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It has been suggested that asymmetric hypsarrhythmia is associated with structural etiology. We devised the Hypsarrhythmic Asymmetric Scoring Scheme (HASS) to quantify the degree of hypsarrhythmic asymmetry in a retrospective series of patients who underwent surgical treatment at our center. The present study aimed to investigate the role of HASS in predicting the postsurgical seizure outcomes.We retrospectively analyzed the records of 46 children with hypsarrhythmia who underwent resective epilepsy surgery between 2018 and 2020 and were followed up for at least 1 year after surgery. Hypsarrhythmia severity in each hemisphere was quantified and scored. The HASS score was calculated as the difference between the two hemispheres. Univariate results were submitted to logistic regression models to identify independent predictors for favorable surgical outcomes.Of the 46 patients who underwent resective surgery, Engel's class I-Ⅱ outcomes were achieved in 34 (73.9%). The Engel I-Ⅱ group had a significantly higher HASS score than the Engel Ⅲ-Ⅳ group (p0.001). Multivariate analysis showed that the HASS score was the only significant predictor of good outcomes (p = 0.011). Further receiver operating characteristic analysis showed that a threshold of 7 yielded a better seizure outcome with a sensitivity of 97.06% and specificity of 83.33%.As the first hypsarrhythmia scoring system specially designed for presurgical evaluation, the HASS score may contribute to predicting the postsurgical seizure outcome from the electroencephalography perspective.

PURPOSE To evaluate the retention rate, efficacy, and safety of ketogenic diet therapy for drug-resistant epilepsy in children and compare the results with those of a previous cohort at our institution. METHODS A total of 634 children with drug-resistant epilepsy were included in this retrospective study. Patients were categorized into two groups. The previous cohort was included as a control group and included 317 children assessed between 2004 and 2011, whereas the current group included 317 children assessed between 2015 and 2019. The control group was provided care as usual, and the current group additionally adopted the goal and long-term management strategy. Outcomes were measured with respect to retention rate, seizure reduction, and adverse reaction. RESULTS Patient demographics were consistent between both cohorts. Compared to the past ten years, the retention rate significantly increased over time (3 months: 62.8% vs. 82.0%, p

Myelin oligodendrocyte glycoprotein (MOG) antibodies mediate inflammatory demyelinating diseases of the central nervous system. This study aimed to understand the clinical characteristics of MOG antibody-associated aseptic meningitis (MOGAM).Here, we report the cases of two children with MOGAM. A systematic literature review was conducted and included patients who had MOGAM only, without neurological parenchymal lesions. The clinical characteristics that may have affected the outcome were statistically analyzed.We reviewed 12 cases of MOGAM; male: female = 9: 3. Prolonged fever lasting over 7 days (11/12) was the most frequent symptom, followed by headache (10/12), vomiting (5/12), and seizures (4/12). None of the patients had focal neurological manifestations or parenchymal lesions on imaging. Cerebrospinal fluid (CSF) leukocytosis was observed in all patients (12/12), and blood leukocytosis and elevated CSF pressure was observed in all patients who had corresponding results (9/9 and 4/4, respectively). Seizures occurrence was lower than that of MOG antibody-associated cortical encephalitis. Seven cases progressed to other MOG antibody-associated diseases (MOGADs) in the later phase of MOGAM. Patients who did not progress to other MOGADs had a shorter disease duration from onset to the initiation of intravenous methylprednisolone than those who did. All the patients achieved full recovery after steroid treatment. One patient had relapses.MOGAM without inflammatory demyelination is a rare but distinct phenotype of MOGAD, with fewer clinical manifestations mimicking bacterial or viral meningitis/encephalomeningitis. Delayed diagnosis and treatment may induce the progression to other severe MOGADs. Early recognition of this unique autoimmune aseptic meningitis may contribute to early diagnosis, treatment, and better outcomes.

Background: Vagal nerve stimulation (VNS) may control seizures of pediatric intractable epilepsy (PIE) as a clinically adjunctive therapy. However, high-evidence level data for younger children are needed. Methods: In this multicenter, double-blind, randomized controlled trial (VNS-PIE), we recruited children aged 3-6 years with PIE from eleven hospitals specialized in epilepsy in China. Enrolled children underwent computer-generated randomization at 1:1 ratio. Within first 12-week double-blind period, children in stimulation group received active electrical stimulation while others in control group received sham treatment. Children, their parents and investigators assessing outcomes were masked to random allocation; unmasked investigators were responsible for stimulation parameter programming. After unblinding, all children received active electrical stimulation for the subsequent 12 weeks. The primary outcome was the reduction percentage of seizure frequency between two groups at the end of the double-blind period. Findings: Between Sep 29, 2017, and May 29, 2018, 93 children were enrolled and randomly assigned to 47(51%) in stimulation group and 46(49%) in control group (Dropout: one (2%) in stimulation group). At the end of the double-blind period, the median reduction in seizure from baseline was 40·0%(IQR:[0, 71·7%]) in stimulation group, compared to 21·2%(IQR:[-15·9, 68·0%]) in control group (Adjusted median difference(AMD), 12·6%; 95% CI, 10·3% to 34·7%; p=0·23; n=93, FAS); Between-group difference of seizure decrease significantly favored electrical stimulation with respect to subgroup analysis without surgery honeymoon effect (AMD: 21·6 %; 95% CI, -4·2% to 43·3%; p=0·043; n=78, PPS). There was no significant difference in the efficacy of mental development on Gesell scale. Incision infection was common in 9 severe adverse events and cough was most frequent in 41 adverse events. Interpretation: The results from this trial of VNS supported its clinical use for younger children with PIE. Trial Registration: This trial was registered with ClinicalTrials.gov, number NCT03062514. Funding: Pins Medical Ltd; NKRDPC; NSFC; SICRP. Declaration of Interests: ZY, JD, XYQ, TYC, XF, YY and ZYW reports grants from The National Key Research and Development Program of China, grants from National Natural Science Foundation of China, grants from Shenzhen International Cooperative Research Project during the conduct of the study. TYJ, QZL, JXL, FY, YHC, LPZ, BML, YXG, XMS, SPH, FG, JML, SFL, JP, SWS, JW, CC, WXS, MQT, LY, YH, YPH, YW, HX, LXC and YWJ reports institution remunerated for recruitment of patients from Pins Medical during the conduct of the study. HWH reports grants from The National Key Research and Development Program of China, grants from National Natural Science Foundation of China, grants from Shenzhen International Cooperative Research Project during the conduct of the study; grants for advisory broad outside the submitted work. LML reports grants from The National Key Research and Development Program of China, grants from National Natural Science Foundation of China, grants from Shenzhen International Cooperative Research Project during the conduct of the study; grants for neuromodulation research outside the submitted work. Ethics Approval Statement: The trial was approved by each ethical committee of participating hospitals (Trial protocol number: G112L31101; version date: 7/31/2017) and was performed in accordance with the principles of the Declaration of Helsinki.

The ketogenic diet (KD) is an effective treatment for intractable epilepsy (IE), however the therapeutic mechanism is still unclear. This study was designed to investigate T helper type 17/regulatory T cell (Th17/Treg) levels in children with IE and age-matched healthy controls following KD.Circulating levels of Th17/Treg cells were analyzed by flow cytometry. Plasma concentration of interleukin (IL)-17 was measured by cytometric bead array assay. Real-time PCR was performed to measure mRNA levels of mTOR, HIF1α and Th17/Treg associated factors in purified CD4(+)CD25(+) T and CD4(+)CD25(-) T cells.By one-way ANOVA, the proportion of circulating Th17 cells and expression of IL-17A and RORγt were significantly higher (P.05), while the proportion of circulating Tregs and expression of Foxp3, GITR, CTLA-4 were significantly lower (P.05) in IE patients than healthy subjects. However, these alternations were reversed following KD (P.05). In CD4(+)CD25(+) T and CD4(+)CD25(-) T cells mTOR and HIF1α expression were significantly higher in IE patients (P.05), however KD reduced mTOR and HIF1α expression (P.05). The plasma IL-17A concentrations were higher in IE patients than controls (P.05). KD partially reduced IL-17A levels (P.05).Our results suggest that Th17/Treg imbalance is characteristic of childhood IE, and may contribute to IE pathogenesis. KD treatment is able to correct this imbalance, probably via inhabiting the mTOR/HIF-1α signaling pathway.