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We describe a Chinese family with severe autosomal-dominant nocturnal frontal lobe epilepsy (ADNFLE) and psychiatric problems in whom whole-exome family trio sequencing identified a heterozygous mutation in the potassium channel subfamily T, member 1 (KCNT1), a sodium-gated potassium channel gene, which was a novel missense mutation c.2153A>T (p. Asp718Val). The typical characteristics of the three patients in the family were refractory epilepsy, acquired cognitive impairment, and psychiatric problems, which include hallucinations and suicidal thoughts and behaviors. The age at onset was found to be earlier in son and daughter of the proband than that of the proband, as proven by the proband’s history of an epileptic seizure at the age of 16 years and her son’s and daughter’s history of seizures at the age of 8 years. Magnetic resonance imaging findings were negative for any abnormalities. Because of psychiatric symptoms, these three patients were administered risperidone at different times during their illness. The protestor’s son had tried fenofibrate treatment, but clinical remission was unclear. In summary, our findings broadened the mutation database in relation to KCNT1 and implicated the sodium-gated potassium channel complex in ADNFLE, more broadly, in the pathogenesis of focal epilepsies.

ObjectiveTo investigate the clinical significance of BCL2L10 expression and promoter methylation status in patients with myelodysplastic syndrome (MDS). Methods andnbsp;Real-time PCR was performed to detect the expression level of BCL2L10 in bone marrow specimens from 56 MDS patients and 30 non-leukemia control patients. According to the results of International Prognostic Scoring System-Revised (IPSS-R), the MDS patients were divided into low/medium risk group (IPSS-R score ≤4.5, n=33) and high-risk MDS group (IPSS-R score >4.5, n=23). Methylation specific PCR (MSP) was employed to analyze the methylation status of the promoter region of BCL2L10 gene. The relationship of the expression level of BCL2L10 with prognosis was analyzed in the subjects of these MDS patients and those from the GEPIA database. Results andnbsp;The expression of BCL2L10 was significantly lower in the MDS patients than the non-leukemia control group (P＞0.05), and the level in the high-risk MDS subgroup was obviously lower than that in the non-leukemia control group and the low/moderate risk MDS subgroup (P＞0.05). The expression of BCL2L10 was regulated by methylation. The positive rate of methylation in BCL2L10 gene promoter was remarkably higher in the high-risk MDS patients than the non-leukemia control group (56.52% vs 36.67%, P＞0.05) and the low/moderate risk subgroup (56.52% vs 30.30%, P＞0.05). The methylation status of BCL2L10 gene promoter was closely related to the risk stratification of MDS patients (Chi-square=5.79, P＞0.05). But age and sex had no such significant difference with (P＞0.05). Among the 24 patients who were not treated with methylation drugs, the 3-year survival rate of patients with high expression of BCL2L10 was lower than that of patients with low expression of BCL2L10 (83.3% vs 50.0%). For the subjects from the GEPIA database, those with lower BCL2L10 expression level had poorer prognosis than those with higher expression, but there was no statistical difference (P＞0.05). Conclusion andnbsp;The expression of BCL2L10 gene and its methylation status can be used as one of the indexes of prognosis in MDS.

The problem of robust tube-based model predictive control (MPC) is considered for a class of discrete-time constrained linear systems with time-delayed states and additional disturbances. This paper presents an active robust control scheme that can simultaneously cope with disturbances and time-delay states using active approaches, rather than inherent system robustness. The proposed robust controller design methodology is implemented by constructing a minimal robust control-invariant set of nominal linear systems with time-delayed states neglecting additional disturbances. Based on the time-delay-dependent robust MPC approach for the nominal time-delay system, a novel active robust tube-based MPC algorithm considering time-delayed states is proposed. Furthermore, a delay-dependent sufficient condition for the tube-based controller is derived. Based on the implementation of the delay-dependent robust tube-based controller, the novel active control method is much less conservative than previous approaches. Numerical simulation results demonstrate the effectiveness of the proposed method.

The industrial architectural heritage of Zhenjiang has unrepeatable technological and cultural art values. This paper fully understands and scientifically evaluates the characteristics and values of industrial architectural heritage, analyzes different sustainable utilization strategies for industrial architectural heritage under the guidance of green concept, and gives corresponding design and measures for conservation and utilization of industrial architectural heritage.

In order to solve the problems of large volume and high hardware requirements of deep convolutional neural network model in missile-borne terminal environment, a lightweight network structure based on improved dual-channel attention mechanism is constructed. Aiming at the problem that the network lightweight will sacrifice the classification accuracy, taking the MobileNetV2 lightweight network as the network basic structure, the self-designed attention module is introduced, and the MobileNetV2 network architecture based on SPP-DCAM module is designed to increase the weight of the significant feature map of convolutional layer learning, so as to improve the classification accuracy. The designed spatial information is input in parallel with channel information. By defining 1×1 and 3×3 small convolution, the computation amount and computational complexity are reduced on the basis of ensuring the lightweight of the structure. Finally, an experimental comparison is conducted on the cifar-100 image classification dataset. The results show that the classification accuracy of improved MobileNetV2 is better than that of the traditional VGG16, ResNet18 and DenseNet convoluted networks with the same number of parameters and computational complexity, and the comprehensive performance is stronger, which is more suitable for rapid classification under the condition of limited onboard computational resources.