Objective:To investigate the clinical and electrophysiological characteristics of facial onset sensory motor neuronopathy (FOSMN) syndrome.Methods:Ten patients diagnosed with FOSMN syndrome in Peking Union Medical College Hospital from January 2012 to December 2022 were included. The clinical and electrophysiological characteristics of patients were analyzed and summarized, and the genetic testing was also performed in these patients.Results:The age of onset was (56.6±6.5) years, and the longest survival duration of disease was 10 years. All patients had numbness around the face and mouth as the first symptom and abnormal blink reflex. A total of 52 sensory nerve conduction nerves were detected, among which 2 median nerves and 2 μlnar nerves showed decreased amplitude of sensory nerve action potential. Needle electromyography showed neurogenic lesions, with both progressive and chronic denervation. Whole exome sequencing identified the heterozygous variant c.272A>C in the exon 4 of the SOD1 gene resulting in the amino acid change p.Asp90Ala in 1 patient. In all patients, the disease progressed relentlessly and eventually led to involvement of respiratory muscle. Conclusion:FOSMN syndrome is characterized by abnormal blink reflex and sometimes abnormal sensory nerve conduction may be shown on electrophysiologic testing.

{L-End}Objective To analyze the spectrum characteristics of noise hazards in the metal products industry. {L-End}Methods A total of six metal product industries were selected as research subjects using stratified sampling method. The noise intensity (A/C-weighted) and noise spectrum (Z-weighted) of workplaces and job positions were detected. The characteristics of the noise spectrum of each job position were analyzed using the difference between equivalent continuous C-weighted sound pressure level (L_Ceq)-equivalent continuous A-weighted sound pressure level (L_Aeq), the dominant frequency of the noise spectrum, and cluster analysis methods. {L-End}Results The workplace noise and job position noise of 11 main noise positions in the metal products industry were exceeded national standard, with incidence of 69.2% and 78.1%, respectively. The average of normalization of equivalent continuous A-weighted sound pressure level to a normal 40 hours working week exceeded the national standard in 100.0% of hydraulic workers, welders, and ultrasonic cleaning workers. The result of spectrum analysis showed that the noise in the metal product industry was mainly broadband. The noise of the collision welder position was classified as low-frequency broadband noise, while the noise of the painter position was classified as low-frequency narrowband noise. The noise spectrum characteristics of other positions were similar and classified into one category, all of which were broadband noise. Among them, numerical control lathe workers, welders, threaders, machinists, and cutters were exposed to high-frequency broadband noise, while press workers were exposed to mid-frequency broadband noise, and grinders, hydraulic workers, and ultrasonic cleaning workers were exposed to low-frequency broadband noise. The detection rate of binaural high-frequency hearing threshold improvement among workers was 61.7%, and there was no statistical correlation between the detection rate of binaural high-frequency hearing threshold improvement and the noise level intensity of each spectrum. {L-End}Conclusion The level of noise hazards in the metal products industry is severe. Effective engineering control measures and hearing protection measures should be implemented based on the spectral characteristics of noises.

Objective: To evaluate the value of cerebrospinal fluid markers expecially total-tau protein (T-tau), phosphorylated-tau protein (P-tau) in diagnosis and differentiation of sporadic Creutzfeldt-Jakob disease (sCJD). Methods: sCJD (according to 2009 Brain criteria, 2018 Neurology amended criteria), Alzheimer's disease (AD; the National Institute on Aging at National Institutes of Health and the Alzheimer's Association revised guidelines 2011 criteria) and other patients without cognitive impairment, matched for sex and age, in the Department of Neurology, Peking Union Medical College Hospital from 2018 to 2019 were enrolled. Twelve sCJD patients, 49 AD patients and 14 normal controls were enrolled. Cerebrospinal fluid (CSF) specimens were collected through gravity dropping directly, and further stored in -80 ℃ and disposed according to widely used standards. The levels of T-tau and P-tau were measured by ELISA. The data on electroencephalogram and neuroimaging findings of sCJD patients were recorded. Moreover, specimens of sCJD patients were sent to the Chinese Center for Disease Control and Prevention to test 14-3-3 protein and PRNP genotype. Results: Using Mann-Whitney U test, T-tau concentrations were found higher in patients with sCJD (1 211(448, 2 227) pg/ml) than in AD patients (549(314, 1 078) pg/ml; U=178, P=0.034 9), and both groups had higher T-tau than the control group (127(79, 192) pg/ml; U=20, 73, P<0.01). The level of P-tau was significantly increased in AD patients (72(58,109) pg/ml) compared to the control group (27(15, 42) pg/ml; U=82, P<0.01), but not in sCJD patients (32(24, 47) pg/ml). The T-tau/P-tau ratio was higher in sCJD patients (29.77(20.01, 54.53)) than in AD patients (7.45(4.79, 10.43); U=87, P<0.01). Twelve sCJD patients had cotical hyperintensity on diffusion weighted imaging and five had periodic three-phase waves on electroencephalogram. Nine sCJD patients, whose CSF samples were tested in the Chinese Center for Disease Control and Prevention, carried an M/M genotype at codon 129 and E/E at codon 219. Conclusion The CSF tau level and T-tau/P-tau ratio are significantly increased in sCJD, which may promote the diagnosis and differentiation of sCJD in routine clinical setting.

Identifying and comparing the chemical constituents of wild silkworm cocoon and silkworm cocoon is of great significance for understanding the domestication of silkworm. In this study, we used high temperature and high pressure and methanol-water system to extract cocoon chemical constituents. We used UHPLC-MS to identify and compare cocoon chemical constituents of wild silkworm and domestic silkworm Dazao and Haoyue strains. The cocoon metabolic fingerprints of wild silkworm and domestic silkworm Dazao and Haoyue strains were obtained by using the UHPLC-MS in the positive ion mode and negative ion mode. By annotation, we found that cocoon chemical compounds with high abundances contained amino acids, flavonoids, alkaloids, terpenes, organic acids, and lignans. PLS-DA showed that the cocoon components were significantly different among the wild silkworm and two domestic silkworm strains Dazao and Haoyue. Proline, leucine/isoleucine and phenylalanine showed significantly higher abundances in the cocoon of domestic silkworm Dazao strain than in those of wild silkworm and domestic silkworm Haoyue strain. The flavonoid secondary metabolites are abundant in the Dazao cocoon, including quercetin, isoquercetin, quercetin 3-O-sophoroside, quercetin-3-O-α-L-rhamnoside, quercetin-3-O- rutinoside, and kaempferol. The other secondary metabolites, alkaloids, terpenes and lignans, showed higher abundances in the wild silkworm cocoon than in the domestic silkworm cocoon, including neurine, candicine, pilocarpidine, artemisiifolin, eupassopin, and eudesobovatol. By exposing cocoons to UV light and observing the green fluorescence of flavonoids, we found that Dazao cocoon had the most flavonoids, and Haoyue cocoon had least flavonoids and wild silkworm cocoon had mediate flavonoids. Alkaloids and organic acids are good anti-insect and antimicrobial agents, which have high abundance in the wild silkworm cocoon and could enhance the defense ability of wild silkworm cocoon. Flavonoids are abundant in the cocoon of domestic silkworm Dazao strain, which the main factors are leading to the yellow-green cocoon of Dazao.
Animals ; Bombyx ; Chromatography, High Pressure Liquid ; Flavonoids ; Mass Spectrometry

Immune checkpoint inhibitors (ICIs) can cause adverse reactions in the nervous system, with the incidence rate ranging from 0.1% to 12%, and 80% occurring within the first 4 months of ICI application. It can cause lesions in various parts of the nervous system, including aseptic meningitis, meningoencephalitis, necrotizing encephalitis, brainstem encephalitis, transverse myelitis and other central nervous system diseases. It can also cause cranial peripheral neuropathy, multifocal radicular neuropathy, Guillain-Barre syndrome, spinal radicular neuropathy and myasthenia gravis, myopathy, etc. For these complications of the nervous system, diagnosis could be made by sufficient collection of disease manifestations combined with imaging, cerebrospinal fluid examinations, electro-encephalogram or electro myography to exclude infection or tumor progression. In the treatment of severe cases, ICIs should be discontinued and treated with high doses of glucocorticoid or gamma globulin with systemic support. After neurological adverse reactions, the prognosis of severe cases is poor.

Objective To characterize the brain microstructure changes of amyotrophic lateral sclerosis (ALS) patients with various levels of cognitive impairment as measured by diffusion tensor imaging (DTI).Methods A total of 55 ALS patients and 20 healthy controls (HC) were enrolled in the Department of Neurology of Peking Union Medical College Hospital From September 2013 to March 2017,and all participants underwent neuropsychological assessments and DTI scans.According to their cognitive performance,ALS patients were further subclassified into ALS with normal cognition (ALS-Cn,n =27),ALS with cognitive impairment (ALS-Ci,n =17) and ALS-frontotemporal dementia (ALS-FTD,n =11)subgroups.Comparisons of voxel-based and atlas-based fractional anisotropy (FA) and mean diffusivity (MD) data were conducted among the four subgroups.Results In the voxel-based analyses,the FA showed significant differences in cingulate gyms,corpus callosum,brain stem and cerebellum,and MD showed significant differences in bilateral frontal lobe,temporal lobe,cingulate gyms,corpus callosum,and cerebellum among the four subgroups.Besides,when compared to ALS-Ci,ALS-Cn and HC groups in the order,the areas of involvement were larger and differences were more significant in ALS-FTD group.In the atlas-based analyses,the FA and MD of the corticospinal tracts revealed no difference within the patients groups,but decreased FA and increased MD were found compared to HC group.The ALS-IFD patients manifested widespread white matter fiber integrity damage and microstructure impairment in the extramotor areas compared to other three groups.Conclusion The brain white matter structural patterns of ALS patients correlate with their cognitive function,and there is a gradient of alterations across the ALS-Cn,ALS-Ci and ALS-FTD continuum.

Objective: To develop a method for Simultaneous and rapid determination of 12 rodenticides including pindone, vacor, coumatetralyl, warfarin, diphacinone, coumachlor, chlorphacinon, difenacoum, brodifacoum, bromadiolone, difethialone and flocoumafen in whole blood and urine samples by high performance liquid chromatography-tandem mass (LC-MS-MS) . Methods: The whole blood samples were precipitated with acetonitrile, purified by OstroTM 96-well plate, The urine samples were extracted by acetonitrile, and then separated on a ODS column, analyzed with high-performance liquid chromatography-tandem mass spectrometry (LC-MS/MS) . The external standard calibration were tested. Results: A good linearity was observed in their respective concentration ranges of 12 rodenticides. The related coefficients were 0.993 0~0.999 8. The limit of detections were 0.05 μg/L~1.4 μg/L. The rates of recovery were 92.5%~118.0%. The relative standard deviations were between 0.8%~17.3%. Conclusion The method was simple, rapid, sensitive, accurate and suitable for simultaneous detection of the 12 rodenticides in whole blood and urine samples of intoxicated patients.

Objective To explore the correlation between old anterior cruciate ligament (ACL) injury with medial menis-cal tears and posterior slope of tibial plateau. Methods Between July 2014 and February 2016, retrospective analyzed 177 pa-tients diagnosed with old ACL injury (injured to surgery time>3 months) who has been underwent arthroscopic treatment. 93 pa-tients included in this study contained 65 male and 28 female,the average age was (32.36±4.50) years old (ranged from 21 to 44 years). All patients were diagnosed with ACL rupture and no associated medial meniscus tear by MR examination at the time of in-jury and before operation. Posterior slope angle of tibial plateau was measured via MR. The patients were divided into two groups according to the presence of medial meniscus tear by preoperative MR examination. Statistical analysis was employed to analyze the difference between the two groups of patients's age, body mass index (BMI), posterior slope angle of tibial plateau, gender and side. The patients were divided into group of posterior slope of tibial plateau ≥10° and posterior slope of tibial plateau<10° re-spectively. Statistical analysis was employed to analyze the incidence of concomitant medial meniscus tear between the two groups. Result All 93 patients in this study were followed up for the average of 8.63 ± 3.74 months. 51(51/93, 55%) patients were com-firmed of concomitant medial meniscus tear and 42 (42/93, 45%) patients without medial meniscus tear through MR and arthro-scope. There was no statistical difference between two groups in age (t=0.843, P=0.843), gender (χ2=1.027, P=0.338), BMI (t=0.568, P=0.571) and side (χ2=0.110, P=0.438). There was a certain correlation between the medial meniscal tears and posterior slope angle of tibial plateau in this group and the values were considered statistically significant ( r=0.602, P=0.000). Posterior slope angle of tibial plateau of medial meniscus tear group (10.51°±2.83°) was significantly higher than that in non medial menis-cus tear group (7.39°±4.62°). Values were considered statistically significant .71.15%(37/51) of the patients showed medial meniscus tear in the group of posterior slope of tibial plateau ≥10° , however, only 34.14%(14/42) patients indicated medial meniscus tear in group of posterior slope of tibial plateau<10°, and the difference was statistically significant(χ2=12.677,P=0.000). Con-clusion There is a certain correlation between old ACL injury with medial meniscal tears and posterior slope angle of tibial pla-teau. With the continuous increase of ACL injury time, high posterior slope angle of tibial plateau (≥10°) is more likely to increase the incidence of medial meniscus tear.

OBJECTIVE:To explore loading dose simvastatin on related indicators in PCI perioperative period of patients with acute myocardial infarction. METHODS:Data of 203 acute myocardial infarction patients undergoing emergency PCI were retro-spectively collected and divided into observation group (102 cases) and control group (101 cases) by different regimens. Control group received conventional treatment for 3 d before PCI,including orally taking Aspirin enteric-coated tablet 300 mg/d,qd + Sim-vastatin tablet 40 mg/d,qd,simvastatin 40 mg/d after surgery,qd,for 4 weeks. Observation group received Simvastatin tablet 80mg 2 h before PCI,the other treatment was the same with control group. Total cholesterol(TC),triglyceride(TG),low-density li-poprotein cholesterol (LDL-C),high-density lipoprotein cholesterol(HDL-C),IL-6,IL-10,TFN-α,plasma super-sensitive tropo-nin(TNT-HSST)level,creatine kinase isoenzyme(CKMB)level,high sensitive C-reactive protein(hs-CRP)level and transami-nase level before and 24 h after treatment were observed and the incidence of adverse reactions was recorded. Meanwhile,the inci-dence of postoperative 30 d of MACE and CIN was followed-up. RESULTS:There was no significant difference in TG,TC, LDL-C,HDL-C before and after treatment(P>0.05). Before treatment,there was no significant difference in TNF-α,IL-6,IL-10, hs-CRP,CKMB,TNT-HSST levels in 2 groups (P>0.05);after treatment,TNF-α,IL-6,hs-CRP,CKMB,TNT-HSST levels were significantly higher than before(P<0.05),and TNF-α,IL-6,hs-CRP levels in observation group was significantly lower than control group,CKMB,TNT-HSST levels were significantly higher than control group;IL-10 was significantly lower than before in 2 groups,and observation group was higher than control group,with statistical significance (P<0.05). The MACE rate and CIN rate in observation were lower than control group with statistical significance(P<0.05). And no obvious adverse reaction was found in 2 groups. CONCLUSIONS:Loading dose simvastatin in PCI perioperative period can significantly reduce patients' PCI, TNF-α,hs-CRP,CKMB,TNT-HSST levels and the incidence of cardiovascular and renal adverse reactions.

Objective:To explore the implicit attitude and explicit attitude toward death of college students,and the relationship between the two kinds of attitude.Methods:Totally 65 college studems participated in this study.The Single Category Implicit Association Test (SC-IAT,including cognition,affection and behavior) was used to exam the implicit attitude toward death of undergraduate students.The Death Attitude Profile-Revised (DAP-R,including five dimensions,i.e.,fear of death,death avoidance,death acceptance,escape acceptance,and neutral acceptance) was used to measure the explicit attitude.Results:Data of the SC-IAT indicated that the differences between the mean reaction time of the incompatible task and compatible in all three dimensions and the total SC-IAT were significant (Ps ＜0.01),and both the D scores of the total SC-IAT and three dimensions were larger than zero.The mean score of neutral acceptance was the highest in the five factors of SC-IAT.Relationships between the implicit effect of SC-IAT and the Mean scores of the DAP-R's five factors were not significant(Ps ＞ 0.05).Conclusion:The implicit attitude toward death of college students is negative,the explicit attitude toward death of college students is neutral.The implicit and explicit attitude toward death of college students is incongruent,and they are relatively independent.