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Objective To summarize the clinical characteristics and RNF216 gene mutation of a patient with Gordon Holmes syndrome (GHS), and to improve the understanding of the genetic and clinica characteristics of this disease through literature review. Methods We collected the clinical data of the patient with GHS, extracted the DNA from 2 mL peripheral venous blood of the patient and his parents for whole exome gene detection, and then we analyzed the clinical and genetic characteristics of all previously reported patients with RNF216 gene mutation. Results The young male patient was short in stature at sixyearsold and was diagnosed growth hormone deficiency.He had no secondary sexual characteristics by the age of 15 and was diagnosed hypogonadal hypogonadism.After the age of 22, he gradually developed abnormal gait and had progressive decline in speech, motor, and cognitive functions.Whole exome sequencing revealed a homozygous, nonsense mutation c.1549C>T (p.R517*) in the RNF216 gene.His parents were consanguineous and were heterozygous carriers of the mutations with phenotypic normality.Combined with literature review and this case report results showed that a total of 21 patients of the disease in the world and among them 15 had pathogenic variants of RNF216 gene mutation.7 of the 15 had truncated mutations, 5 had missense mutations, and 1 synonym mutation, 1 splice mutation, and 1 deletion mutation respectively.RNF216 gene mutation can be seen in neurodegenerative diseases with multiple overlapping symptoms of GHS, Huntington-like disease, and 4H syndrome.The main clinical manifestations are hypogonadotropic hypogonadism and early-onset progressive neurological dysfunction in adolescence or early adulthood.The median age of onset of neurological symptoms is 28 years old, featuring cerebellar ataxia, dysarthria, and cognitive impairment, as well as imaging manifestations of extensive white matter lesions and cerebellar atrophy. Conclusions The mutation of RNF216 gene can cause GHS.Genetic testing is helpful to the diagnosis and treatment of rare diseases.
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Moody and colleagues discovered the presence of venous collagenosis (VC) in periventricular of human brains by pathological study obtained at autopsy in 1995, which was described as a noninflammatory collagenous thickening of venous walls resulting in severe periventricular venous stenosis or occlusion. Due to the lack of specific markers and staining methods, there are few studies of cerebral venous disease, and the pathological features and pathogenesis are still unclear. However, studies have reported that VC is associated with cerebral small vessel disease (CSVD) and Alzheimer′s disease (AD), suggesting that VC may play an important role in exploring and elucidating pathogenesis. The article aims to provide a review of researches on VC pathological features, possible pathogenesis and correlation with CSVD and AD.
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The diagnosis of cerebral small vessel disease (CSVD) is highly dependent on neuroimaging, and its imaging changes include lacune, lacunar infarction, white matter hyperintensity (WMH), perivascular space (PVS), cerebral microbleed, etc. In previous studies, the definitions of these imaging changes were quite different, resulting in misdiagnosis of lacuna, WMH and PVS. This comment will summarize the clinical, imaging, and pathological characteristics of CSVD, sort out the process and effectiveness of the gradually normalized diagnostic standards, and propose errors that should be avoided, aiming to improve the accuracy and consistency of clinical diagnosis and research.
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Paraneoplastic neurological syndromes (PNS) are heterogeneous disorders caused by autoimmune responses of cancer, which can affect any part of the nervous system. Anti-amphiphysin antibody is one of the high-risk PNS antibodies, which is usually associated with small cell lung cancer and breast cancer. However, extrapulmonary neuroendocrine carcinoma is rare in patients with anti-amphiphysin antibody. A case of anti-amphiphysin-associated paraneoplastic brainstem encephalitis with esophageal neuroendocrine carcinoma is reported. The tumor was detected by fluorine 18 fluorodeoxyglucose positron emission tomography and pathologically confirmed by gastroscopic biopsy. The patient′s neurological symptoms were partially improved after treatment of intravenous immunoglobulin and glucocorticoids. However, the disease prognosis is closely related to the accompanying tumor.
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Rare diseases refer to diseases characterized by very low prevalence rate but causing serious public health problem in the society. The diagnosis and treatment of the diseases pose great challenges.During the period of 13th Five-Year, China has accelerated the pace in all aspects targeting on policy support and scientific research in the rare diseases and on patients having the diseases. The efforts include establishing the cataglog for rare disease, introducing a series of policies to accelerate the R & D of drugs, upgrading the standardization of the diagnosis and treatment competences and so forth. In addtion, the establishment of National Rare Diseases Registry System(NRDRS) has been instrumental in knowing the burden of the diseases and enhancing the scientific research to rare diseases.
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We presented an adolescent with recurrent intracranial hemorrhage and skin lesion. The diagnosis was unclear and the treatment was difficult. Through a multidisciplinary effort type Ⅰ interferon disease was suspected and later, an interferon-stimulated gene was further detected. Considering the high morbidity and fatality rate of recurrent intracranial hemorrhage, tofacitinib and hydroxychloroquine were administered. After treatment, the livedo reticularis was significantly regressed. Unfortunately, the intracranial hemorrhage recurred due to a pre-existing cerebral aneurysm, leading to death of the patient. The diagnosis and treatment of this case highlight the importance of multidisciplinary collaboration in the diagnosis and treatment of difficult and rare diseases.
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Objective This study aimed at describing the frequency of rare variants of monogenic cerebral small vessel diseases (CSVD) in a cohort of patients with CSVD, and to explore its clinical relevance. Methods This study included CSVD patients visiting the Neurology Department of Peking Union Medical College Hospital(PUMCH) from March 2017 to January 2022, collecting their demographic and clinical information and DNA samples for whole-exome sequencing. Descriptive analysis and statistical analysis were conducted exploring the differences between monogenic CSVD-related gene mutation carriers and noncarriers. Results A total of 292 patients were included, 51.03% of whom carried one or more rare variants of monogenic CSVD-related genes. The most common rare low-frequency variants were located in the NOTCH3 gene (70 patients, 23.97%), followed by HTRA1 and COL4A1/COL4A2 (22 patients, 7.53%) respectively. Among the subgroup of patients without a family history of stroke (n=176), the frequency of rare variants was as high as 47.16%. Compared with non-carriers, the carriers were diagnosed at a younger age (58.76±13.71 vs. 63.46±13.21, P=0.003). No difference was found in phenotypes among single-SNP carriers, multiple-SNPs carriers, and noncarriers. Conclusions The frequency of rare mutation of monogenic CSVD-related genes were relatively high in Chinese CSVD cohort. The most common rare variant was within the NOTCH3, followed by HTRA1 and COL4A1/COL4A2 genes. For CSVD patients of unknown causes, genetic screening should not be neglected even if there is not a family history of the disease.
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Objective To evaluate the clinical and paraclinical features of Chinese patients with anti- LGI1 encephalitis. Methods Patients with memory deficits, psychiatric symptoms, seizures or altered level of consciousness, suspicious of encephalitis, at presentation to Peking Union Medical College Hospital were recruited between July 2013 and January 2018, and tested for anti-LGI1 antibodies in their serum and/or cerebrospinal fluid(CSF) samples. Patients with anti-LGI1 antibodies were enrolled. The demographic characteristics, clinical manifestations, laboratory examination results, neuroimaging features, immunotherapy, follow-up practices and outcomes for included patients were registered and analyzed. Results The study enrolled 120 patients, of whom 66.7% were male. The median age was 61 years (interquartile range [IQR]: 49-66 years). Seizures(65.0%) were the most common initial symptoms. Most patients developed seizures (95.0%), including faciobrachial dystonic seizures (54.2%), memory deficits (92.5%), and psychiatric symptoms (69.1%). Brain MRI and 18F-FDG PET / CT showed that the lesions were mainly located in unilateral or bilateral medial temporal lobes, and (or) basal ganglia. Of the patients, 95.0% received intravenous immunoglobulin (IVIg) or corticosteroids, 47.5% received mycophenolate mofetil as long-term immunotherapy, and no one received second-line immunotherapy. The median follow-up was 34.2 months(IQR: 22.0-45.6 months). 91.2% had a good outcome (modified Rankin Scale score≤2 points). Residual mild memory deficits were present in 47.8% of the patients. Nine deaths were documented. Relapses occurred in 24.8% of the patients in the first year. In total, 24 (20%)cases were young patients(onset age ≤45 years).There were fewer males among the younger patients(37.5% vs. 74.0%, P < 0.01). Besides, there were fewer younger patients with psychiatric symptoms(50.0% vs. 74.0%, P=0.02), hyponatremia(33.3% vs. 68.8%, P < 0.01), and abnormalities on brain 18F-FDG PET/CT(20.8% vs. 47.9%, P=0.02). The relapse-free survival rate was significantly higher in the young patients. Conclusions Elderly males were predominant in patients with anti-LGI1 encephalitis. Most patients developed symptoms of limbic encephalitis and/or FDBS during the disease course. Several patients were young adults and lacked typical symptoms. Neuroimaging features were consistent with the involvement of limbic system or basal ganglia. Patients with anti-LGI1 encephalitis respond well to immunotherapy, irrespective of the age.
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Rare diseases have been a major challenge for clinical medicine and public health challenge in China. One of the effective measures is to conduct proactive research on rare diseases to deal with the disease burden of the diseases. However, low prevalence, disperse distribution of patients, lack of knowledge about the disease course, and phenotype heterogeneity hamper the development of research for rare diseases. Recently, it has been found that patients registry is effective in understanding the course of the disease and accu- mulating the cases and data of clinical research or clinical trial design. At present, most of developed countries or regions in the world have promoted clinical research and clinical trials of new medications on rare diseases by using the registration of rare disease. In 2016, Peking Union Medical College Hospital established China's first registry system at the national level-National Rare Disease Registry System of China(NRDRS). NRDRS has accumulated 68 137 cases data registered by the researchers from China's 101 collaborating hospitals in 29 provinces/municipalities/autonomous regions, covering 171 different, and forming 188 cohorts. To date, NRDRS complete the initial stage of resources buildup.Nex stage will be focused on clinical research and clinical trials related to rare diseases based on NRDRS. This article is on the process of building NRDRS, the potential support for conducting clinical research and clinical trials related to rare diseases, and the challenges will be faced.
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The clinical diagnosis of cerebral small vessel disease (CSVD) is largely dependent on neuroimaging. Features seen on neuroimaging of CSVD include white matter hyperintensities, lacunes and lacunar stroke, microbleeds, and perivascular spaces, suggesting brain tissue injury secondary to microvascular changes. Additionally, with the rapid development of neuroimaging method, several new methods from magnetic resonance imaging (MRI), functional MRI and positron emission computed tomography are applied in the clinical diagnosis of CSVD. Therefore, unified understanding of terminology and de?nitions for imaging features of CSVD, and of protocols for image acquisition and assessment, makes a significant meaning in clinical diagnosis and research of CSVD.
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Objective:To explore whether there were changes in midbrain morphology related to motor function in patients with cerebral small vessel disease (CSVD).Methods:The study was conducted on a subset of patients with CSVD registered at the Department of Neurology of Peking Union Medical College Hospital from 2010 to 2018. All magnetic resonance imaging images were taken with a 3.0 T nuclear magnetic resonance imager. The measurement of anteroposterior diameter of the mesencephalon and the tegmentum of mesencephalon was performed on a personal computer with the image processing software RadiAnt DICOM Viewer. Several clinical manifestations of dyspraxia, dysphagia, dysarthria and dysuria were evaluated by interviewing the patient and family members living with the patient. Kinect depth camera combined with self-developed software platform was used to conduct quantitative evaluation of patients′ motor function. Three parameters, namely walking speed, walking time of three meters and time of standing up and sitting down, were selected from the obtained parameters. SPSS 17.0 software was used for statistical analysis.Results:A total of 176 patients were included, aged 30-88 (64.16±11.57) years. One hundred and fifteen patients were males, accounting for 65.34%. In patients with CSVD, anteroposterior diameter of the mesencephalon and the tegmentum of mesencephalon were negatively correlated with age ( B=-0.032, P<0.001; B=-0.020, P=0.006). The anteroposterior diameter of the mesencephalon was negatively correlated with symptoms of dyskinesia ( OR=0.006, 95% CI 0-0.135, P=0.001), even when the age and gender were adjusted ( OR=0.014,95% CI 0-0.416, P=0.013). The anteroposterior diameter of the mesencephalon was negatively correlated with symptoms of dysuria ( OR=0.046,95% CI 0.002-0.936, P=0.045), but no longer correlated when the age and gender were adjusted. The anteroposterior diameter of tegmentum of mesencephalo was also negatively correlated with symptoms of dyskinesia ( OR=0.035,95% CI 0.002-0.684, P=0.027), but no longer correlated after adjusting for age and gender. Video recording and evaluation of motor function were performed on 87 patients. Spearman correlation analysis showed that none of the three motor function scores was correlated with age or gender. The anteroposteric diameter of the midbrain was positively correlated with walking speed ( r=0.231 ,P=0.040) and negatively correlated with walking time of three meters ( r=-0.304, P=0.005), but not with standing up and sitting down time, while the anteroposteric diameter of tegmentum of mesencephalo was not correlated with all of them. Conclusion:There are changes in mesencephalon morphology in patients with CSVD, structural parameters of mesencephalon are related to motor function and urination function, and mesencephalon atrophy may be an independent related factor for symptoms of dyskinesia in patients with CSVD.
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Objective:To evaluate whether dialysis modality will affect cognitive function in dialysis population.Methods:This was a cross-sectional study. Chronic dialysis patients in our center was screened from July 2013 to July 2014. All of the subjects received brain magnetic resonance imaging (MRI) examination and comprehensive cognitive function evaluation.Results:A total of 189 chronic dialysis patients were enrolled in this study, 122 cases on hemodialysis (HD) and 67 cases on peritoneal dialysis (PD). There was no significant difference in age between HD and PD groups [(56.4±13.2) years vs (56.4±16.1) years, t=0.004, P=0.997]. The dialysis vintage and serum albumin of HD patients was higher than those of PD patients[58.0(16.8, 107.5) months vs 31.0(7.0, 67.0) months, Z=-3.490, P<0.001; (39.6±3.9) g/L vs (35.3±3.8) g/L, t=7.328, P<0.001, respectively]. The prevalence of cerebral small vessel diseases (CSVDs) was comparable between HD and PD groups (all P>0.05). Compared with HD patients, PD patients presented a 11.90-fold risk of immediate memory impairment (95% CI 1.40-101.08, P=0.023) and a 6.18-fold risk of long-delayed memory impairment (95% CI 2.12-18.05, P=0.001). After adjusting for age, educational lever, dialysis vintage, serum creatinine, and CSVDs, the influence of dialysis modality on memory still worked. PD patients presented a 43% risk of executive function impairment of HD patients ( OR=0.43, 95% CI 0.17-1.04, P=0.061). Conclusions:HD patients manifested better memory than PD patients, while PD probably performed better in executive function than HD patients. There was no significant difference in language function between the two groups. The difference in cognitive function may not be related to CSVDs.
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Objective:To explore the clinical profile and prognostic features of relapsing polychondritis (RP) with nervous system involvement.Methods:The clinical data of 28 RP patients with nervous system involvement admitted to Peking Union Medical College Hospital from December 2005 to November 2019 were collected and analyzed.Results:The median age of the patients was 50 years and 57.1% (16/28) were men. Patients with central nervous system involvement accounted for 92.9% (26/28) of the total. Common manifestations included headache (69.2%, 18/26), cognitive impairment (38.5%, 10/26), and mental disorders (38.5%, 10/26); only two cases had peripheral nervous system damage. Brain magnetic resonance imaging revealed that subcortical (13/18) and cortical (8/18) tissues were most affected. Cerebrospinal fluid tests showed that the median white blood cell count was 55×10 6/L, while the protein level was also slighted elevated ((0.54±0.17) g/L). All patients were treated with glucocorticoids, and 85.7% (24/28) of patients with immunosuppressants; 27 patients showed optimistic responses to the treatment, while 24 had recurrent courses. Cognitive dysfunction was the most common residual symptom. Follow-up data of 16 patients were available with an average follow-up time of 3.3 years. Ten of the patients were in stable condition and three patients died. Conclusions:Nervous system damage, especially limbic encephalitis and meningitis may present in course of RP. However, it is a challenge to diagnose for the non-specific clinical manifestations. Treatment with corticosteroids and immunosuppressants can lead to a favorable response.
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Objective:To characterize the digital language markers in Alzheimer′s disease (AD) and mild cognitive impairment (MCI) patients, and to explore the pathological effect and aging effect on these markers.Methods:AD ( n=14) and MCI ( n=16) patients from memory clinic in Peking Union Medical College Hospital, age-matched cognitively normal elderly adults ( n=18) and youthful adults ( n=19) as controls participated in the study. The digital speech data of animal fluency test were collected. Novel language markers such as response time, semantic similarity and word frequency were analyzed in addition to the traditional word production, clustering and switching indicators by trained professionals. Multiple linear regression analysis with multiple comparison was used to test the associations of language markers with the cognitive status, adjusting for education. Results:The results of multiple regression analysis showed that, after adjusting for the years of education, statistically significant differences existed in 11 language markers among the four groups ( P<0.001), except for the effective word production in the last 15 s, cluster size, the first word response time and the average semantic similarity. Compared to other three groups, AD group differed significantly in effective word production, effective rate, repetitive rate, effective word production in the first 15 s, the number of subcategories, single word production time and inter-subcategory switching interval ( P<0.001). In addition, compared to cognitively normal youthful group, AD group had less number of switching and shorter semantic maximum distance ( P=0.001, P<0.001); both AD and MCI groups had longer intra-subcategory switching interval ( P<0.001, P=0.001); AD, MCI and cognitively normal elderly groups had significantly higher word frequency ( P<0.001); MCI group had significantly less number of effective word production and subcategories ( P=0.002, P=0.003); both MCI and cognitively normal elderly groups had significantly longer single word production time ( P<0.001). Conclusions:The performance of AD patients on the semantic fluency test task was affected by both pathological effect and normal aging effect. Pathological indicators included effective word production, effective rate, repetition rate, effective word production in the first 15 s, the number of subcategories, inter-subcategory switching interval and single word production time. These results provide a new approach to identify the specific effects of AD dementia.
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A 78-year-old man was admitted to Peking Union Medical College Hospital with fever, weakness of lower extremities, less speech, loss of memory. Fever was relieved after antibiotic treatment, while cognitive impairment and disorder of consciousness progressed rapidly, followed by critical pulmonary infections, respiratory failure, and septic shock. Lab tests showed negative occult blood, normal serum CEA level and positive Anti-nuclear-antibody. PET-CT suggested that strong FDG uptake signals were seen at sigmoid, while bilateral frontal lobe, temporal lobe, parietal lobe, posterior cingulate gyrus showed lower metabolic activity. Colonoscopy biopsy revealed differentiated adenocarcinoma of sigmoid colon. Therefore, paraneoplastic syndrome of nervous system secondary to colon cancer was considered. Rapid and proper diagnosis and treatment were completed by multidisciplinary team including departments of neurology, gastroenterology, general surgery, ICU, rheumatology, clinical nutrition. The laparoscopic sigmoid colectomy was performed under general anesthesia. The patient finally presented with significant improvement of cognition and consciousness. Respiratory function was totally recovered.
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Objective To evaluate the efficacy and safety of mycophenolate mofetil (MMF) in antileucine-rich glioma-inactivated 1 (LGI1) encephalitis by analyzing the clinical and immunologic data of patients treated with MMF in this prospective cohort of anti-LGI1 encephalitis.Methods Patients treated with MMF for more than one year in Peking Union Medical College Hospital were included in this study.MMF was given at a dosage of 1.5-3.0 g/d in the induction period (two to four months) and 0.75-2.00 g/d in the maintenance period.All the patients were followed up regularly.Modified Rankin Scale (mRS) score evaluation,serum IgG and peripheral CD19-positive B cells,CD4-positive T cells and CD8-positive T cells testing were performed every two months.Results Fifteen patients were included in this study who received first-line immunotherapy combined with MMF.No other second-line therapy including rituximab was used.Thirteen patients responded well to MMF combined with first-line immunotherapy (a decrease in mRS score of more than 1).All 15 patients had a good outcome (i.e.,a mRS score of 0-2),including nine patients without residual symptoms (a mRS score of 0).After 12 months of MMF treatment,CD19-positive B cells were significantly decreased (median 320 (227,628) × 106/L vs 152 (105,223) × 106/L;Z=-2.028,P=0.043),while serum IgG (9.07 (6.70,11.32) g/L vs 8.35 (6.63,10.69) g/L,P=0.144)),CD4-positive T cells (1 136 (736,1 432) × 106/L vs 1 055 (802,1 072) × 106/L,P =0.866) and CD8-positive T cells (627 (413,784) × 106/L vs 568 (393,743) × 106/L,P =0.735) were not significantly changed.Three patients relapsed and were treated with additional cycle of first-line immunotherapy and increased dosage of MMF (induction dosage) resulting in remission.CD19-positive B cells were tested to be increased during the patients' relapse.No serious adverse event was noted in all these patients.Conclusions MMF is safe and effective as a long-term immunotherapy in patients with anti-LGI1 encephalitis.MMF can be used as an add-on therapy to first-line immunotherapy for autoimmune encephalitis.CD19-positive B cell count should be monitored and used as a parameter to individualize dosage of MMF.
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Objective To investigate the clinical,therapeutic and prognostic features of patients with lateral sinus stenosis and isolated intracranial hypertension,and further explore the possible mechanisms of their coexistence.Methods We retrospectively enrolled 16 patients with neurosurgery in our hospital from January 2009 to December 2016,who were clinically diagnosed as simple intracranial hypertension with bilateral or predominant lateral sinus stenosis and lateral stenting.These 16 patients were recorded surgical procedures and postoperative outcomes,and followed-up to understand the long-term prognosis of them.Results There were 14 females in the 16 patients,with an average age of (32.4 ± 10.1) years,a mean duration of (10.9 ± 7.3) months,and an average body mass index of (28.9 ± 3.6) kg/m2.In terms of clinical manifestions,majority of the patients presented with headache (n =15) and visual symptoms (n =14),and all with papilledema by fundus examination.The elevated opening cerebrospinal fluid (CSF) pressure was noticed:five cases between 25-33 cmH2O (1 cmH2O =0.098 kPa),11 cases more than 33 cmH2O.The mean pressure difference in the proximal and distal sinus of the anterior chamber was (36.3 ± 9.4) cmH2O in the range of 15-91 cmH2O.The pressure difference between the two ends of the stenosis disappeared immediately after the operation in 12 cases and the pressure difference less than 15 (2-12) cmH2O in four cases.Thirteen patients underwent lumbar puncture at one week after operation.The CSF pressure of them decreased significantly,of which eight were in the normal range.Six months after the operation,11 patients underwent DSA/MRV,none of which had serious surgical complications.With the average follow-up of (35.4 ± 9.8) months,the overall prognosis of these patients was good.Headaches in 14 of 15 patients were improved,out of which 12 were free of headache,two with only mild headache and a slight intracranial hypertension (19 and 23 cmH2O,respectively);visual complaints were reversed in nine out of 14 cases;10 patients underwent fundus examination,and nine of them were observed the improvement of papilledema.During the follow-up period,the symptoms of other patients were improved (headache relief and visual improvement) except one,the overall effective ratio being 15/16.Conclusions The interventional treatment of lateral sinus stenting is effective in patients with sinus stenosis associated with simple intracranial hypertension.The stenosis of the lateral sinus may be the main mechanism of increased intracranial pressure.
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Objective To investigate the prevalence and cardiovascular risk factors of silent brain infarct (SBI) in Shunyi Cohort.Methods This study was based on the population based Shunyi Study in China.One thousand and twenty-seven stroke-free participants older than 35 years,who completed cerebral MRI,were included.Cardiovascular risk factors were assessed by interview,physical examination and blood sample tests.SBI was evaluated on 3D-T1WI,T2WI and FLAIR sequences.Associations between risk factors and SBI were analyzed by Logistic regression and adjusted for age,sex,and relevant confounders.Results One thousand and twenty-seven participants,aged (55.9 ± 9.4) years,37.7％ male,were assessed.One hundred sixty-four participants(16.0％) had SBI on MRI.The prevalence of SBI increased by age (every 10 years,OR=2.12,95％ CI 1.74-2.58,P＜0.01).Hypertension(OR =2.67,95％ CI 1.77-4.04,P＜0.01),diabetes(OR=2.48,95％ CI 1.64-3.76,P＜0.01) and smoking(OR=1.98,95％ CI 1.08-3.62,P =0.028) were significantly associated with SBI.Conclusions The prevalence of SBI in this Chinese population is 16.0％,which increases with age.Hypertension,diabetes and smoking are associated with SBI.
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Objective To integrate research training into clinical teaching effectively, the essential steps are understanding the students` demand and mastery, factors hindering learning and how to learn effectively.MethodsStudents in 7th grade in Peking Union Medical College (PUMC), graduate students and residents under clinical training, trained doctors for advanced studies from primary hospitals were surveyed with questionnaire by collecting educational background, the demand of learning medical research methodology, factors hindering learning, specific issues need in medical research methodology, learning model and assessment approaches.Results Totally 69 people were surveyed.92.75% of the respondents said they had studied medical research methodology and related courses before.However, 51 people (73.91%) answer they cannot read literatures with critical thinking.52 people (75.37%) indicated that they had difficulty in conducting research.58 (84.06%) people said it is very necessary to study the medical research methodology.No learning opportunities and enough time were the main hinders forlearning relevant knowledge.The top three high-demanding issues are sample size calculation and statistical knowledge, study design, and how to use the statistical software.44 (63.77%) people suggest e-learning with case based learning.Conclusions Integrate medical research methodology teaching into clinical teaching is in high-demand for students.E-learning is suggested with case based learning.
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Objective Mini presentation is a new teaching model, which is performed by interns based on a typical case or disease at the medical ward.The aim of our study is to evaluate the effect of mini presentation on clinical neurologic teaching actions, according to a survey results in the interns.Methods A total of eighty-nine clinical medical students in Peking Union Medical College finally responded to the survey questionnaire, including ten questions regarding the mini presentation.Results 69.23% of the interns and 22.3% of the probationers have performed mini presentation at the neurological wards.Conclusion 43.82% of the students agreed that mini presentation was very necessary.80.9% of the students supported that mini presentation was helpful to their clinical reasoning of the neurology.ConclusionsMini presentation could motivate the independent learning interests of the students, and could improve the teaching quality in neurology.It is worth to practice and popularize further.