Objective To explore the effect of antinuclear antibody ( ANA+dsDNA),extractable nuclear antigen (ENA) and antineutrophil cytoplasmic antibody (ANCA) on the clinical manifestation and cerebrospinal fluid characteristics of neuromyelitis optica (NMO).Methods All 41 patients with NMO in PUMC hospital from 1985 to 2009 were retrospectively reviewed.All patients underwent examination of serum ANA+dsDNA,ENA and ANCA.Fourteen positive-autoantibody patients were compared with 27negative-autoantibody patients in gender,onset age,duration,relapse ratio,first demyelination event,the extent of optic neuritis and myelitis,EDSS,CSF protein,WBC,Oligoclonal band, 24 hours IgG index and myelin basic protein.Results The 14 NMO patients (34.1%) had positive non-organ-specific antibodies.NMO patients who had negative autoantibodies were compared with NMO patients with positive autoantibodies with significantly higher EDSS (the EDSS score were 4.5 and 2.5 respectively,U=92.5,P=0.008),more complete damage of spinal cord (3/14 vs 0/27, x2=6.736, P=0.0095) and tended to have higher visual Function Scale in remitting phase.There was no significant difference on the gender,onset age,duration,relapse ratio,first demyelination event.The positive-autoantibody patients had higher CSF WBC (2.0 vs 0,U=68.0,P=0.007) and tended to have lower 24 hours IgG index (-8.663 vs 0.163,U=30.0,P=0.053).There was no significant difference in CSF protein,MBP and OB.Conclusion NMO patients with positive autoantibodies have more severe intrathecal autoimmune inflammatory and disability,so they might need more intensive treatment.

Objective To explore the diagnostic value of oligoclonal band (OB) detected by isoelectric focusing (IEF) with immunoblotting in inflammatory demyelinating diseases (IDD) in nervous system.Methods Serum and cerebrospinal fluid (CSF) OB was detected by IEF with immunoblotting in 112 patients with IDD ( multiple sclerosis ( MS):n = 48;neuromyelitis optica ( NMO):n = 21:acute disseminated encephalomyelitis ( ADEM):n = 4;secondary IDD from systemic autoimmune diseases:n = 19;peripheral nervous system IDD:n =20) and 24 patients with non-inflammatory neurological disease (NIND).Results CSF-restricted OB was detected in 91.7% (44/48) of MS patients,23.8% (5/21) of NMO patients(x2nmO vs MS= 32.679),1/4 of ADEM patients (Fisher' s excact test),15.8% (3/19) of secondary IDD patients (x2secondary IDD vs MS = 37.425 ),0 of peripheral nervous system IDD patients (x2peripheral nervous system IDD vs MS =37.425) and 0 of NIND patients (x2NIND vs MS =37.425).MS patients had significantly higher percentage of patients with CSF-restricted OB ( all P ＜0.01),compared with NIND and other IDD patients.The sensitivity and specificity of OB detected by IEF with immunoblotting for MS were 91.7% and 89.8%,which were higher than that of OB detected by other methods.Identical serum and CSF OB ( mirror pattern ) was detected in 2 of 4 ADEM patients and 1 of 48 MS patients.Conclusion IEF with immunoblotting to detect OB is a reliable method of diagnosis for MS.

Objective To explore the walking ability and cognitive function changes in normal pressure hydrocephalus patients after cerebrospinal fluid ( CSF ) tap test for helping clinicians choose evaluation time and methods.Methods Twenty-seven patients with probable normal pressure hydrocephalus in Peking Union Medical College Hospital from 2013 to 2014 were included.All patients were evaluated using Minimum Mental State Examination, the Montreal Cognitive Assessment, Ability of Daily Life, and Idiopathic Normal Pressure Grade Scale, underwent 1.5 T head MRI scan and had ventriculo-peritoneal shunt after informerd consent.A lumbar tap with removal of 30 ml of CSF was performed in all patients.Evaluations included the 10 m walking time and steps, Trail Making Test A, number code and Stroop test.Those tests were performed 1 day before and 4, 8, 24, 72 hours after CSF tap test.The walking test and neuropsychological test results were compared between those before and after the CSF tap test.Correlation analysis was conducted between the normal pressure hydrocephalus featured MRI characters and CSF tap test responses including Evan′s index, callosum corpus angle, mismatch between narrowed high-convexity and medial subarachnoid spaces and enlarged Sylvian fissure associated with ventriculomegaly . Results Compared with 0 h walking time (23.56(14.00) s), the 10 m walking time on the 8 hours and 24 hours after CSF tap test, which were 19.41 ( 9.00 ) s and 19.67 ( 11.00 ) s respectively, were significantly improved ( Z values in Wilcoxon signed ranks test were -3.416 and -3.443 respectively,both P<0.01).There were no statistically significant differences on every evaluation time point.The neuropsychological tests changings were significant on 24 hours and 72 hours.Compared with 0 h neuropsychological test z scale (-10.28(21.60)), the z scale on the 24 hours and 72 hours after CSF tap test, which were -6.29 (26.72), -3.37(36.15)respectively, were significantly improved (Z values in Wilcoxon signed ranks test were -3.506,-2.701 respectively, both P<0.01).The Evan′s index, callosum corpus and the feature of mismatch between narrowed high-convexity and medial subarachnoid spaces and enlarged Sylvian fissure were not statistically correlated with the response of CSF tap test.Conclusions Walking ability in normal pressure hydrocephalus patients was improved after the CSF tap test.The Evan′s index, callosum corpus and the feature of mismatch between narrowed high-convexity and medial subarachnoid spaces and enlarged Sylvian fissure might not be correlated with the response of CSF tap test.

Objective:To assess the age-related differences in the management strategies and outcomes of patients with acute coronary syndrome (ACS) under the chest pain center model.Methods:Clinical data of 2 833 patients with ACS were enrolled in the retrospective observational registry between January 2017 and June 2019 at 11 hospitals with chest pain centers in Chengdu. The patients were divided into four groups according to their ages: < 55 years old group ( n = 569), 55-64 years old group ( n = 556), 65-74 years old group ( n = 804), ≥ 75 years old group ( n = 904). The collected data included the patients' demographic characteristics, cardiovascular risk factors, medical history, symptoms and signs of onset, experimental examination, types of ACS and the time from the symptom to the hospital (S-to-D), etc., and the clinical characteristics, management strategies, all-cause mortality in the hospital, and the incidence of major adverse cardiovascular and cerebrovascular events (MACCE) within 1 year after discharge were compared. The primary end point was the clinical outcome of ACS patients in different age groups, including all-cause deaths in the hospital and the incidence of MACCE within 1 year after discharge. The secondary end point was the proportion of ACS patients underwent percutaneous coronary intervention (PCI) in different age groups. Multivariate Logistic regression was used to analyze the risk factors of all-cause deaths in ACS patients. Kaplan-Meier curve was used to express the incidence of MACCE within 1 year after discharge in different age groups. Multivariate Cox regression was used to analyze the factors affecting the incidence of MACCE within 1 year after discharge of ACS patients. Results:As age increased, the proportion of male patients gradually decreased, and the percentages of male patients aged < 55 years old, 55-64 years old, 65-74 years old, and ≥ 75 years old were 87.2% (496/569), 77.0% (428/556), 66.4% (534/804), and 60.1% (543/904), respectively; and ACS patients combined with hypertension, diabetes, coronary heart disease, and stroke history were more common [the percentages of patients with hypertension aged < 55 years old, 55-64 years old, 65-74 years old, ≥ 75 years old were 41.3% (235/569), 52.2% (290/556), 59.7% (480/804), and 66.9% (605/904); the percentages of diabetes were 18.6% (106/569), 25.5% (142/556), 27.0% (217/804), and 28.2% (255/904); the percentages of coronary heart disease were 10.1% (57/564), 13.9% (77/555), 17.6% (141/803), and 23.7% (213/899); the percentages of stroke were 0.7% (4/564), 4.0% (22/552), 4.5% (36/801), and 8.6% (77/894)]. But the percentages of patients with a history of active smoking, typical chest pain/chest tightness and dyslipidemia were significantly reduced [the percentages of smoking history were 60.2% (340/565), 48.0% (266/554), 33.7% (270/801), and 21.7% (195/899), typical chest pain/chest tightness were 96.9% (536/553), 96.4% (516/535), 91.8% (716/780), 90.2% (776/860); the percentages of dyslipidemia were 11.2% (63/565), 9.2% (51/553), 5.7% (46/802), and 4.9% (44/896)], the time of S-to-D was significantly prolonged [minutes: 176.0 (73.5, 557.0), 194.5 (89.3, 682.3), 221.0 (98.8, 940.5), and 270.0 (115.0, 867.0)], hemoglobin (Hb) level was significantly reduced(g/L: 145.44±17.43, 135.95±19.25, 129.75±19.03, 122.19±20.55), and the incidence of non-ST-segment elevation myocardial infarction (NSTEMI) increased significantly [18.6% (106/569), 20.5% (114/556), 26.6% (214/804), 26.5% (240/904)], and the differences were statistically significant (all P < 0.05). The proportion of Killip grade Ⅲ -Ⅳ were the highest in patients aged ≥ 75 years old, 9.0% and 12.6%, respectively. Compared with the groups aged < 55 years old, 55-64 years old, and 65-74 years old, the proportion of patients aged ≥ 75 years old who underwent PCI was the lowest, and the all-cause mortality in the hospital and the incidence of 1-year MACCE of patients underwent PCI were significantly lower than those of patients underwent conservative treatment [6.0% (28/463) vs. 10.4% (45/434), 14.6% (43/294) vs. 24.3 % (55/226), both P < 0.05]. As age increased, the hospital all-cause mortality and the 1-year MACCE incidence increased (all-cause mortality rates in < 55 years old, 55-64 years old, 65-74 years old, ≥ 75 years old groups were 0.9%, 2.2%, 5.5%, 8.3%, and the 1-year MACCE incidences were 5.0%, 6.7%, 13.9%, 18.7%, both P < 0.01). The multivariate Logistic regression analysis showed that age, cardiogenic shock, ST-segment elevation myocardial infarction (STEMI), the number of vascular disease and underwent PCI were the independent risk factors of all-cause mortality [the odds ratio ( OR) and 95% confidence interval (95% CI) were 1.644 (1.356-1.993), 11.794 (7.469-18.621), 2.449 (1.419-4.227), 1.334 (1.096-1.624), 0.391 (0.247-0.619), all P < 0.001]. Cox regression analysis showed that age, STEMI, the number of vascular disease and underwent PCI were independent risk factors of the occurrence of MACCE within 1 year after discharge [hazard ratio ( HR) and 95% CI were 1.354 (1.205-1.521), 1.387 (1.003-1.916), 1.314 (1.155-1.495), 0.547 (0.402-0.745), all P < 0.05]. Conclusions:In the chest pain center model, compared with other age of ACS patients, the proportion of NSTEMI in elderly patients group aged ≥ 75 years old was higher, the proportion of PCI was lower, and the clinical outcome was worse. However, the prognosis of elderly patients receiving PCI treatment was better than the patients receiving conservative treatment.

Objective To describe the clinical, neuroimaging and genetic profiles of amyotrophic lateral sclerosis with frontotemporal lobe degeneration ( ALS-FTLD).Methods From August 2011 to May 2015, patients with FTLD or other types of neurodegenerative dementia were physically examined in detail and electromyography was performed to those with suspected dysarthria, limb atrophy or weakness.Cognitive and behavioral screenings were performed to all ALS patients.Patients with ALS-FTLD entered further analysis of neuroimaging and genetics.Results Among the 8 patients diagnosed as ALS-FTLD, 4 patients began with personality change or amnesia, while diseases in the remaining 4 cases began with limb weakness or dysarthria.Dementia type of 7 cases was behavioral variant FTLD ( bvFTD) and 1 case was diagnosed as semantic dementia.Electromyography of all the 8 patients showed diffuse neurogenic changes.Constructional neuroimaging of 6 patients showed cerebral atrophy predominantly in frontal and temporal lobes.Fluorodeoxyglucose-positron emission tomography was conducted in 5 patients, indicating hypometabolism mainly in frontal and ( or) temporal lobes.NeuroQ analysis revealed that bilateral frontal lobes were the most hypometabolic areas for ALS-FTLD.Among 4 patients who underwent genetic screening, 1 patient was C9ORF72 mutation carrier.Conclusions bvFTD is the major type of dementia in the context of ALS.Metabolic neuroimaging could assist accurate diagnosis, and it reveals that bilateral frontal lobes are the most hypometabolic areas for ALS-FTLD.C9ORF72 gene mutation is an important pathogenic mutation for ALS-FTLD, although it is rare in Chinese population.

Objective: To analyze the relationship between the risk of tuberculosis outbreaks in schools and the visit interval of index cases, so as to provide a scientific reference for predicting the risks of tuberculosis outbreak and making preventive measures. Methods: A total of 630 index cases from school tuberculosis outbreaks were studied during January, 2015 to December, 2022. Data on demographics, consultation history, etiological diagnosis, and methods of detection were collected. Restricted Cubic Splines (RCS), unconditional Logistic regression, and the receiver operating characteristic curve (ROC curve) were used for analysis. Results: The RCS fitted curve showed that the risk of a tuberculosis outbreak linearly increased when the consultation interval for etiologically negative patients exceeded 5.79 days, or for etiologically positive patients exceeded 8.37 days. After multi factor adjustment, for every additional day in the visit interval of the index case, the odds ratio ( OR ) value for a high risk outbreak was 1.10 (95% CI =1.07-1.13)( P <0.05). When analyzed by tertiles of visit intervals, compared to an interval of <14 days, the OR values (95% CI ) for high risk outbreaks in schools with intervals of 14-<28 days and ≥28 days were 10.32(3.04-35.10) and 82.58( 28.42 -239.95), respectively( P <0.01), indicating a trend of increasing outbreak risk with longer visit intervals. Based on the ROC curve analysis, the optimal threshold for predicting a high risk school tuberculosis outbreak was 23.5 days, with an area under the curve ( AUC ) of 0.93 (95% CI =0.89-0.98). Conclusion An extended visit interval of index cases is a good early warning indicator for high risk tuberculosis outbreaks in schools and could be considered a key factor in early intervention and risk control strategies.

Objective To report the clinical and paraclinical features of a case series with antiIgLON5 antibody related encephalopathy.Methods One hundred and fifty patients with sleep disorders and subacute or chronic onset of movement disorders,parkinsonism or bulbar palsy were included.The serum and cerebrospinal fluid specimens of these patients were screened for anti-IgLON5 antibody.The clinical and paraclinical features of patients with seropositive anti-IgLON5 antibody were summarized.Results Three patients with seropositive anti-IgLON5 antibody were identified,with one female and two males.The onset age ranged from 61 to 64 years.Case 1 presented with symptoms of involuntary movement,unsteady walk and insomnia;case 2 with symptoms of insomnia,sleep behavioral disorder,psychiatric behavior and dysphagia;case 3 with symptoms of insomnia,sleep behavioral disorder,dysarthria,and tremor.When examined by polysomnography,obstructive sleep apnea syndrome was revealed in cases 1 and 2,serious insomnia was found in cases 2 and 3,and sleep behavioral disorder was revealed in case 2.All three patients were positive for HLA-DQB1 * 0501,and cases 2 and 3 were positive for HLA-DRB1 * 1001.All three patients received immunotherapy and only one patient (case 1) responded well to immunotherapy with intravenous immunoglobulin,steroids and mycophenolate mofetil.Conclusions Anti-IgLON5-related encephalopathy is a rare disease with distinct clinical features of both autoimmune disorders and neurodegeneration disorders.These patients may benefit from immunotherapy.

Objective:To investigate the diagnostic accuracy of serological indicators and evaluate the diagnostic value of a new established combined serological model on identifying the minimal hepatic encephalopathy (MHE) in patients with compensated cirrhosis.Methods:This prospective multicenter study enrolled 263 compensated cirrhotic patients from 23 hospitals in 15 provinces, autonomous regions and municipalities of China between October 2021 and August 2022. Clinical data and laboratory test results were collected, and the model for end-stage liver disease (MELD) score was calculated. Ammonia level was corrected to the upper limit of normal (AMM-ULN) by the baseline blood ammonia measurements/upper limit of the normal reference value. MHE was diagnosed by combined abnormal number connection test-A and abnormal digit symbol test as suggested by Guidelines on the management of hepatic encephalopathy in cirrhosis. The patients were randomly divided (7∶3) into training set ( n=185) and validation set ( n=78) based on caret package of R language. Logistic regression was used to establish a combined model of MHE diagnosis. The diagnostic performance was evaluated by the area under the curve (AUC) of receiver operating characteristic curve, Hosmer-Lemeshow test and calibration curve. The internal verification was carried out by the Bootstrap method ( n=200). AUC comparisons were achieved using the Delong test. Results:In the training set, prevalence of MHE was 37.8% (70/185). There were statistically significant differences in AMM-ULN, albumin, platelet, alkaline phosphatase, international normalized ratio, MELD score and education between non-MHE group and MHE group (all P<0.05). Multivariate Logistic regression analysis showed that AMM-ULN [odds ratio ( OR)=1.78, 95% confidence interval ( CI) 1.05-3.14, P=0.038] and MELD score ( OR=1.11, 95% CI 1.04-1.20, P=0.002) were independent risk factors for MHE, and the AUC for predicting MHE were 0.663, 0.625, respectively. Compared with the use of blood AMM-ULN and MELD score alone, the AUC of the combined model of AMM-ULN, MELD score and education exhibited better predictive performance in determining the presence of MHE was 0.755, the specificity and sensitivity was 85.2% and 55.7%, respectively. Hosmer-Lemeshow test and calibration curve showed that the model had good calibration ( P=0.733). The AUC for internal validation of the combined model for diagnosing MHE was 0.752. In the validation set, the AUC of the combined model for diagnosing MHE was 0.794, and Hosmer-Lemeshow test showed good calibration ( P=0.841). Conclusion:Use of the combined model including AMM-ULN, MELD score and education could improve the predictive efficiency of MHE among patients with compensated cirrhosis.

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.