The effect of N-methyl-D-aspartate receptor antagonists on the mismatch negativity of event-related potentials and its regulatory factors: A systematic review and meta-analysis.

This study investigates the influence of N-methyl-D-aspartate receptor (NMDAR) antagonists on the mismatch negativity (MMN) components of event-related potentials (ERPs) in healthy subjects and explores whether NMDAR antagonists have different effects on MMN components under different types of antagonists, drug dosages, and deviant stimuli. We conducted a comprehensive literature search of PubMed, EMBASE, and the Cochrane Library from inception to August 1, 2023 for studies comparing the MMN components between the NMDAR antagonist intervention group and the control group (or baseline). All statistical analyses were performed using Stata version 12.0 software. Sixteen articles were included in the systematic review: 13 articles were included in the meta-analysis of MMN amplitudes, and seven articles were included in the meta-analysis of MMN latencies. The pooled analysis showed that NMDAR antagonists reduced MMN amplitudes [SMD (95% CI) = 0.32 (0.16, 0.47), P < 0.01, I2 = 47.3%, p < 0.01] and prolonged MMN latencies [SMD (95% CI) = 0.31 (0.13, 0.49), P = 0.16, I2 = 28.3%, p < 0.01]. The type of antagonist drug regulates the effect of NMDAR antagonists on MMN amplitudes. Different antagonists, doses of antagonists, and types of deviant stimuli can also have different effects on MMN. These findings indicate a correlation between NMDAR and MMN, which may provide a foundation for the application of ERP-MMN in the early identification of NMDAR encephalitis.

Corrigendum: Effectiveness of mycophenolate mofetil in the treatment of pediatric anti-NMDAR encephalitis: A retrospective analysis of 6 cases.

[This corrects the article DOI: 10.3389/fneur.2020.584446.].

Electroclinical characteristics of photosensitive epilepsy: A retrospective study of 31 Chinese children and literature review.

Objective: The objective of this study was to better understand the clinical features of photosensitive epilepsy (PSE) in Chinese children. Methods: Thirty-one children with PSE were screened out of 398 children with epilepsy who were consecutively diagnosed by the video-electroencephalogram (VEEG) monitoring method and by using an intermittent photic stimulation (IPS) test. Their EEGs and clinical features were retrospectively analyzed, and their treatment outcomes were followed up. Results: PSE accounted for 7.79% (31/398) of children with epilepsy during the observation period in our single epilepsy center. The male to female ratio of PSE was 1:3.43, and the average seizure onset age was 7.8 ± 3.28 years. The highest range of frequency sensitivity of the IPS test for the induction of EEG epileptic discharge or electroclinical seizures was within 10-20 Hz. Electroclinical seizures were induced in 41.94% (13/31) of PSE patients by using the IPS test, while EEG discharge without clinical seizures was induced in 58.06% (18/31) of PSE patients. Among all PSE patients, an IPS-positive reaction in the eye-closure state was induced in 83.87% of patients, and this rate was significantly higher than that in the eye-opened state (41.94%) or eye-closed state (35.48%). (Eye-closure IPS stimulation means: make the subjects close their eyes at the beginning of each stimulation, open their eyes at the end of the stimulation, and close their eyes again at the beginning of the next stimulation, and so on. While Eye-closed IPS stimulation means the stimulation is started after 5 s of eye closure, and the subjects are kept closed throughout the whole process.) The common and effective drugs used for single or combined therapy in PSE children were valproic acid and levetiracetam. Conclusion: This study provides some useful information about electroclinical characteristics in a cohort of 31 PSE children. It may be beneficial for pediatric neurologists in terms of paying more attention to PSE and correctly dealing with it.

Risk factors and prognosis of spinal cord injury without radiological abnormality in children in China.

BACKGROUND: Compared to adults, spinal cord injury without radiographic abnormality (SCIWORA) is more common in children due to the congenital spinal soft tissue elasticity and immature vertebral bodies. In this study, we aimed to investigate the risk factors and prognosis associated with SCIWORA in China. METHOD: We retrospectively examined patient records at the First Hospital of Jilin University from January 2007 to December 2020. Patients diagnosed with SCIWORA were included in the study group (n=16). The age, gender, history of trauma, symptoms, injury level of the spinal cord, the American Spinal Injury Association (ASIA) impairment score according to the International Standards for Neurological Classification of Spinal Cord Injury (ISNCSCI), as well as laboratory and imaging findings were analyzed. RESULT: The study group included 16 patients with SCIWORA with a mean age of 6.69±2.51 y. The ISNCSCI impairment scale was significantly different between the pre-school age patients (≤7 years old) and school age patients (>7 years old) before (P=0.044) and after therapy (P=0.002). Similarly, magnetic resonance imaging demonstrated a significant difference in the spinal injury level between pre-school age and school age patients (P=0.041). Further, the study group was subdivided into three subgroups according to the cause of trauma: Dance, Taekwondo, or Falls. Magnetic resonance imaging revealed significant differences among the three subgroups (P=0.041). CONCLUSION: Compared to school-age patients, pre-school-age patients were more vulnerable to SCIWORA with more severe ISNCSCI scores. Dance and Taekwondo are among the risk factors associated with SCIWORA in Chinese children.

Paroxysmal tonic upgaze accompanied by occipital discharge on electroencephalography: a case report and literature review.

BACKGROUND: Paroxysmal tonic upgaze (PTU) is an infantile-onset paroxysmal neurological disorder that is characterized by episodes of sustained conjugate upward eye deviation. The paroxysmal abnormal eye movements need to be differentiated from seizures. We report a case of PTU with occipital discharge on electroencephalography (EEG), which made the diagnosis more complicated. CASE PRESENTATION: A 6-month-old girl presented with paroxysmal upward deviation or left strabismus of the eyes, with a bowed head, lowered jaw, raised eyebrows, closed lips, and slight grin. Each episode lasted for a few seconds, and episodes occurred multiple times per day. EEG showed spike waves in the right occipital region, and the girl was initially misdiagnosed with epilepsy. After further analysis using video EEG, we corrected her diagnosis as PTU and stopped the administration of an antiepileptic drug. CONCLUSION: PTU accompanied by discharge on EEG may lead to a misdiagnosis. Video EEG monitoring, and especially the analysis of EEG traces synchronized with attacks, can provide evidence to distinguish between seizures and non-epileptic events.

Effectiveness of Mycophenolate Mofetil in the Treatment of Pediatric Anti-NMDAR Encephalitis: A Retrospective Analysis of 6 Cases.

Objective: To explore the effectiveness and safety of mycophenolate mofetil (MMF) as a second-line medication in the treatment of anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis, the most common and severe autoimmune encephalitis. Methods: The clinical data of six children with anti-NMDAR encephalitis admitted to the First Hospital of Jilin University were retrospectively analyzed, and the effectiveness and safety of MMF were evaluated. Results: Six children with anti-NMDAR encephalitis were treated with MMF in the 2nd or 3rd treatment disease event (3 cases vs. 3 cases). MMF initiation was mean 19.2 months (range 6-39 months) after disease onset at a mean dose of 25.6 mg/kg (range 19.6-28.4 mg/kg) for 14 months (range 6-26 months). Only two patients had transient mild diarrhea within 2 weeks of MMF application. During follow-up, one patient relapsed whilst on MMF, one patient discontinued MMF, and 4 cases were still on MMF. Conclusion: The use of MMF in anti-NMDAR encephalitis may be effective and safe. MMF can be used as one of the relapse prevention options in patients who already have relapsed or possibly even after the first event. Delayed use may be the main reason for MMF failure.

Clinical characteristics, treatment, and long-term outcomes in children suffering from benign convulsions with mild gastroenteritis: a retrospective study.

BACKGROUND: Benign convulsions with mild gastroenteritis (BCWG) is a common condition in children in Asia and is generally not associated with pH or electrolyte imbalances. When BCWG is diagnosed, a lumbar puncture is usually recommended to rule out potential intracranial infections. This study examined the clinical characteristics of BCWG and evaluated the necessity of lumbar puncture. METHODS: Medical records of children admitted to the First Hospital of Jilin University with BCWG between January 2018 and May 2019 were reviewed and analyzed. Children were stratified by rotavirus positivity or lumbar puncture status. Clinical characteristics and long-term outcomes were compared between groups. RESULTS: A total of 51 children were included in the analyses (55.1% rotavirus [HRV] positive). The average age of convulsion onset was 21.12 ± 7.44 months, the male-to-female ratio was 1.8:1, and convulsions occurred primarily between October 2018 and April 2019. The main clinical presentations of BCWG were convulsions, vomiting, diarrhea, and fever. Convulsions occurred predominantly two days after diagnosis of gastroenteritis, were mainly generalized tonic-clonic with 88.2% of children having ≤ 3 convulsions per episode, and had a mean duration of 2.0 minutes (interquartile range [IQR]: 1.0, 3.0). Children with BCWG had mild metabolic acidosis (HCO3- 17.82 ± 3.63 mmol/L) with an elevated anion gap (AG; 20.98 ± 3.00 mmol/L), mild hyponatremia (134.56 ± 2.85 mmol/L), and slightly increased levels of creatine kinase myocardial band (CKMB). HRV + children had more severe acidosis and higher CKMB levels. Cerebrospinal fluid (CSF) samples collected via lumbar puncture were normal. No developmental abnormalities were noted as assessed by the Social Life Ability Scale. CONCLUSIONS: BCWG is a situation-related seizure, with clinical presentations of tonic-clonic or focal convulsions and mild gastroenteritis (vomiting, diarrhea). Mild metabolic acidosis and hyponatremia may exist. The prognosis of the disease is favorable; lumbar puncture and long-term antiepileptics are unnecessary and should not be recommended.

Extracellular Vesicle-Derived microRNA-410 From Mesenchymal Stem Cells Protects Against Neonatal Hypoxia-Ischemia Brain Damage Through an HDAC1-Dependent EGR2/Bcl2 Axis.

Hypoxia-ischemia brain damage (HIBD) is a neurological disorder occring in neonates, which is exacerbated by neuronal apoptosis. Mesenchymal stem cells (MSCs)-derived extracellular vesicles (EVs) have been proposed as a promising strategy for treating or preventing ischemia-related diseases. However, their mechanisms in HIBD remain unclear. Thus, we aimed to address the role of EV-derived microRNA (miR)-410 in HIBD. Neonatal HIBD mouse model was constructed using HI insult, from which neurons were isolated, followed by exposure to oxygen glucose deprivation (OGD). EVs were isolated from human umbilical cord (hUC)-derived MSCs. In silico analyses, dual-luciferase reporter gene and chromatin immunoprecipitation assays were adopted to determine relationships among miR-410, histone deacetylase 1 (HDAC1), early growth response protein 2 (EGR2), and B cell lymphoma/leukemia 2 (Bcl2). The functional roles of EV-derived miR-410 were determined using loss- and gain-of functions experiments, and by evaluating neuronal viability, cell-cycle distribution and neuronal apoptosis in vitro as well as modified neurological severity score (mNSS), edema formation, and cerebral infarction volume in vivo. hUC-MSCs-derived EVs protected against HIBD in vivo and inhibited the OGD-induced neuronal apoptosis in vitro. miR-410 was successfully delivered to neurons by hUC-MSCs-EVs and negatively targeted HDAC1, which inversely mediated the expression of EGR2/Bcl2. Upregulation of EV-derived miR-410 promoted the viability but inhibited apoptosis of neurons, which was reversed by HDAC1 overexpression. EV-derived miR-410 elevation reduced mNSS, edema formation, and cerebral infarction volume by increasing EGR2/Bcl2 expression through downregulating HDAC1 expression in vivo. In summary, EV-derived miR-410 impeded neuronal apoptosis by elevating the expression of EGR2/Bcl2 via HDAC1 downregulation, thereby providing a potential strategy for treating or preventing HIBD.

Color density spectral array for early evaluation of prognosis of patients with coma in pediatric intensive care unit.

The aim of this study was to assess the bedside brain function monitoring of color density spectral array (CDSA) for early prognostic evaluation of coma patients in pediatric intensive care unit (PICU).Forty-two consecutive pediatric coma patients were enrolled. The individual conscious state was evaluated according to the Glasgow coma scale (GCS). CDSA parameters including CDSA pattern (CDSAP), sleep-wake cycle (SWC), sleep stage (SS), and drug-induced fast wave activity (DIFWA) were recorded. Three months later, prognosis was evaluated according to pediatric cerebral performance category (PCPC) score, based on which the patients were divided into FP-group (favorable prognosis) and PP-group (poor prognosis).The changeable type of CDSAP, appearance of SWC, SS, and DIFWA were significantly correlated with favorable prognosis. Both GCS and SWC were significantly correlated with the prognosis. However, there was substantial overlap in GCS between FP-group and PP-group. Although the absence of SWC was statistically an independent risk factor for poor prognosis but with a high false positive rate (0.143), a linear logistic regression showed the odds ratio of GCS for predicting prognosis was 0.93 (95% confidence interval: 0.48-1.80; P = .83) and that of SWC was 0.12 (95% confidence interval: 0.03-0.47; P = .03). Furthermore, the absence of SWC was correlated with poor prognosis in nonintracranial infection patients.Our study found that several CDSA factors are associated with prognosis of coma patients in PICU. SWC may be a potential indicator for evaluating the prognosis of coma patients in PICU.

Dynamic Expression of CX36 Protein in Kainic Acid Kindling induced Epilepsy.

Connexin (Cx) 36 is known to be a component of gap junctions, and has been suggested to play an important role in epilepsy. In order to determine dynamic changes of Cx36 protein expression in epilepsy and investigate the role of Cx36 in electroencephalographic activity and pathogenesis, we utilized kainic acid (KA) to induce epileptogenesis. We found that epileptic discharges began 71.8 ± 23.7 s after KA administration. Spike frequency and amplitude of epileptiform activity reached maximal levels at 30 ± 5.2 min. The maximum level of spike frequency and amplitude of epileptiform activity was 13.9 ± 0.3 Hz and 198 ± 14.3mV respectively. Employing Western blotting and immunohistochemistry, we demonstrated that hippocampal Cx36 protein expression was significantly increased 6 h after KA kindling compared to control or sham groups, but decreased in 3 d and 7d groups. Our results suggested that the dynamic change of Cx36 expression may play an important role inepilepsy, and the specific manipulation of Cx36 expression may be a potential target for the treatment of epilepsy.

3-Hydroxy-3-methylglutaric aciduria with bilateral basal ganglia lesion: A case report.

3-Hydroxy-3-methylglutaric aciduria (3-HMG, OMIN 246450) is a rare autosomal recessive metabolic disorder caused by a deficiency of 3-hydroxy-3-methylglutaryl-CoA lyase, a key enzyme in leucine metabolism and ketone body synthesis. Acute episodes of 3-HMG may be triggered by fasting or infection, and symptoms include vomiting, diarrhea, lethargy and hypotonia. If left untreated, prolonged hypoglycemia and metabolic acidosis may cause breathing problems, seizures, and coma. In addition, 3-HMG is associated with damage to the central nervous system, and there are several reports of white matter abnormality or cerebral atrophy. The presence of bilateral basal ganglia damage in 3-HMG has been rarely reported. Here, we present a case report of a 20-month old male with severe 3-HMG and prominent bilateral lesions in the basal ganglia.

Infantile mitochondrial disorder associated with subclinical hypothyroidism is caused by a rare mitochondrial DNA 8691A>G mutation: a case report.

Mitochondrial diseases, ~15% of cases, are because of mitochondrial DNA mutations. This study reported a case of an 11-month-old male infant with mitochondrial disease characteristics and subclinical hypothyroidism (a high thyrotropin level). Laboratory tests were all normal and the enzymatic activities of mitochondrial respiratory chain enzyme complexes I-IV were normal. However, thyroid tests showed abnormal results, and complex V showed a deficiency activity of 52.8% of the low limit of healthy individuals (normal activity is >60.7%). The patient experienced convulsions, and the 24-h ambulatory electroencephalography results showed abnormalities, but the electromyography results were normal. Axial brain MRI showed abnormal dysplasia over the white matter myelination in the bilateral horn of the lateral ventricle. Furthermore, DNA sequencing data showed a novel mutation at 8691A>G of the mitochondrial ATPase 6 gene. This case adds to the growing literature of mitochondrial disorders caused by mitochondrial ATPase 6 mutations.

[Clinical efficacy of oxcarbazepine suspension in children with focal epilepsy].

OBJECTIVE: To investigate the clinical efficacy and safety of oxcarbazepine (OXC) suspension in children with focal epilepsy. METHODS: A total of 118 children aged 2-14 years, who were newly diagnosed with focal epilepsy between October 2009 and December 2011, were randomly divided into experimental group (n=60) and control group (n=58). The experimental group was treated with an orally suspension of OXC and the control group was orally administered with carbamazepine (CBZ) tablets. The two treatment regimens were compared in terms of clinical efficacy and safety. RESULTS: After 13 and 26 weeks of treatment, the experimental group had response rates of 75% and 72% respectively and seizure-free rates of 53% and 50%, and the control group had response rates of 71% and 66% and seizure-free rates of 50% and 43% respectively. There were no significant differences in the clinical efficacy between the two groups (P>0.05). After 26 weeks of treatment, the adverse event rates of the experimental and control groups were 18% and 40% respectively, with a significant difference between the two groups (P<0.05). CONCLUSIONS: OXC suspension has a comparable clinical efficacy to that of CBZ tablets in children aged 2-14 years who are newly diagnosed with focal epilepsy, but OXC suspension causes fewer adverse events and has higher safety.