Background: and Purpose CGG repeat expansion in the 5' untranslated region (5'UTR) of the Notch 2 N-terminal-like C gene (NOTCH2NLC) has been associated with neuronal intranuclear inclusion disease (NIID) and oculopharyngodistal myopathy type 3 (OPDM3). Few OPDM3 patients have been reported. This report describes two OPDM3 patients with novel imaging findings who presented the typical features of NIID, and reviews all OPDM3 cases available in the literature. Methods: The available clinical, imaging, and pathological information was reviewed and investigated. CGG repeat expansion in the 5'UTR of NOTCH2NLC was tested using the repeatprimed polymerase chain reaction (PCR), followed by the fluorescence amplicon-length PCR to determine the number of CGG repeats. Results: Our two OPDM3 patients and most patients reported in the literature developed the typical clinical characteristics of NIID, including leukoencephalopathy, peripheral neuropathy, cognitive deterioration, pigmentary retinopathy, ataxia, tremor, acute encephalitis-like episodes, pigmentary retinopathy, miosis, and sensorineural hearing loss. In addition to typical imaging findings of NIID, our two patients exhibited diffusion weighted imaging (DWI) hyperintensities in the middle cerebellar peduncles, which have not been described previously. Muscle biopsies revealed rimmed vacuoles and p62-positive intranuclear inclusions in the myofibers in both patients. The skin biopsy performed in one patient detected typical eosinophilic intranuclear inclusions. Genetic analysis identified CGG repeat expansion in NOTCH2NLC as the causative mutation in the two patients. Conclusions Our two patients with OPDM3 had clinical characteristics of NIID and exhibited DWI abnormality in the cerebellum. Our results indicate that OPDM3 is within the spectrum of NIID and that DWI hyperintensities in the cerebellum are helpful for diagnosing NIID or OPDM3.

Background: and Purpose CGG repeat expansion in the 5' untranslated region (5'UTR) of the Notch 2 N-terminal-like C gene (NOTCH2NLC) has been associated with neuronal intranuclear inclusion disease (NIID) and oculopharyngodistal myopathy type 3 (OPDM3). Few OPDM3 patients have been reported. This report describes two OPDM3 patients with novel imaging findings who presented the typical features of NIID, and reviews all OPDM3 cases available in the literature. Methods: The available clinical, imaging, and pathological information was reviewed and investigated. CGG repeat expansion in the 5'UTR of NOTCH2NLC was tested using the repeatprimed polymerase chain reaction (PCR), followed by the fluorescence amplicon-length PCR to determine the number of CGG repeats. Results: Our two OPDM3 patients and most patients reported in the literature developed the typical clinical characteristics of NIID, including leukoencephalopathy, peripheral neuropathy, cognitive deterioration, pigmentary retinopathy, ataxia, tremor, acute encephalitis-like episodes, pigmentary retinopathy, miosis, and sensorineural hearing loss. In addition to typical imaging findings of NIID, our two patients exhibited diffusion weighted imaging (DWI) hyperintensities in the middle cerebellar peduncles, which have not been described previously. Muscle biopsies revealed rimmed vacuoles and p62-positive intranuclear inclusions in the myofibers in both patients. The skin biopsy performed in one patient detected typical eosinophilic intranuclear inclusions. Genetic analysis identified CGG repeat expansion in NOTCH2NLC as the causative mutation in the two patients. Conclusions Our two patients with OPDM3 had clinical characteristics of NIID and exhibited DWI abnormality in the cerebellum. Our results indicate that OPDM3 is within the spectrum of NIID and that DWI hyperintensities in the cerebellum are helpful for diagnosing NIID or OPDM3.

Background: and Purpose CGG repeat expansion in the 5' untranslated region (5'UTR) of the Notch 2 N-terminal-like C gene (NOTCH2NLC) has been associated with neuronal intranuclear inclusion disease (NIID) and oculopharyngodistal myopathy type 3 (OPDM3). Few OPDM3 patients have been reported. This report describes two OPDM3 patients with novel imaging findings who presented the typical features of NIID, and reviews all OPDM3 cases available in the literature. Methods: The available clinical, imaging, and pathological information was reviewed and investigated. CGG repeat expansion in the 5'UTR of NOTCH2NLC was tested using the repeatprimed polymerase chain reaction (PCR), followed by the fluorescence amplicon-length PCR to determine the number of CGG repeats. Results: Our two OPDM3 patients and most patients reported in the literature developed the typical clinical characteristics of NIID, including leukoencephalopathy, peripheral neuropathy, cognitive deterioration, pigmentary retinopathy, ataxia, tremor, acute encephalitis-like episodes, pigmentary retinopathy, miosis, and sensorineural hearing loss. In addition to typical imaging findings of NIID, our two patients exhibited diffusion weighted imaging (DWI) hyperintensities in the middle cerebellar peduncles, which have not been described previously. Muscle biopsies revealed rimmed vacuoles and p62-positive intranuclear inclusions in the myofibers in both patients. The skin biopsy performed in one patient detected typical eosinophilic intranuclear inclusions. Genetic analysis identified CGG repeat expansion in NOTCH2NLC as the causative mutation in the two patients. Conclusions Our two patients with OPDM3 had clinical characteristics of NIID and exhibited DWI abnormality in the cerebellum. Our results indicate that OPDM3 is within the spectrum of NIID and that DWI hyperintensities in the cerebellum are helpful for diagnosing NIID or OPDM3.

Neuronal intranuclear inclusion disease (NIID) is a neurodegenerative disease, characterized by eosinophilic transparent inclusions in the central and peripheral nervous systems, and internal organs. NIID clinical characteristics are varied, including cognitive impairment, muscle weakness, episodic symptoms, movement disorders and autonomic dysfunction. This article reports a patient with NIID who manifested with episodes of aphasia, dysgraphia and dyslexia without fever, headache, nausea and vomiting confirmed by genetic testing. The patient was a 62-year-old female with acute onset who was diagnosed with transient ischemic attack. This article aims to improve the knowledge of NIID with stroke-like onset by this case presentation and avoid misdiagnosis.

Objective: Adolescents are at a special stage of physical and mental development, which is a susceptible period for mental disorders. Since the outbreak of coronavirus pneumonia in December 2019, long term stress may have negative effects on the mental health of the adolescents. In the context of the coronavirus disease 2019 (COVID-19), the study was designed to investigate the mental and psychological health of adolescents in China and its possible related factors. Methods: A cross-sectional study design was adopted using a structured questionnaire which were distributed through the Internet to measure depression, anxiety, life events and stress related factors. Descriptive statistics and multiple regression analyses were conducted to process the data. Results: The final sample comprised 795 adolescents. The total detection rate of depression was 76.48% and the total detection rate of anxiety was 33.08%. ANOVA showed that there were significant differences in depression scores in terms of gender, anxiety scores, history of mental disorders, COVID-19 knowledge reserve, family and social contradictions (p<0.05). And there were significant differences in anxiety scores in terms of gender, depression scores, mental health knowledge reserves, family and social contradictions (p<0.05). Multiple regression analysis showed that anxiety score, health status and COVID-19 knowledge reserve were positively associated with depression score (p<0.01), and history of psychosocial disorders was negatively associated with depression score (p<0.05); depression score, family and social contradictions were significantly positively correlated with anxiety score (p<0.01), and history of mental disorders was significantly negatively correlated with SDS score (p<0.01). Conclusion During the outbreak of COVID-19, adolescent students with better understanding of the pandemic, more complete knowledge of mental health, and better family and social relationship had less impact on their mental health. Therefore, to ensure a sound social support system, elaborate health instruction, and family communication and mutual understanding are conducive to alleviating the psychological stress caused by the epidemic, and it is positive for adolescent students to maintain a good mental health.

The secondary structures of hepatitis C virus (HCV) RNA and the cellular proteins that bind to them are important for modulating both translation and RNA replication. However, the sets of RNA-binding proteins involved in the regulation of HCV translation, replication and encapsidation remain unknown. Here, we identified RNA binding motif protein 24 (RBM24) as a host factor participated in HCV translation and replication. Knockdown of RBM24 reduced HCV propagation in Huh7.5.1 cells. An enhanced translation and delayed RNA synthesis during the early phase of infection was observed in RBM24 silencing cells. However, both overexpression of RBM24 and recombinant human RBM24 protein suppressed HCV IRES-mediated translation. Further analysis revealed that the assembly of the 80S ribosome on the HCV IRES was interrupted by RBM24 protein through binding to the 5'-UTR. RBM24 could also interact with HCV Core and enhance the interaction of Core and 5'-UTR, which suppresses the expression of HCV. Moreover, RBM24 enhanced the interaction between the 5'- and 3'-UTRs in the HCV genome, which probably explained its requirement in HCV genome replication. Therefore, RBM24 is a novel host factor involved in HCV replication and may function at the switch from translation to replication.
Cells, Cultured ; Hepacivirus ; genetics ; growth & development ; metabolism ; Humans ; Protein Biosynthesis ; RNA-Binding Proteins ; metabolism ; Virus Replication ; genetics

Objective To explore the severe fever with thrombocytopenia syndrome bunyavirus (SFTSV) existence time in the body,and the correlation between viral load and the severity and prognosis of disease.Methods The clinical data of 125 SFTS patients from May 2015 to October 2016 in Weihai Central Hospital in Shandong province were analyzed retrospectively.Patients were divided into low viral load group and high viral load group according to the SFTSV RNA levels.Neurological symptoms,bleeding tendency,the incidence of myocardial damage and severe pneumonia,laboratory biochemical index and prognosis of two groups were compared.SFTSV RNA of 46 cases were detected dynamically.Data with homogeneity of variance were tested by t test,and data with heterogeneity of variance was tested by rank sum test.Results Among the 125 cases,64 were male and 61 were female.The mean age was (59.0±3.6) years old.One hundred and one cases were cured,and 24 died.SFTSV RNA loads in low viral load group(81 cases) were (3.08± 1.01) copies/mL,and those in high viral load group (44 cases) were (5.69 ± 0.99) copies/mL,with statically significant difference (t =11.78,P＜0.05).By the dynamic detection of SFTSV RNA load in 46 patients,viral loads in most patients were gradually declined after 1 week of onset,and cleared after 23 days.The incidence of neurological symptoms,bleeding tendency,severe myocardial damage and pneumonia of two groups showed significant difference (x2 =92.987,38.711,75.889 and 54.680,respectively,all P＜0.05).The viral loads of patients who died varied from 1.06× 104 copies/mL to 5.78 × 107 copies/mL.White blood counts of two groups showed no significant difference (t =0.181,P＞ 0.05).The platelet counts of two groups had significant difference (t =2.869,P＜0.05).AST and γ-GT of two groups also had significant difference (P＜0.01 and 0.05,respectively).creatine kinase,creatine kinase isoenzyme,lactic dehydrogenase and hydroxybutyrate dehydrogenase of two groups all had significant difference (P＜0.01 or 0.05).Serum sodium,blood calcium and glucose of the two groups had significant difference (P＜0.01 or 0.05).activated partial thromboplastin time of the two groups showed significant difference as well (t=5.623,P＜0.01).Conclusions After the onset of SFTSV infection,the virus existence in the body may less than 4 weeks.Viral loads are closely associated with disease severity and prognosis.The higher the viral loads are,the heavier organ dysfunction could be and the higher mortality is.

Objective To investigate the evaluation value of spatiotemporal image correlation (STIC) on fetal cardiac systolic function .Methods The fetal cardiac cycle images were collected by the four-dimensional(4D) STIC ultrasound technique in 160 normal singleton pregnancies .The virtual organ computer-aided analysis(VOCAL) technique was used to measure the ventricular end-systolic volume (VESV) and ventricular end -diastolic volumes(VEDV) .The stroke volume(SV) ,ejection fraction(EF) and cardiac output(CO) were calculated .Results VESV ,VEDV together with SV and CO in normal pregnancy were increased with gestation week increase .EF remained constant with advancing gestational week .VESV ,VEDV ,SV ,CO and EF of right ventricle were all larger than those of the left ventricle .Conclusion The fetal cardiac systolic function in normal pregnancy is increased with gestational age increase ,the right heart systolic function is superior to the left heart .

Objective To investigate the changes of non-linear dynamics characteristics of electroencephalogram(EEG) in patients with the stagnation of Liver-Qi syndrome.Methods 15 Liver-Qi stagnation syndrome patients and 15 normal persons were paired by sex and age.The data of EEG under three states(eyes closed,eyes opened,and mental arithmetic with eyes opened) were analyzed by the parameter of point-wise correlation dimension(PD2).Results PD2 of the stagnation of Liver-Qi syndrome group increased significantly compared with that of the healthy control group and always maintain high level.The difference of PD2 between two groups was gradually reduced along with the increase of stimulated condition.Conclusion Non-linear dynamic analysis is more appropriate for the study of mental functions changes of stagnation of Liver-Qi syndrome and can be used to investigate the brain mechanism of the stagnation of Liver-Qi syndrome.

Objective To study the personality characteristics of the stagnation of liver-qi syndrome cases with Minnesota Multiphasic Personality Inventory (MMPI).Methods 20 liver-qi stagnation syndrome cases and 20 normal persons whose paired by sex and age were tested by MMPI. The scores of 13 clinical scales and the section plane of MMPI were acquired and analyzed. Results There was a significant difference in 9 clinical scales (F, Pa, D, Pt, Si, Sc, Hs, Hy, Pd) between the liver-qi stagnation syndrome cases and normal persons ( P<0.05～0.001). The scores of Hs, D, Hy, Pd in the liver-qi stagnation syndrome group were higher than the Chinese normal model. The MMPI section plane of liver-qi stagnation group presented the type of 3/1. Conclusion The patients with liver-qi stagnation have some special personality characteristics such as depression, anxiety, loneliness, indifferent and attention.