Restless sleep disorder was first reported in children, manifested with the large muscle groups movement during sleep named as large muscle movement. The movement of large muscle group refers to the non-periodic abnormal movement of large muscle group such as head, trunk and limbs during sleep period according to the criteria of American Sleep Association in 2022. The clinical and neuroelectrophysiological research progress of restless sleep disorder, interpretation and clinical significance of large muscles movement are summarized in this article.

Objective To study the clinical,electrophysiological,and genetic characteristics of familial cortical myoclonic tremor and epilepsy(FCMTE),and to review relevant literature for the potential pathogenic genes of the dis-ease.Methods We analyzed the clinical presentations and neuroelectrophysiological and second-generation whole exome gene sequencing results of a patient with FCMTE and her family members.Results The 72-year-old female proband com-plained of paroxysmal tremor of the upper limbs for more than 30 years and convulsions of the upper and lower limbs for 13 years.The somatosensory evoked potential test showed large potentials.Video electroencephalography detected a pattern of sharp waves with fast waves at 7-8 Hz during attacks.She was diagnosed as FCMTE.The symptoms almost disappeared after treatment with sodium valproate.The condition occurred across the three successive generations of the family.Next-generation whole exome sequencing detected a heterozygous mutation each in the exon region of the GFAP gene and KCNQ3 gene.Protein stability analysis found that the two variants had no significant effects on the spatial structures of the proteins encoded by the GFAP gene and KCNQ3 gene.Conclusion FCMTE features an autosomal dominant inheritance pattern,and presents in adulthood with tremor and/or epileptic seizures.KCNQ3 mutations may be implicated in the patho-genesis of this disease.

Objective To analyze the risk factors for iatrogenic pseudoaneurysm(PSA)occurring after cardiovascular interventional procedures.Methods The clinical data of 48 patients,who developed PSA after receiving cardiovascular interventional procedure at the Yantai Yuhuangding Hospital of China between January 2018 and December 2022,were retrospectively analyzed.The control group included 192 patients who had no PSA.At a case-control ratio of 1∶4,the PSA patients and non-PSA patients were paired,and the paired indicators included age,and puncture site.Univariate and multivariate logistic regression analyses were used to analyze the patients'basic data,hematological examination,and situation of the interventional procedure,and the independent risk factors were screened out.Results Multivariate logistic regression analysis showed that the high body mass index(BMI,OR=1.324,95％CI=1.097-1.598,P=0.003),smoking history(OR=4.477,95％CI=1.599-12.536,P=0.004),use of antiplatelet agents(OR=4.861,95％CI=1.018-23.214,P=0.047),combination use of antiplatelet and anticoagulant(OR=26.994,95％CI=2.353-309.686,P=0.008),the operator of the interventional procedure being an attending physician(OR=5.817,95％CI=1.139-29.717,P=0.034),low haemoglobin level(OR=0.946,95％CI=0.922-0.971,P＜0.01),elevated D-dimer level(OR=2.407,95％CI=1.367-4.239,P=0.002),long-time interventional operation(OR=1.019,95％CI=1.005-1.033,P=0.009),and sheath size＞6 F(OR=4.368,95％CI=1.196-15.947,P=0.026)were the independent risk factors for PSA occurring after cardiovascular interventional surgery.Conclusion High BMI,smoking history,use of antiplatelet agents,combination use of antiplatelet and anticoagulant,the operator of the interventional procedure being an attending physician,low haemoglobin level,elevated D-dimer level,long-time interventional operation,and sheath size＞6 F are the independent risk factors for PSA occurring after cardiovascular interventional procedure,which can provide a basis for the early prevention of PSA.(J Intervent Radiol,2024,33:646-650)

Innate immunity is the first line of the host cell defensing against viral infection,in which the pattern recognition re-ceptors(PRRs)such as Toll-like receptors(TLRs)and retinoic acid induces gene Ⅰ-like receptors(RLRs)play an important role.After virus infection,mitochondrial antiviral signaling protein(MAVS)in PRRs-mediated signaling pathway,which are of the com-mon linker molecule for downstream signal transmission,can receive signals transmitted by upstream TLR and RIG-Ⅰ,activate down-stream NF-κB and IRF3/7 signaling pathways leading to the activation of interferon(IFN)expression.Therefore MAVS acts as a bridge in the innate immune signaling pathway.More and more studies have shown that viruses have evolved a series of mechanism to escape the innate immune response over the long course of their evolution,and evaded the host's antiviral immune response by interfer-ing with multiple sites in the MAVS-mediated signaling pathway so as to complete its own replication and proliferation.In this paper,the role of MAVS in IFN-Ⅰ pathway and its latest research progress in the mechanism of anti-DNA viruses and anti-RNA viruses reac-tion are reviewed,providing theoretical basis for further studying the detailed mechanism of anti-virus of MAVS.

Xenogenic organ transplantation has been considered the most promising strategy in providing possible substitutes with the physiological function of the failing organs as well as solving the problem of insufficient donor sources. However, the xenograft, suffered from immune rejection and ischemia-reperfusion injury (IRI), causes massive reactive oxygen species (ROS) expression and the subsequent cell apoptosis, leading to the xenograft failure. Our previous study found a positive role of PPAR-γ in anti-inflammation through its immunomodulation effects, which inspires us to apply PPAR-γ agonist rosiglitazone (RSG) to address survival issue of xenograft with the potential to eliminate the excessive ROS. In this study, xenogenic bioroot was constructed by wrapping the dental follicle cells (DFC) with porcine extracellular matrix (pECM). The hydrogen peroxide (H₂O₂)-induced DFC was pretreated with RSG to observe its protection on the damaged biological function. Immunoflourescence staining and transmission electron microscope were used to detect the intracellular ROS level. SD rat orthotopic transplantation model and superoxide dismutase 1 (SOD1) knockout mice subcutaneous transplantation model were applied to explore the regenerative outcome of the xenograft. It showed that RSG pretreatment significantly reduced the adverse effects of H₂O₂ on DFC with decreased intracellular ROS expression and alleviated mitochondrial damage. In vivo results confirmed RSG administration substantially enhanced the host's antioxidant capacity with reduced osteoclasts formation and increased periodontal ligament-like tissue regeneration efficiency, maximumly maintaining the xenograft function. We considered that RSG preconditioning could preserve the biological properties of the transplanted stem cells under oxidative stress (OS) microenvironment and promote organ regeneration by attenuating the inflammatory reaction and OS injury.
Mice ; Humans ; Rats ; Animals ; Swine ; PPAR gamma/pharmacology* ; Reactive Oxygen Species/pharmacology* ; Heterografts ; Hydrogen Peroxide/pharmacology* ; Rats, Sprague-Dawley ; Rosiglitazone/pharmacology* ; Oxidative Stress

Objective:To investigate the clinical and electrophysiological characteristics of patients with sudden unexpected death of epilepsy (SUDEP).Methods:Using "epilepsy" as the keyword, the relevant cases entered from October 2011 to March 2012 were searched in the database of the Electroencephalogram (EEG) Monitoring Center, Xijing Hospital, the Air Force Military Medical University. Telephone follow-up was conducted for all confirmed epilepsy patients, and for the death cases confirmed by telephone follow-up, the patients identified as consistent with SUDEP diagnosis were included in this study based on their past medical history, clinical data, death details, etc, and their clinical and neuroelectrophysiological characteristics were summarized and analyzed.Results:Among the 1 232 patients who underwent 24-hour video-EEG monitoring during the study period, 354 patients were successfully followed up by telephone interview, of whom 17 patients were died (4.8%), 12 individuals met the diagnosis of SUDEP (7 men, 5 women). The duration of the disease in 9 patients exceeded 10 years. Eight cases presented with focal-bilateral tonic clonic seizures. Nine patients were treated with anti-seizure drug monotherapy. All the 24-hour video EEG of 12 patients were abnormal. There were 8 occasions when the EEG occipital α background rhythm slowed down compared with the standard frequency of peers or was dominated by slow waves. Interictal epileptic discharge (IED) located in temporal lobe were found in 12 EEG records, of which 9 EEG records were found with frontal IED. One of the 12 cases received 24-hour video EEG twice within 6 years, and his EEG background rhythm was significantly slower and the IED region was expanded compared with the first EEG record. At the third year after reexamination of EEG, SUDEP developed in this patient.Conclusions:SUDEP patients have a long course of disease and bilateral tonic-clonic seizure. The interictal EEG shows occipital slow α activity and temporofrontal epileptiform discharges, which may increase the risk of SUDEP.

OBJECTIVES: Nephrotic syndrome is a common disease of the urinary system. The aim of this study is to explore the effect of astragalus polysaccharides (APS) on multidrug resistance gene 1 (MDR1) and P-glycoprotein 170 (P-gp170) in adriamycin nephropathy rats and the underlying mechanisms. METHODS: A total of 72 male Wistar rats were divided into a control group, a model group, an APS low-dose group, an APS high-dose group, an APS+micro RNA (miR)-16 antagomir group and an APS+miR-16 antagomir control group, with 12 rats in each group. Urine protein (UP) was detected by urine analyzer, and serum cholesterol (CHOL), albumin (ALB), blood urea nitrogen (BUN), and creatinine (SCr) were detected by automatic biochemical analyzer; serum interleukin-6 (IL-6), IL-1β, tumor necrosis factor α (TNF-α) levels were detected by ELISA kit; the morphological changes of kidney tissues were observed by HE staining; the levels of miR-16 and MDR1 mRNA in kidney tissues were detected by real-time RT-PCR; the expression levels of NF-κB p65, p-NF-κB p65, and P-gp170 protein in kidney tissues were detected by Western blotting; and dual luciferase was used to verify the relationship between miR-16 and NF-κB. RESULTS: The renal tissue structure of rats in the control group was normal without inflammatory cell infiltration. The renal glomeruli of rats in the model group were mildly congested, capillary stenosis or occlusion, and inflammatory cell infiltration was obvious. The rats in the low-dose and high-dose APS groups had no obvious glomerular congestion, the proliferation of mesangial cells was significantly reduced, and the inflammatory cells were reduced. Compared with the high-dose APS group and the APS+miR-16 antagomir control group, there were more severe renal tissue structure damages in the APS + miR-16 antagomir group. Compared with the control group, the levels of UP, CHOL, BUN, SCr, IL-6, IL-1β, TNF-α, and MDR1 mRNA, and the protein levels of p-NF-κB p65 and P-gp170 in the model group were significantly increased (all P<0.05); the levels of ALB and miR-16 were significantly decreased (both P<0.05). Compared with the model group, the levels of UP, CHOL, BUN, SCr, IL-6, IL-1β, TNF-α, and MDR1 mRNA, and the protein levels of pNF-κB p65 and P-gp170 in the low-dose and high-dose APS groups were significant decreased (all P<0.05); and the levels of ALB and miR-16 were significantly increased (both P<0.05). Compared with APS+miR-16 antagomir control group, the UP, CHOL, BUN, SCr, IL-6, IL-1β, and TNF-α levels, MDR1 mRNA, and the protein levels of p-NF-κB p65 and P-gp170 were significantly increased (all P<0.05). The levels of ALB and miR-16 were significantly decreased in the APS+miR-16 antagomir group compared with the APS+miR-16 antagomir control group (both P<0.05). CONCLUSIONS APS can regulate the miR-16/NF-κB signaling pathway, thereby affecting the levels of MDR1 and P-gp170, and reducing the inflammation in the kidney tissues in the adriamycin nephropathy rats.
ATP Binding Cassette Transporter, Subfamily B, Member 1/genetics* ; Animals ; Antagomirs ; Doxorubicin/toxicity* ; Genes, MDR ; Interleukin-6/metabolism* ; Kidney Diseases/genetics* ; Male ; MicroRNAs/metabolism* ; NF-kappa B/metabolism* ; Polysaccharides/pharmacology* ; RNA, Messenger ; Rats ; Rats, Wistar ; Tumor Necrosis Factor-alpha/metabolism*

Objective:To explore the effect of Olmesartan on the antigen presenting function of dendritic cells (DCs) in rats.Methods:Bone marrow-derived dendritic cells of female Lewis rats were obtained. Bone marrow-derived dendritic cells were cultured with Olmesartan (final concentration 10 μmol/L; OLM-DCs) or equal volume of DMSO (Con-DCs). Mean fluorescence intensity (MFI) of the surface costimulatory molecule CD80, CD86 and MHCⅡ on DCs and the levels of IL-10 and TGF-β of DCs were analyzed by flow cytometry. DCs and CD4 +T lymphocytes were cocultured. T lymphocytes proliferation was analyzed by flow cytometry. IFN-γ in the supernatants were determined by ELISA. Results:The expression of MHC Ⅱ on DCs was inhibited by Olmesartan. The level of intracellular IL-10 in DCs was up-regulated by Olmesartan. Compared with Con-DCs, T lymphocytes proliferation and the level of IFN-γ were inhibited by OLM-DCs.Conclusions:Olmesartan could induce tolerogenic DCs in vitro. These tolerogenic DCs could inhibit T lymphocytes proliferation and the production of Th1 cytokine.

Objective:To explore the electro-clinical characteristics of Jeavons syndrome complicated with non-convulsive status epilepticus (NCSE).Methods:Three patients of Jeavons syndrome complicated with NCSE during electroencephalogram (EEG) monitoring in the EEG Monitoring Center, Department of Neurology, Xijing Hospital in 2018 were studied and followed up.Results:Among the three patients, there are two female patients, one male patient, aged 12 to 24 years. Generalized spike-wave discharges and impaired consciousness were recorded during video-EEG monitoring, which lasted for 8 minutes to 6.5 hours and evolved in generalized tonic clonic seizure (GTCS), and did not recur after receiving correct anti-epileptic drugs.Conclusion:NCSE can also occur in Jeavons syndrome, and NCSE in patients with Jeavons syndrome ends with GTCS, suggesting that early detection and timely termination of NCSE in patients with Jeavons syndrome may avoid the occurrence of GTCS.

Purpose: Reduced quality of life after cystectomy has made bladder preservation a popular research topic for muscle-invasive bladder cancer (MIBC). Previous research has indicated significant tumor downstaging after neoadjuvant chemotherapy (NAC). However, maximal transurethral resection of bladder tumor (TURBT) was performed before NAC to define the pathology, impacting the real evaluation of NAC. This research aimed to assess real NAC efficacy without interference from TURBT and apply combined modality therapies guided by NAC efficacy. Materials and Methods: Patients with cT2-4aN0M0 MIBC were confirmed by cystoscopic biopsy and imaging. NAC efficacy was assessed by imaging, urine cytology, and cystoscopy with multidisciplinary team discussion. Definite responders (≤ T1) underwent TURBT plus concurrent chemoradiotherapy. Incomplete responders underwent radical cystectomy or partial cystectomy if feasible. The primary endpoint was the bladder preservation rate. Results: Fifty-nine patients were enrolled, and the median age was 63 years. Patients with cT3-4 accounted for 75%. The median number of NAC cycles was three. Definite responders were 52.5%. The complete response (CR) was 10.2%, and 59.3% of patients received bladder-sparing treatments. With a median follow-up of 44.6 months, the 3-year overall survival (OS) was 72.8%. Three-year OS and relapse-free survival were 88.4% and 60.0% in the bladder-sparing group but only 74.3% and 37.5% in the cystectomy group. The evaluations of preserved bladder function were satisfactory. Conclusion After stratifying MIBC patients by NAC efficacy, definite responders achieved a satisfactory bladder-sparing rate, prognosis, and bladder function. The CR rate reflected the real NAC efficacy for MIBC. This therapy is worth verifying through multicenter research.