This comprehensive review synthesizes the latest advancements in understanding inflammatory disorders affecting cerebral small vessels, a distinct yet understudied category within cerebral small vessel diseases (SVD). Unlike classical SVD, these inflammatory conditions exhibit unique clinical presentations, imaging patterns, and pathophysiological mechanisms, posing significant diagnostic and therapeutic challenges. Highlighting their heterogeneity, this review spans primary angiitis of the central nervous system, cerebral amyloid angiopathy-related inflammation, systemic vasculitis, secondary vasculitis, and vasculitis in autoinflammatory diseases. Key discussions focus on emerging insights into immune-mediated processes, neuroimaging characteristics, and histopathological distinctions. Furthermore, this review underscores the importance of standardized diagnostic frameworks, individualized immunomodulation approaches, and novel targeted therapies to address unmet clinical demands.
Humans ; Cerebral Small Vessel Diseases/pathology* ; Inflammation/pathology* ; Cerebral Amyloid Angiopathy/pathology* ; Vasculitis, Central Nervous System/pathology* ; Vasculitis/pathology*

To develop a milestone-based evaluation system for the core "knowledge and skills" competency of neurology residents that is tailored to China's medical context, so as to provide precise guidance for their training and assessment.

To explore the effectiveness of "near peer learning" (NPL) in the electromyography(EMG)teaching module for neurology residents.

Resident training is a necessary path to cultivate excellent clinical doctors. Based on the Consensus on Core Competency Framework for Residency Education Among China Consortium of Elite Teaching Hospitals for Residency Education, Department of Neurology from Peking Union Medical College Hospital has established a training program for neurology residents with a graded evaluation system and a progressive clinical responsibility concept guided by core competency. Overcoming the teaching difficulties of neurology and considering the clinical characteristics of neurology, we have established a series of replicable and promotable neurology residency training curriculum through inheritance and innovation, which provides reference for the continuous improvement of China's neurology residency training program.

  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.

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.

Cell death plays important roles in living organisms and is a hallmark of numerous disorders such as cardiovascular diseases, sepsis and acute pancreatitis. Moreover, cell death also plays a pivotal role in the treatment of certain diseases, for example, cancer. Noninvasive visualization of cell death contributes to gained insight into diseases, development of individualized treatment plans, evaluation of treatment responses, and prediction of patient prognosis. On the other hand, cell death can also be targeted for the treatment of diseases. Although there are many ways for a cell to die, only apoptosis and necrosis have been extensively studied in terms of cell death related theranostics. This review mainly focuses on molecular imaging and therapeutic strategies directed against necrosis. Necrosis shares common morphological characteristics including the rupture of cell membrane integrity and release of cellular contents, which provide potential biomarkers for visualization of necrosis and necrosis targeted therapy. In the present review, we summarize the updated joint efforts to develop molecular imaging probes and therapeutic strategies targeting the biomarkers exposed by necrotic cells. Moreover, we also discuss the challenges in developing necrosis imaging probes and propose several biomarkers of necrosis that deserve to be explored in future imaging and therapy research.

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.

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.

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.