronic hydrocephalus compared to patients without IVH (49.1% vs. 16.5%, p

Fractures of multi-scales and multi-origins are primary storage space and effective seepage channels for metamorphic buried-hill reservoirs. They not only allow communication between various pores to enhance storage and seepage capacity, but also are essential for high yields. Fracture development and controlling factors at metamorphic buried-hill reservoirs of Bozhong 19-6 Gas Field were investigated based on imaging log, core data, experiments, e.g., thin sections and scanning electron microscope, and outcrop description. Results show that structural fractures, weathering fractures and dissolution fractures were developed in the metamorphic buried-hill reservoirs in the study area, among which structural fractures and weathering fractures are the most popular ones. However, fracture types varied obviously among different structure positions. Lithology, fault, weathering crust and ancient landform are primary factors affecting fracture distribution. Fractures were prone to be developed at lithology with high bright mineral contents. Faults were characterized by a dual structure including fault core and damage zone. Fracture density followed a decreasing trend with increasing distance from faults. Damage zone could be identified when fracture density was consistent with regional fracture density. The width of the damage zone was determined by factors such as fault scale and structure location. Well-connected weathering fractures were popular at the leached unit, with structural fractures of secondary importance. The unweathered unit was dominated by structural fractures with low density.

Natural fractures are regarded as important reservoir spaces and effective seepage channels at metamorphic buried hills. Continuous networks associated with multi-scale fractures with good connectivity is critical for high-quality reservoirs as well as high and stable production in the tight metamorphic rocks. The multi-scale fractures in Bozhong 19–6 metamorphic buried hills were well characterized through integrating image logs, cores, thin-sections, and scanning electron microscope, etc. After that, power-law distribution of multi-scale fractures was established to understand contribution of fractures to reservoir quality and figure out structure models of fracture networks as well as their impact on production. Results show that parameters of fracture systems vary regularly with fracture scales. Fracture development degree, e.g., cumulative areal density, increases as a power law function with decreasing fracture size from macro to micro (e.g., aperture and/or length), where storage space associated with micro fractures is also increased. Reversely, fracture connectivity and permeability follow a significant decreasing trend. Five structure models of fracture network were established based on combination pattern of multi-scale fractures: multi-scale fracture network with high-density and multi-sets, large-scale fracture network with medium-density and multi-sets, small-scale fracture network with high-density and multi-sets, large-scale fracture network with low-density and multi-sets, and small-scale fracture network with low-density and single-set. The former two fracture networks can be widely developed into high-quality reservoirs, contributing greatly to high and stable yields. Fracturing is required for the third and the fourth fracture networks to obtain stable production, while it is difficult for the fifth fracture network to obtain industrial oil and gas flow.

The identification of the “sweet spot” of low-permeability sandstone reservoirs is a basic research topic in the exploration and development of oil and gas fields. Lithology identification, reservoir classification based on the pore structure and physical properties, and petrophysical facies classification are common methods for low-permeability reservoir classification, but their classification effect needs to be improved. The low-permeability reservoir is characterized by low rock physical properties, small porosity and permeability distribution range, and strong heterogeneity between layers. The seepage capacity and productivity of the reservoir vary considerably. Moreover, the logging response characteristics and resistivity value are similar for low-permeability reservoirs. In addition to physical properties and oil bearing, they are also affected by factors such as complex lithology, pore structure, and other factors, making it difficult for division of reservoir petrophysical facies and “sweet spot” identification. In this study, the logging values between low-porosity and -permeability reservoirs in the Paleozoic Es3 reservoir in the M field of the Bohai Sea, and between natural gamma rays and triple porosity reservoirs are similar. Resistivity is strongly influenced by physical properties, oil content, pore structure, and clay content, and the productivity difference is obvious. In order to improve the identification accuracy of “sweet spot,” a semi-supervised learning model for petrophysical facies division is proposed. The influence of lithology and physical properties on resistivity was removed by using an artificial neural network to predict resistivity R0 saturated with pure water. Based on the logging data, the automatic clustering MRGC algorithm was used to optimize the sensitive parameters and divide the logging facies to establish the unsupervised clustering model. Then using the divided results of mercury injection data, core cast thin layers, and logging faces, the characteristics of diagenetic types, pore structure, and logging response were integrated to identify rock petrophysical facies and establish a supervised identification model. A semi-supervised learning model based on the combination of “unsupervised supervised” was extended to the whole region training prediction for “sweet spot” identification, and the prediction results of the model were in good agreement with the actual results.

Background: Patients with intraventricular hemorrhage (IVH) are at a higher risk of developing hydrocephalus and often require external ventricular drainage or long-term ventriculoperitoneal shunt surgery. Objective: To investigate whether cerebrospinal fluid drainage in patients with IVH due to aneurysmal subarachnoid hemorrhage (aSAH) reduces the incidence of chronic hydrocephalus. Method: A retrospective analysis was conducted on patients with aSAH treated at our hospital between January 2020 and December 2022. The first analysis compared patients with and without IVH, while the second analysis compared IVH patients with and without chronic hydrocephalus. The third analysis compared IVH patients who underwent in different drainage methods which is lumbar drainage (LD) or external ventricular drainage (EVD). The primary outcome measure was the incidence of chronic hydrocephalus. Result: Of the 296 patients hospitalized with aSAH, 108 (36.5%) had IVH, which was associated with a significantly higher incidence of chronic hydrocephalus compared to patients without IVH (49.1% vs. 16.5%, p < 0.001). Multivariate logistic regression analysis showed that IVH was independently associated with the formation of chronic hydrocephalus (OR: 3.530, 95% CI: 1.958-6.362, p < 0.001). Among the 108 IVH patients, 53 (49.1%) developed chronic hydrocephalus. Multivariate logistic regression analysis revealed that the Hunt Hess grade at admission (OR: 3.362, 95% CI: 1.146-9.863, p = 0.027) and postoperative cerebrospinal fluid drainage (OR: 0.110, 95% CI: 0.036-0.336, p < 0.001) were independent risk factors for the development of chronic hydrocephalus in IVH patients. Among all IVH patients who underwent cerebrospinal fluid drainage, 45 (75%) received continuous lumbar puncture drainage, and 15 (25%) received external ventricular drainage. Univariate analysis did not show a statistically significant difference between the two groups in terms of postoperative chronic hydrocephalus (p = 0.283). However, multivariate logistic regression analysis suggested that the drainage methods of LD and EVD might be associated with the development of chronic hydrocephalus. Conclusion: The presence of IVH increases the risk of chronic hydrocephalus in patients with aSAH, and postoperative cerebrospinal fluid drainage appears to reduce this risk. The specific effects of lumbar puncture drainage and ventricular drainage on the incidence of chronic hydrocephalus require further investigation. [ABSTRACT FROM AUTHOR]

ObjectiveTo explore the electroencephalogram (EEG) and clinical characteristics of childhood bathing epilepsy.MethodsWe conducted a prospective summary of the clinical data from 10 children with bathing epilepsy who were admitted to Hunan Children’s Hospital from April 2019 to November 2023 and analyzed their EEGs and clinical characteristics.ResultsOur 10 patients included eight males and two females, with seizure-onset ages ranging from 4 months and 20 days to 14 months. Nine cases showed normal intellectual development, and one case manifested delayed development. The Video-EEG (VEEG) findings showed that nine cases exhibited normal background with no interictal epileptic discharge. The seizures were characterized by lip cyanosis, tachycardia or bradycardia, weakness, paleness, and loss of consciousness. Ictal EEG revealed rhythmic fast waves, spike waves, spike-slow waves, or slow and sharp-wave activity over the temporal region (eight cases) or the occipital and temporal regions (one case), finally evolving into a delta rhythm that lasted for 57–201 s. These children exhibited no seizures after discontinuing bathing and were not administered antiseizure medication. The interictal EEG of one case reflected mild slow background and focal interictal epileptic discharge; and her semiology was eyes gazing to right, with clonic movements of the right face and lips, lip cyanosis, bradycardia, and impaired consciousness. Ictal EEG showed spike–wave and spike-slow-wave rhythms over the left central, parietal, and temporal regions; these then spread to the left hemisphere, lasting for approximately 104 s. This patient did not exhibit bathing seizures after stopping her bathing but later experienced frequent spontaneous and drug-resistant seizures. The interictal EEG background slowed down, while focal epileptic discharge increased. Her intellectual development was significantly delayed, and a novel pathogenic mutation in the SMC1A gene, c.298+2T>C, was detected. She was diagnosed with developmental and epileptic encephalopathy.ConclusionA majority of children with bathing epilepsy in our study showed focal autonomic seizures accompanied by impaired consciousness. Stopping bathing could control the seizures and showed a good prognosis. A few infants manifested a poor prognosis, and we posit that bathing seizure rarely constitute the early manifestations of developmental and epileptic encephalopathy. VEEG findings and clinical features can also indicate the prognosis.

ObjectiveThis study aimed to examine the clinical and gene-mutation characteristics of pediatric patients with sodium channel gene mutation-related childhood epilepsy and to provide a basis for precision treatment and genetic counseling.MethodsThe clinical data from 94 patients with sodium channel gene mutation-related childhood epilepsy who were treated at Hunan Children's Hospital from August 2012 to December 2022 were retrospectively evaluated, and the clinical characteristics, gene variants, treatment, and follow-up status were analyzed and summarized.ResultsOur 94 pediatric patients with sodium channel gene variant-related childhood epilepsy comprised 37 girls and 57 boys. The age of disease onset ranged from 1 day to 3 years. We observed seven different sodium channel gene variants, and 55, 14, 9, 6, 6, 2, and 2 patients had SCNlA, SCN2A, SCN8A, SCN9A, SCN1B, SCN11A, and SCN3A variants, respectively. We noted that 52 were reported variants and 42 were novel variants. Among all gene types, SCN1A, SCN2A, and SCN8A variants were associated with an earlier disease onset age. With the exception of the SCN1B, the other six genes were associated with clustering seizures. Except for variants SCN3A and SCN11A, some patients with other variants had status epilepticus (SE). The main diagnosis of children with SCN1A variants was Dravet syndrome (DS) (72.7%), whereas patients with SCN2A and SCN8A variants were mainly diagnosed with various types of epileptic encephalopathy, accounting for 85.7% (12 of 14) and 88.9% (8 of 9) respectively. A total of five cases of sudden unexpected death in epilepsy (SUDEP) occurred in patients with SCN1A, SCN2A, and SCN8A variants. The proportion of benign epilepsy in patients with SCN9A, SCN11A, and SCN1B variants was relatively high, and the epilepsy control rate was higher than the rate of other variant types.ConclusionSodium channel gene variants involve different epileptic syndromes, and the treatment responses also vary. We herein reported 42 novel variants, and we are also the first ever to report two patients with SCN11A variants, thereby increasing the gene spectrum and phenotypic profile of sodium channel dysfunction. We provide a basis for precision treatment and prognostic assessment.

ObjectiveAnti-contactin-associated protein 2 (CASPR2)-related autoimmune encephalitis (AE) is more common in adults than in children. Clinical understanding of anti-CASPR2-antibody (Ab)-related AE, diagnosis and treatment standards are lacking in children. Therefore, this retrospective study on clinical symptoms and treatment outcomes in children with anti-CASPR2-Ab-related AE was conducted, to improve the clinical understanding of the disease, its diagnosis and treatment.MethodsThis study retrospectively assessed children with anti-CASPR2-Ab-related AE from January 1, 2020, to June 30, 2022, in the Department of Neurology at Hunan Children’s Hospital. Data regarding demographics, clinical symptoms, laboratory examinations, electroencephalography (EEG), imaging, and curative were collected.ResultsThirteen patients were positive for serum anti-CASPR2-Ab (age at manifestation, 25 months to 13 years old; median, 8.1 years old; male-to-female ratio, 8/5). One patient (P1) had dual Abs, including anti-CASPR2 and anti-N-methyl-D-aspartate receptor Abs; his symptoms were more severe than those of children with anti-CASPR2 Abs alone. The clinical symptoms of the 13 patients with anti-CASPR2 Ab were movement disorders (9/13), consciousness disorders (9/13), abnormal demeanor (8/13), seizures (7/13), language disorders (6/13), fever (6/13), pain (4/13), involuntary exercise (4/13), poor diet (4/13), vomiting (3/13), sleep disorders (3/13), mood disorders (3/13), eczema/itching/redness (2/13), sweating (P8), urinary disorders (P13), and cognitive disorders (P9). No tumors were found in any patient. Additionally, EEG results of six patients were abnormal and imaging findings such as abnormal signals were found in 10 patients. Moreover, all except one patient recovered well after treatment; P1 with overlapping syndrome underwent recovery for more than 2 years. None of the patients who recovered have had a relapse.Discussion and conclusionAnti-CASPR2-Ab-related AE has several clinical manifestations. Anti-CASPR2-Ab levels were higher in male patients than in female patients. Moreover, related tumors are relatively rare. Most patients benefit from immunotherapy and have a lower chance of recurrence in the short term. Furthermore, different from patients who had anti-CASPR2-Ab AE alone, those with overlapping syndrome had a severe and complex condition requiring lengthy treatment and rehabilitation. Additional studies are needed to evaluate the long-term prognosis of these patients.

BackgroundAntibody-mediated disorders of the central nervous system (CNS) have seen a gradual rise in their incidence and prevalence. This retrospective observational study aimed to investigate the clinical characteristics and short-term prognosis of children with antibody-mediated CNS autoimmune diseases at Hunan Children’s Hospital.MethodsWe collected the clinical data of 173 pediatric patients diagnosed with antibody-mediated CNS autoimmune diseases between June 2014 and June 2021 and analyzed their demographics, clinical features, imaging and laboratory data, treatment, and prognosis.ResultsA total of 187 patients tested positive for anti-neural antibodies and 173 patients were finally diagnosed with antibody-mediated CNS autoimmune diseases after excluding the 14 false-positive cases through clinical phenotypic evaluation and follow-up of treatment outcomes. Of the 173 confirmed patients, 97 (56.06%) were positive for anti-NMDA-receptor antibody, 48 (27.75%) for anti-MOG antibody, 30 (17.34%) for anti-GFAP antibody, 5 (2.89%) for anti-CASPR2 antibody, 3 (1.73%) for anti-AQP4 antibody, 2 (1.16%) for anti-GABABR antibody, and 1 (0.58%) for anti-LGI1antibody. Anti-NMDAR encephalitis was the most commonly seen among the patients, followed by MOG antibody-associated disorders and autoimmune GFAP astrocytopathy. Psycho-behavioral abnormalities, seizures, involuntary movements, and speech disorder were the most common clinical presentations of anti-NMDAR encephalitis, while fever, headache, and disturbance of consciousness or vision were the most seen among patients with MOG antibody-associated disorders or autoimmune GFAP astrocytopathy. The coexistence of multiple anti-neural antibodies was detected in 13 patients, among which 6 cases had coexistent anti-NMDAR and anti-MOG antibodies (including 1 case with anti-GFAP antibody also), 3 cases had coexistent anti-NMDAR and anti-GFAP antibodies, 3 cases had coexistent anti-MOG and anti-GFAP antibodies, 1 case had coexistent anti-NMDAR and anti-CASPR2 antibodies, and 1 case had coexistent anti-GABABR and anti-CASPR2 antibodies. All the survivors were followed up for at least 12 months; 137 recovered completely, 33 had varying sequelae, and 3 died; 22 had one or more relapses.ConclusionAntibody-mediated CNS autoimmune diseases occur in children of all ages. Most such pediatric patients have a good response to immunotherapy. Despite the low mortality rate, some survivors have a non-negligible risk of developing relapses.

Biallelic NARS2 mutations can cause various neurodegenerative diseases, leading to growth retardation, intractable epilepsy, and hearing loss in early infancy and further progressing to spastic paraplegia, neurodegeneration, and even death. NARS2 mutations are associated with mitochondrial dysfunction and cause combined oxidative phosphorylation deficiency 24 (COXPD24). Relatively few cases have been reported worldwide; therefore, the pathogenesis of COXPD24 is poorly understood. We studied two unrelated patients with COXPD24 with similar phenotypes who presented with intractable refractory epilepsia partialis continua, hearing loss, and growth retardation. One patient died from epilepsy. Three novel NARS2 variants (case 1: c.185T > C and c.251 + 2T > G; case 2: c.185T > C and c.509T > G) were detected with whole-exome sequencing. c.251 + 2T > G is located at the donor splicing site in the non-coding sequence of the gene. The minigene experiment further verified that c.251 + 2T > G caused variable splicing abnormalities and produced truncated proteins. Molecular dynamics studies showed that c.185T > C and c.509T > G reduced the binding free energy of the NARS2 protein dimer. The literature review revealed fewer than 30 NARS2 variants. These findings improved our understanding of the disease phenotype and the variation spectrum and revealed the potential pathogenic mechanism of non-coding sequence mutations in COXPD24.

BackgroundThe incidence and prevalence of autoimmune encephalitis (AE) is gradually increasing in pediatric patients (between the ages of 3 months and 16 years). The aim of this retrospective observational study was to investigate the clinical characteristics and short-term prognosis of children with antibody-mediated AE at Hunan Children's Hospital.MethodsAntibody analysis of blood and/or cerebrospinal fluid was performed in suspected AE patients admitted to the Department of Neurology, Hunan Children's Hospital from June 2014 to June 2021. Ultimately, 103 patients were diagnosed with antibody-mediated AE and were enrolled in this study. Clinical data and corresponding demographic, clinical characteristics, laboratory and imaging data, treatment, and prognosis data were collected and analyzed.ResultsIn our study, 103 AE patients with antibody-positive were identified. The main subtype of AE in our cohort was anti-NMDAR encephalitis. Few patients have anti-CASPR2 encephalitis, anti-GABABR encephalitis, or anti-LGI1 encephalitis. In our AE patients, the most common clinical manifestations were behavioral symptoms, seizures, and involuntary movements, with seizures being the most common initial symptom. All patients underwent brain magnetic resonance imaging (MRI) and electroencephalography (EEG). Forty-five (43.7%) patients had abnormal MRI findings. And 96 (93.2%) patients had abnormal EEG results. All 103 patients were given first-line immunotherapy, 21 of which were also treated with the combination of the second-line immunotherapy. All surviving patients were followed up for at least 6 months. Seventy-seven patients recovered completely, 23 had sequelae of different degrees, and 3 died. Eight patients had one or more relapses during the follow-up period.ConclusionsAE is a treatable disease that can occur in children of all ages. The mortality rate is low, as most patients have a good response to immune therapy. Compared with the older children, infants and young children (≤ 3 years old) with anti-NMDAR encephalitis have a higher incidence of fever and status epilepticus, more severe condition, higher PICU admission rate and worse prognosis. AE patients with high maximum mRS scores and PICU admissions may require second-line immunotherapy.

ObjectiveTo analyze the clinical manifestations, imaging, electroencephalography, treatment, and prognosis of 35 cases of autoimmune glial fibrillary acidic protein astrocytopathy (GFAP-A) in children.MethodsChildren hospitalized in the Department of Neurology, Hunan Children’s Hospital, China, between January 2015 and June 2021, owing to autoimmune diseases of the central nervous system were subjected to a cell-based assay (CBA). The assay identified 40 children positive for GFAP-immunoglobulin (Ig)G antibodies in the serum and/or the cerebrospinal fluid. Based on clinical manifestations and imaging characteristics, five children who were only positive for GFAP-IgG antibodies in serum were excluded, and the remaining 35 children were diagnosed with autoimmune GFAP-A. The clinical data derived from the 35 children were retrospectively analyzed.ResultsA total of 35 children, including 23 males and 12 females with a mean age of 6.3 ± 0.6 years, manifested clinical symptoms of fever (62.9%), headache (42.9%), convulsions (42.9%), abnormal mental behavior (51.4%), disorders of consciousness (54.3%), visual disturbance (22.9%), ataxia (11.4%), paralysis (40%), and autonomic dysfunction (25.7%). One child exhibited only the clinical symptom of peripheral facial nerve palsy. Eleven out of 35 children were also positive for other antibodies. In addition to the common overlapping autoimmune syndromes, one case of autoimmune GFAP-A also manifested as Bickerstaff’s brainstem encephalitis. Linear periventricular enhancement upon MRI was significantly less frequent in children (8.5%) than in adults. In pediatric patients, MRI contrast enhancement was principally seen in the meninges and brain lobes. Although repeated relapse (17.1%) and sequelae symptoms (20%) occurred in some cases, most children showed a favorable prognosis. Spearman’s rank correlation showed that the antibody titer was not significantly associated with the severity of the initial disease conditions.ConclusionsThe disease diagnosis in children seropositive for GFAP antibodies only should receive a comprehensive diagnosis based on their clinical symptoms, imaging, electroencephalographic characteristics, and treatment responses. Some patients with relapses should receive repeated gamma globulin and corticosteroid therapy or the addition of immunosuppressants to their therapeutic regimen, and slow-dose tapering of corticosteroids and extended treatment are recommended for patients with overlapping autoimmune syndromes.