Objective To review the clinical characteristics of left ventricular pseudo-aneurysm (LVPA) in China mainland.Methods The China National Knowledge Infrastructure (CNKI),Wanfang Data,VIP information and PubMed database were searched to extract information on clinical profile,diagnostic imaging,treatment and outcome of patients with LVPA from January 1986 to April 2012.Results Clinical data of 77 patients with LVPAs (including 5 coexistent with true aneurysm) were extracted from 65 articles.There were 56 males (72.7％) and 11 females (27.3％) with a median age of 48 years old(range 5 to 82).The most common etiology was myocardial infarction (40,51.9％).Breathlessness/dyspnea (44.6％),chest discomfort (41.9％) and chest pain (41.9％) were the most frequently reported symptoms,but asymptomatic patients accounted for 10.8％ of all.86.4％ of the patients had electrocardiographic abnormalities,most of which were nonspecific ST segment changes.No event of thromboembolism was reported.Transthoracic echocardiography was the most commonly used diagnostic imaging test,followed by angiography with which no complication was reported.19 cases of pseudo-aneurysms(24.7％)were reported to rupture and 15 patients(19.5％) died,mostly of cardiac tamponade and sudden death.Conclusions LVPA is a rare and life-threatening disorder.It lacks of specific clinical manifestations distinguishable from coronary disease or heart failure.Emergency surgery is warranted once it is identified.

Objective To investigate the lateralizing value of head deviation(HD) during complex partial seizures (CPS) in patients with refractory mesial temporal lobe epilepsy (mTLE).Methods Presurgical videotypes of 43 patients who were seizure-free for at least one year after temporal lobectomy were retrospectively reviewed.Attention was paid to the relationship between time and type of HD and the side of epileptogenic zone.Results HD was seen in 88 CPS from 43 patients who had total 206 CPS with or without secondary generalization.Both versive and non-versive HD displayed high positive predictive value (83％ (33/40) and 88％ (22/25)) for localization of an ipsilateral and contralateral seizure onset,respectively.Conclusion Both non-versive HD and versive HID during CPS in patients with mTLE are reliable lateralizing signs that can complement other diagnostic modalities in presurgical evaluation.

Objective To investigate the results and complications of hemispherotomy for drug resistant hemispheric epilepsy.Methods The authors reviewed 5 patients who were diagnosed as drug resistant hemispheric epilepsy and operated in the neurosurgery department of Peking Union Medical College Hospital from 2011 to 2013.All the 5 patients were underwent hemispherotomy after carefully multidisciplinary pre-operation evaluation.Results All patients tolerated the procedure well and the postoperative course was smooth.All the 5 patients didn`t have seizure in the period of following up of 46 to 69 months.Conclusions According to literatures and the authors` experience, hemispherotomy is as safe and efficient for hemispheric epilepsy as hemispherectomy.It is most important advance for hemispherectomy.The procedure of hemispherotomy is complex but not very difficult, illustrating a good prospect of application and extension.

Objective To find out whether conversation analysis helps to differentiate psychogenic nonepileptic seizure (PNES) from epileptic seizure in Chinese patients.Methods Twelve unselected patients from Peking Union Medical College Hospital during 2014 to 2016 with diagnostic uncertainty were included.Interactions following standard protocol were carried out.A linguist blinded to all medical data and a neurologist studied videos and transcripts of the interactions.Using a diagnostic scoring aid which includes 17 conversation features summarized from previous researches, they attempted to predict the medical diagnosis of those patients independently.Results Accurate diagnosis was predicted in 10/12 patients by both raters.Average scores of patients with epileptic seizures were 8.00 (linguist) and 6.75 (neurologist), while average scores of paitents with PNES were-5.75 (linguist) and-7.88 (neurologist).Both raters agreed on most individual items (81.86%, 167/204).To demonstrate different features between these two groups, a case comparison was made between one patient with frontal lobe epilepsy and one patient with PNES.Conclusion In Chinese patients, conversation analysis can help differentiate between epileptic seizure and PNES.

Objective To investigate the clinical characteristics and associated prognostic factors of the acute aortic syndrome. Methods The clinical data of 391 patients with acute aortic syndrome (AAS) admitted to Beijing University People's Hospital from January 2000 to December 2015 were analyzed. Results In 391 patients with AAS, the average age was (52.7 ±13.3) with a male/female ratio of 4.3:1, and 73.4％ patients had hypertension. The most common clinical manifestation of patients with AAS was pain accounting for more than 90.0％, and the nature of pain was expansible and/or transitive pain. The level of D-dimer was elevated in 91.1％ of patients with AAS (ELISA), and significantly higher in type A patients than type B patients. Ultrasound/echocardiography was used to diagnose AAS with a sensitivity of 88.4％, of which the sensitivity of type A patients was 99.1％; the mortality of type A patients was significantly higher than type B patients (34.3％ vs. 0.9％, P <0.01). The mortality was declined obviously (11.7％ vs. 28.0％, P <0.01) when patients were treated with stent. Compared with the in-hospital surviving group, the in-hospital mortality group had decreased platelet counts and FIB, higher level of D-dimer, FDP and NLR (neutrophil to lymphocyte ratio). Conclusions Transitive and(or) expansible pains were the characteristic clinical manifestations of acute aortic syndrome,which usually happened suddenly or tearing;D - dimer and ultrasound were valueble and simple methods in AAS patients; Compared with In-hospital surviving group,the In-hospital mortality group had decreased platelet counts and FIB,higher level of D-dimer, FDP and NLR.The mortality of type A was significantly higher than type B,operation can lower the mortality of AAS patients obviously.

Objective To investigate the clinical characteristics and prognosis of patients with acute aortic dissection (AAD) and hypertension,and explore other related prognostic factors in AAD.Methods The present study enrolled consecutive patients diagnosed with AAD who were admitted to Peking University People's Hospital between January 2000 to December 2015.Patients diagnosed with AAD by CT angiography,aortography or magnetic resonance imaging within 14 days of onset were included.Patients with infectious diseases,haematological diseases,malignancies,autoimmune diseases and patients without clearly clinical diagnosis or incomplete data were excluded.The patients were initially divided into two groups based on their history of hypertension,and their clinical characteristics were compared and analyzed.We further divided AAD patients into survival group and death group according to their in-patient outcomes,and factors related to their prognoses were analyzed.Logistic regression analysis was applied to analyze the independent risk factors related to hospital death in AAD patients with P＜0.05 as the significant value.Results The hypertensive group contained 237/346 cases included (68.45％),patients in this group were generally older than their non-hypertensive counterparts,accompanied by increased prevalence of comorbidities (coronary heart diseases or diabetes) and a statistical significant elevated admission blood pressures (systolic and diastolic,P＜0.05).No significant difference were found between the groups in terms of white blood cell and platelet count,D-dimer,neutrophil to lymphocyte ratio(NLR),fibrinogen,serum creatinine and serum lipid profiles (P＞0.05).Hypertensive patients were less likely to receive surgical treatment compared with those without hypertension(P＜0.05),with increased risk of in-hospital mortality (P＞0.05).Further logistic regression analysis revealed the presence of hypertension did not independently predict in-hospital mortality of AAD patients.Factors such as age,Stanford classification of the AAD,NLR and platelet counts were found to have independent predictive values for in-hospital mortality (P＜0.05).Conclusion AAD patients with hypertension are generally older,have more comorbidities such as coronary heart diseases and diabetes.The presence of hypertension itself is not directly associated with in-hospital mortality in AAD patients,while the Stanford classification,age,NLR and platelet counts are independent risk predictors.

Objective: To investigate the clinical characteristics, coagulation function and associated prognostic factors of acute aortic dissection. Methods: The clinical data of 119 patients with acute aortic dissection (AAD) admitted to Beijing University People's Hospital from November 2008 to January 2016 were analyzed. All the participants were confirmed by computed tomography angiography, and the onset time was less than 14 days. Data of blood routine test, coagulation function at the first admission were collected, and surgical intervention and prognosis were recorded. All the patients, according to the prognosis, or whether disseminated intravascular coagulation (DIC) occurred, were divided into two groups, and the differences between the two groups were compared. Logistic regression analysis was applied to analyze independent risk factors related to in-hospital death in AAD patients. Results: In 119 patients with AAD, the average age was (52.9±14.2) years, with a male/female ratio of 5.3:1. Pain was the most common clinical manifestation in patients with AAD, accounting for more than 90.0%. The nature of pain was mostly expansible and/or transitive pain. Dominant DIC occurred in 13 cases (10.9%), and 7 patients died (53.8%). There were significant differences between the DIC group and non-DIC group in neutrophil/lymphocyte ratio (NLR), platelet count, fibrinogen, D-dimer, FDP, PT, APTT and mortality rate (P<0.05). All the 16 patients in the death group were type A AAD, among which, 7 patients (43.8%) developed with DIC. There were significant differences between the death group and survival group in NLR, platelet count, fibrinogen, D-dimer, FDP, PT, APTT, DIC proportion and surgical operation rate (P<0.05).Logistic regression analysis showed that NLR and platelet count were independent risk factors of in-hospital death (P<0.05). Conclusions D-dimer has a high diagnostic and prognosis value for AAD. The mortality increased with the activation and depletion of platelet. Once DIC occurs, the prognosis is extremely poor. NLR and platelet count are independent risk factors for in-hospital death in patients with AAD.

Objective:To investigate the clinical characteristics, risk stratification, thrombolytic effects and prognosis of 110 patients with acute pulmonary embolism (PE) treated with thrombolysis.Methods:The clinical data of 110 patients with PE admitted to Beijing University People's Hospital from May 2009 to March 2019 were retrospective analyzed. The clinical data including general information, symptoms and signs, blood pressure, artery blood gas, coaglulation, and radiography were collected. Inclusion criteria: high-risk and intermediate high-risk group. Exclusion criteria: intermediate low-risk and low-risk group. According to the prognosis and risk stratification, the patients were divided into survival group and non-survival group, high-risk group and intermediate high-risk group. The indicators above were compared between with χ 2 test, t test or nonparametric test where appropriate. Results:Of the 110 patients with PE, 49 patients were male and 61 female with an average age of 65±16 years old; and 12 patients were in the high-risk group and 98 in the intermediate high-risk group. The respiratory rate of the high-risk group was higher, and blood pressure, PO 2, SaO 2 before thrombolysis were more lower than the intermediate high-risk group ( P<0.05). One hundred and nine patients were treated with systemic recombinant tissue plasminogen activator (rtPA), 70 patients with 50 mg, and 39 patients with 100 mg. One patient, who was contraindicated to systemic thrombolysis (with active vagina bleeding), was treated with interventional local thrombolysis; another 5 patients treated with interventional local thrombolysis because the clinical symptom were not improved markedly. One hundred and two patients survived and 8 patients died, among which, 3 patients were in the high-risk group and 5 in the intermediate high-risk group. The age, heart rate, respiration rate of the non-survival group were higher than those in the survival group, and the PO 2 before thrombolysis, PCO 2 after thrombolysis were lower ( P<0.05). Bleeding complication were occurred in 22 patients: 18 patients with minor bleeding, such as bleeding gums, skin ecchymosis, and 4 patients with moderate-severe bleeding, such as cerebral hemorrhage, abdominal bleeding, gastrointestinal bleeding, and vagina bleeding. Thirteen of 70 patients in the 50 mg group and 9 of 39 patients in the 100 mg group occurred bleeding complication. The bleeding complication of the low dose group was lower than that of the standard dose group ( P<0.05). Conclusions:Thrombolysis is first-line therapy to high-risk PE. Thrombolysis is safe and effective in the intermediate high-risk group with a lower incidence rate of bleeding complication.

Objective: To investigate the correlation between clinical phenotype, electroencephalogram (EEG) characteristics and genotype in children with Angelman syndrome(AS). Methods: A total of 103 children with AS at Department of Neurology, Children′s Hospital of Fudan University from June 2017 to June 2018, were included in this study.The information of clinical characteristics, EEG manifestations, genotypes as well as the epileptic outcome were collected retrospectively.The correlations between clinical phenotype, genotype, and epileptic outcome were evaluated. Results: (1) Of the 103 cases, 48 were male (46.6%) and 55 were female (53.4%). (2) Genotypes on AS critical region were maternal chromosome 15q11.2-q13 [86.4%(89/103 cases)], paternal uniparental disomy [3.9%(4/103 cases)], imprinting defects [1.9%(2/103 cases)], and mutations in the maternal copy of UBE3A [7.8%(8/103 cases)]. (3) Apparent happy demeanor or smile and general developmental delay were observed in all AS children.Dyskinesia accounted for 98.1% (101/103 cases), followed by oral movement or suck disorders [97.1%(100/103 cases)] and abnormal posture [67.0%(69/103 cases)]. The proportion of acquired small head circumfe-rence or microcephaly, flat occiput or occipital groove and wide-spaced teeth were 61.2%(63/103 cases), 85.4%(88/103 cases) and 44.7%(46/103 cases), respectively.(4) Behavioral problems like fascination with water, sleep problems and feeding difficulties were found in 86.4%(89/103 cases), 89.3%(92/103 cases) and 85.5%(88/103 cases) of the children, respectively.Sleep disorders [94.4%(84/89 cases) vs.57.1%(8/14 cases)] and feeding difficulties [93.3%(83/89 cases) vs.35.7%(5/14 cases)] were more frequently seen in children with maternal absence group, compared those with no absence, and the differences were statistically significant (all P<0.05). (5) Epilepsy was present in 77.7% (80/103 cases) of children with onset age varying from 8 to 72 months and 80.8% (59/73 cases) were developing seizures prior to 3 years old.Children with maternal absence showed more multiple seizure types than those with no absence[41.7%(32/68 cases) vs.0(0 case)], and the difference was statistically significant(P<0.05). Children with well-controlled epilepsy had more atonic seizure, compared with those with poorly controlled seizure [48.3%(14/29 cases) vs.18.5%(4/27 cases)], and the difference was statistically significant(P<0.05). Conclusions Sleep disorders, feeding difficulties in infancy and multiple seizure types are more commonly seen in AS children with maternal absence.Atonic seizure is easier to be controlled over other types of seizures.