
Primary angiitis of the central nervous system (PACNS) is a rare immune-inflammatory disease confined to the small and medium blood vessels of the central nervous system, with unclear etiology and pathogenesis. The incidence of PACNS in the general population has not been accurately defined, and its clinical management is challenged by diagnostic difficulty, a high recurrence rate,and limited evidence supporting therapeutic interventions. The disease predominantly occurs in people aged 40‒60 years,with headache, cognitive impairment, and neurological deficits as the main clinical manifestations, which are non-specific.There are no characteristic indicators in laboratory tests, and cerebrospinal fluid examination can only rule out infection.Imaging findings include multiple infarctions and segmental vascular stenosis. Brain tissue biopsy is the gold standard for diagnosis. Clinically, the Calabrese and Mallek diagnostic criteria are often used, combined with supplementary criteria for stratified diagnosis. Treatment is based on glucocorticoids, with stratified induction, maintenance, and intensive therapy according to the type of involved blood vessels and disease severity, combined with immunosuppressants such as cyclophosphamide and mycophenolate mofetil. Biologics may be considered for refractory or recurrent cases.Long-term immunosuppressive therapy may reduce the risk of recurrence. Antiplatelet drugs, thrombolysis, and endovascular therapy can be used as needed. Currently, the diagnosis and treatment of PACNS still rely on diagnosis of exclusion and expert consensus,lacking high-level evidence.Future efforts should focus on exploring specific diagnostic markers and precise treatment regimens to promote standardized diagnosis and treatment.
Minor stroke is a common subtype of acute ischemic stroke, primarily defined by a National Institutes of Health Stroke Scale score ≤3 or ≤5. Despite mild clinical symptoms, it carries a risk of early deterioration, and its intravenous thrombolysis strategy has long been controversial. This article systematically summarizes the results of key randomized controlled trials in recent years, which found that although non-inferiority has been confirmed among thrombolytic agents such as alteplase, tenecteplase, and prourokinase, none of these have shown superior functional improvement in the treatment of minor stroke compared with dual antiplatelet therapy or aspirin monotherapy, and they carry safety risks such as symptomatic intracranial hemorrhage. Subgroup analyses suggested potential benefits for patients with a National Institutes of Health Stroke Scale score>3, disabling symptoms, or the large artery atherosclerosis subtype, but the evidence is inconsistent. The limited net benefit of thrombolysis is primarily attributed to heterogeneous definitions, a generally favorable natural prognosis, and the offsetting effect of bleeding risks. Future studies should focus on unifying definitions, leveraging multimodal imaging for precise patient selection, optimizing drug regimens, and ultimately achieving precise stratification and individualized thrombolytic interventions for minor stroke.
Objective To systematically summarize the pharmacological characteristics and clinical applications of antithrombotic medications in ischemic stroke, as well as their interactions with drugs used for common comorbidities, and to provide a reference for clinical medication. Methods Based on the UpToDate database and the integration with pharmacokinetic and pharmacodynamic mechanisms, this study systematically analyzed interactions between antithrombotic agents and between antithrombotics and drugs for comorbid conditions. Interactions were categorized and evaluated based on the level of evidence. Results The distinct pharmacological mechanisms of different antithrombotic drugs determine their patterns of drug-drug interactions. Combination antithrombotic therapy produces synergistic antithrombotic effects but increases the risk of hemorrhage. Extensive interactions exist when antithrombotics are co-administered with medications for cardiovascular diseases, metabolic disorders, neuropsychiatric conditions, and infectious diseases. These interactions primarily involve the cytochrome P450 enzyme system and P-glycoprotein pathways, which may compromise therapeutic efficacy or elevate the risk of bleeding and thrombosis. Conclusion Multiple drug interactions are present in antithrombotic therapy for ischemic stroke. In clinical practice, it is essential to consider the impact of pharmacokinetic and pharmacodynamic mechanisms. By integrating tools such as pharmacogenomics and therapeutic drug monitoring, individualized medication strategies should be implemented to achieve an optimal balance between antithrombotic efficacy and safety.
Objective To investigate the effect of remote ischemic postconditioning (RIPostC) on the serum levels of matrix metallopeptidase-9 (MMP-9), hypoxia-inducible factor 1α (HIF-1α), and interleukin-1β (IL-1β) in patients with acute ischemic stroke (AIS), and to further clarify the mechanism and clinical significance of RIPostC in the treatment of AIS. Methods A total of 115 AIS patients who were admitted to Suzhou First People’s Hospital from May 2024 to February 2025 were enrolled, among whom 60 patients receiving conventional treatment were enrolled as control group, and 55 patients receiving RIPostC combined with conventional treatment for 7 consecutive days were enrolled as experimental group. ELISA was used to measure the serum levels of MMP-9, HIF-1α, and IL-1β, and National Institutes of Health Stroke Scale (NIHSS) was used to assess neurological function. Results After treatment, the experimental group had a significant increase in the level of HIF-1α (P<0.001) and significant reductions in the levels of MMP-9 and IL-1β (P<0.001). The experimental group had a significantly better NIHSS score than the control group (P<0.001). The increase in the serum level of HIF-1α could promote neurological function recovery. Conclusion RIPostC combined with conventional treatment can promote early neurological recovery in AIS patients, possibly by regulating the HIF-1α signaling pathway.
Objective To systematically evaluate the effect of Xingnaojing injection on the levels of inflammatory factors in patients with acute ischemic stroke (AIS) as an adjuvant therapy for intravenous thrombolysis with alteplase, and to investigate the neuroprotective significance of Xingnaojing injection. Methods Chinese databases (CNKI, Wanfang Data, and VIP) and English databases (PubMed, Embase, and the Cochrane Library) were searched for studies on the serum levels of inflammatory factors in the treatment of AIS published up to August 2024, and the patients were divided into control group and experimental group based on the method of randomized controlled trial (RCT). The patients in the control group received intravenous thrombolysis with alteplase, while those in the experimental group received intravenous thrombolysis with alteplase combined with Xingnaojing injection. The Cochrane Collaboration Risk of Bias Tool was used to assess the methodological quality of the studies included for analysis, and RevMan 5.3 software was used to conduct a meta-analysis on NIHSS, response rate, C-reactive protein (CRP), TNF-α, IL-6, IL-8, IL-1, and adverse reactions for the experimental group and the control group. Results A total of 19 RCTs were included, involving 1 711 patients (856 in the experimental group and 855 in the control group). The meta-analysis showed that there were significant differences between the two groups in NIHSS (SMD=-2.12,95%CI -2.74 to -1.50, P<0.001), response rate (OR=5.22, 95%CI 3.44‒7.91, P<0.001), CRP (SMD=-1.27, 95%CI -1.74 to -0.79, P<0.001),TNF-α(SMD=-2.91, 95%CI -3.74 to -2.08, P<0.001), IL-6 (SMD=-6.74, 95%CI -9.06 to -4.41, P<0.001),IL-8 (SMD=-4.85,95%CI -6.49 to -3.20, P<0.001), IL-1 (SMD=-4.89,95%CI -6.86 to -2.92, P<0.001), and adverse reactions (OR=0.41, 95%CI 0.26-0.64, P<0.001). Conclusion As an adjuvant therapy for thrombolysis with alteplase, Xingnaojing injection significantly reduces the levels of inflammatory factors in AIS patients and exerts a neuroprotective effect on brain cells, with a high response rate and few adverse reactions, which provides a safe and reliable reference for clinical practice.
Objective To investigate the serum levels of basic helix-loop-helix transcription factor 40 (BHLHE40), serine protease inhibitor family E member 1 (SERPINE1), and biliverdin reductase B (BLVRB) in patients with acute cerebral infarction (ACI) and their association with the degree of carotid atherosclerosis (CAS) and short-term prognosis. Methods A total of 112 ACI patients who were admitted to our hospital from April 2023 to August 2024 were enrolled as study group, and 110 healthy individuals who underwent physical examination in our hospital were enrolled as control group. According to intima-media thickness (IMT), the patients in the study group were further divided into normal group with 22 patients, thickening group with 38 patients, and plaque group with 52 patients; based on the results of prognostic evaluation, the patients in the study group were divided into good prognosis group with 68 patients and poor prognosis group with 44 patients. ELISA was used to measure the expression levels of BHLHE40, SERPINE1, and BLVRB in the serum of ACI patients. Multivariate logistic regression analysis of risk factors for poor prognosis in ACI; ROC curve analysis of the predictive value of BHLHE40, SERPINE1, and BLVRB. Results Compared with the control group, the study group had significant increases in the serum levels of BHLHE40, SERPINE1, and BLVRB (P<0.05). Compared with the normal group, the thickening group and the plaque group had significant increases in the serum levels of BHLHE40, SERPINE1, and BLVRB, and the plaque group had significantly higher levels than the thickening group (P<0.05). Compared with the good prognosis group, the poor prognosis group had significant increases in the serum levels of BHLHE40, SERPINE1, and BLVRB and a significant increase in NIHSS score (P<0.05). The area under the curve (AUC) for the combined prediction of poor prognosis in ACI using BHLHE40, SERPINE1, and BLVRB was 0.939; the AUC for the combined prediction was superior to that of individual predictions (Z=3.804, 3.197, 3.240, P<0.05). Conclusion Elevated serum levels of BHLHE40,SERPINE1, and BLVRB in ACI patients are associated with disease severity and prognosis; the combined use of these three markers has some value in predicting poor outcomes in ACI patients.
Objective To investigate the association of blood pressure variability (BPV) with early neurological deterioration (END) and prognosis in patients with acute mild non-disabling ischemic stroke. Methods The patients with acute mild non-disabling ischemic stroke who were admitted to Stroke Center of Affiliated Hospital of Inner Mongolia University for Nationalities from January 2022 to August 2024 were enrolled, and according to the presence or absence of END, they were divided into non-END group and END group. General information, clinical data, and outcome measures were collected from all patients, and a binary logistic regression analysis was performed to determine independent risk factors for END. According to the modified Rankin Scale (mRS) score, the patients were divided into good prognosis group (mRS score ≤2 points) and poor prognosis group (mRS score >2 points), and a univariate logistic regression analysis was performed to investigate the influence of END on the 90-day neurological function prognosis of patients. Results A total of 61 patients were enrolled, with 14 patients in the END group and 47 patients in the non-END group. There was a significant difference in the coefficient of systolic pressure variation between the END group and the non-END group (P<0.05). The binary logistic regression analysis showed that systolic BPV(BPV>75%) was an independent risk factor for END (OR=14.000, 95%CI 1.471‒133.233,P=0.011).Compared with the non-END group, the END group had a significantly higher proportion of patients with a poor 90-day prognosis.Of all patients, there were 11 patients with poor prognosis and 50 patients with good prognosis on day 90. The univariate logistic regression analysis showed that END (OR=19.556,95%CI 4.038‒94.696,P<0.001) was an independent risk factor for poor prognosis. Conclusion In patients with acute mild non-disabling ischemic stroke, the increase in coefficient of systolic pressure variation is an independent risk factor for END and affects the prognosis of neurological function on day 90.
Objective To investigate the association between stress hyperglycemia ratio (SHR) and post-stroke cognitive impairment (PSCI) in patients with large-artery atherosclerotic cerebral infarction. Methods The patients with large-artery atherosclerotic cerebral infarction who were consecutively admitted to Beijing Jingmei Group General Hospital from July 1, 2023 to June 30, 2024 were selected, and a total of 1 222 patients were included for analysis based on the inclusion and exclusion criteria. According to the tertiles of SHR, the patients were divided into low SHR group (L-SHR group, 408 patients with an SHR of 0.175-0.764), middle SHR group (M-SHR group, 407 patients with an SHR of 0.765-0.905), and high-SHR group (H-SHR group, 407 patients with an SHR of 0.906-2.760). A univariate analysis was used for comparison of related indicators between two groups, and the univariate and multivariate logistic regression analyses were used to identify the independent influencing factors for PSCI; a restricted cubic spline analysis was used to clarify the association between SHR and PSCI, and a threshold effect analysis was performed. Results The incidence rate of PSCI was 16.3% (199/1 222) in all patients undergoing follow-up. The H-SHR group had significant increases in age, MAP, NIHSS score on admission, MRS score at discharge, and the incidence rate of PSCI and significant reductions in MMSE score and the proportion of patients with a high education level (all P<0.05). After adjustment for related factors, high SHR (OR=5.19, 95%CI 3.15-8.55, P<0.001) was an independent risk factor for PSCI in addition to old age, a high level of low-density lipoprotein, a high level of homocysteine, a high level of serum creatinine, a history of diabetes, and a high MRS score at discharge. There was an “inverted V-shaped” nonlinear relationship between SHR and the incidence rate of PSCI (Pfor overall<0.001, Pfor nonlinear<0.001), and there was a threshold effect in the relationship between them (Pfor likelihood test<0.001),; specifically, when SHR was less than 1.28, there was a positive association between SHR and PSCI (OR=93.26,95%CI 27.23-319.42), and when SHR was higher than 1.28, there was a negative association between SHR and PSCI (OR=0.08,95%CI 0.02-0.41). Conclusion There is a relatively high incidence rate of PSCI among patients with large-artery atherosclerotic cerebral infarction, and there is an “inverted V-shaped” nonlinear relationship between SHR and the onset of PSCI.
Objective To investigate the clinical features of patients with neuromyelitis optica spectrum disorders (NMOSD) mainly manifesting as somnolence, and to improve the awareness of this disease among clinicians. Methods A retrospective analysis was performed for the medical records of nine patients with NMOSD who were admitted to Xuanwu Hospital, Capital Medical University, from May 2021 to May 2024 and had the main clinical manifestation of somnolence, including general information, cranial and/or spinal MRI findings, polysomnography (PSG) results, cerebrospinal fluid (CSF) routine parameters, aquaporin-4 (AQP4) antibody, the level of orexin in CSF, treatment response, and prognosis. Results The age of onset in the nine patients ranged from 18 to 62 years, with a median of 30 (24,47) years, and the male/female ratio was 1∶8. Among the nine patients, six had the initial symptom of somnolence, three developed somnolence after other symptoms, and five had the manifestations of other diencephalic syndromes. All nine patients were tested for AQP4 in CSF and serum and were found positive for AQP4 antibody in CSF and/or serum. All nine patients had abnormal signals in both thalami and para-third ventricles on cranial MRI. Three patients underwent PSG and were found to have a shortened sleep latency and sleep architecture disorder, among whom one had the electrophysiological manifestation of narcolepsy and one had the manifestation of hypoventilation in severe sleep apnea. The level of orexin in CSF was measured for two patients, and the results showed that the level of orexin was lower than the normal level in both patients. All nine patients received glucocorticoid pulse therapy, and some patients were given intravenous immunoglobulin and/or subsequent sequential immunotherapy. All patients had a significant improvement in the symptom of somnolence at discharge. Conclusion In the core symptoms of NMOSD, acute diencephalic syndrome manifesting as somnolence is relatively rare in clinical practice and is easily misdiagnosed in the early stage, and the possibility of NMOSD should be considered for unexplained somnolence. Imaging examination and AQP4 antibody detection are of significant value for the diagnosis of this disease, and sleep monitoring can provide an objective assessment of sleep status. Immunotherapy is the main treatment method for this disease, and most patients have a good prognosis.
Objective To investigate the clinical features and key diagnostic points of aceruloplasminemia (ACP), as well as the features of a novel pathogenic mutation. Methods A systematic analysis was performed for the clinical data of one patient with a confirmed diagnosis of ACP, and a literature review was performed based on related articles in China and globally. Results Based on the clinical features of this patient and the analysis of the family, it was clarified that the homozygous c.1944C>G (p.Ser648Arg) mutation in the Ceruloplasmin (CP) gene could cause ACP and was reported for the first time worldwide. Conclusion ACP is an extremely rare autosomal recessive disease due to abnormal iron metabolism caused by a significant reduction in ceruloplasmin, with the main clinical manifestations of retinopathy, diabetes, ataxia, and cognitive impairment, and genetic testing of the CPgene has a relatively high diagnostic value.
Objective To investigate the influence of high copper load on neurobehavior in rats and its possible mechanism of action based on the deacetylase sirtuin 3 (SIRT3)/peroxisome proliferator-activated receptor-γ coactivator-1α (PGC-1α) signaling pathway. Methods A total of 50 adult Sprague-Dawley rats were randomly divided into model group and blank group, with 25 rats in each group. The rats in the model group were fed with 1 g/kg high-copper diet and 0.185% copper sulfate solution (prepared using deionized water) to establish a rat model of high copper load, and after 12 weeks, the shuttle box test and the pole-climbing test were used to observe neurobehavioral changes, while Western blotting and immunofluorescent staining were used to measure the protein expression levels of SIRT3 and PGC-1α in hippocampal tissue. Results The shuttle box test showed that compared with the blank group, the model group had a significant reduction in the number of times of active avoidance reaction after stimulation (P<0.01) and significant increases in the times of active avoidance reaction and passive avoidance reaction (P<0.01);the pole-climbing test showed that compared with the blank group, the model group had a significant increase in pole-climbing time(P<0.01). Western blotting and immunofluorescent staining showed that compared with the blank group, the model group had significant reductions in the protein expression levels of SIRT3 and PGC-1α in hippocampal tissue (P<0.05). Conclusion High copper load has a marked influence on the neurobehavior of rats, possibly by regulating the expression of the SIRT3/PGC-1α signal pathway in hippocampal neurons.
Objective To investigate the differences in emotional processing characteristics and electroencephalography (EEG) power values in patients with Parkinson disease (PD). Methods A total of 24 PD patients were enrolled as subjects, and 30 healthy individuals were enrolled as control group. With the use of the EPIE experimental paradigm, SAM questionnaire was used to determine the scores of emotional valence and arousal, and EEG was used for real-time monitoring of cortical EEG signals. The two groups were compared in terms of the differences in valence/arousal and EEG power values under different emotions and their correlation. Results The PD group had significantly higher BAI and BDI scores than the control group[BAI(16.92±3.83)vs(11.62±3.65),t=4.521,P<0.05;BDI(22.69±2.30)vs(14.17±4.06),t=7.981,P<0.05]. In the negative mood, there were significant differences in valence/arousal between the two groups (t=4.505,-7.705,bothP<0.05). There were significant differences between the two groups in power values at Fp1,Fp2,F7,F3,F4,T3,T4, and T5(t=-4.12,-12.43,5.76,-2.90,-4.72,-5.34,-5.81,-2.65,all P<0.05). In the negative mood, for the control group, valence score was correlated with Fp1 (r=-0.837, P<0.01), Fp2 (r=-0.920, P<0.01),F4(r=-0.604,P=0.008),P3(r=-0.658,P=0.003),and P4(r=-0.546,P=0.019), and arousal score was correlated with Fp1(r=0.887, P<0.01), Fp2 (r=0.958, P=0.003),F4(r=0.683,P=0.003),P3 (r=0.721, P=0.003),and P4 (r=0.610,P=0.007); for the PD group, valence score was correlated with Fp2(r=-0.490,P=0.015) and F7(r=-0.564,P=0.004), and arousal score was correlated with Fp2 (r=0.440, P=0.031) and F7(r=0.853,P<0.01). Conclusion Patients with PD have negative emotional processing abnormalities associated with right PFC and left lateral FL.
Oculopharyngodistal myopathy (OPDM) is a hereditary neuromuscular disorder occurring in adolescence or early adulthood and is characterized by slowly progressive external ophthalmoplegia, facial muscle weakness, dysphagia, and distal limb weakness. This article reports a patient with OPDM type 4 who had the initial presentation of bilateral pyroptosis and external ophthalmoplegia and was misdiagnosed with thyrotoxic myopathy, and later the patient developed the clinical manifestations of bilateral masticatory and facial muscle weakness, bulbar paralysis, and limb weakness. Genetic testing revealed abnormal CGG repeat expansion in the RILPL1 gene. The clinical data of the patient was analyzed, and a literature review was performed, so as to improve the awareness of this condition among clinicians.
This article analyzes the clinical features, diagnosis, and treatment of temporal lobe encephaloceles (TEs), a rare and frequently underdiagnosed etiology of drug-resistant temporal lobe epilepsy (DR-TLE). Based on the data of a patient with a six-year history of DR-TLE, this patient was diagnosed with left TEs after cranial MRI, PET-CT, and ictal video-electroencephalography and then underwent surgical resection of the lesion. The patient achieved effective seizure control after surgery, with complete freedom from seizures after follow-up for 5 years. With reference to this case and the literature review, TEs is a rare cause of DR-TLE and is easily missed clinically, which warrants vigilance. Radiological examinations such as high-resolution three-dimensional cranial MRI and PET are of great importance for the confirmed diagnosis of this disease. For such patients, surgical treatment can achieve significant and sustained therapeutic outcomes.
Spinal cord injury (SCI) is a severe traumatic condition of the central nervous system for which current therapeutic strategies rarely achieve neural regeneration. In recent years, exosomes and their carried microRNA(miRNA) have demonstrated considerable potential in SCI treatment. Functioning as key mediators of intercellular communication, exosomal miRNAs coordinately regulate multiple pathophysiological processes after SCI through multiple targets and pathways.This review systematically summarizes their core mechanisms of action: (1) regulating angiogenesis via miR-126 and miR-27a-3p, and promoting the repair of the blood-spinal cord barrier via miR-210 and miR-2861, thereby improving local microcirculation; (2) precisely modulating the autophagic process via miR-421-3p and miR-374-5p to clear toxic proteins, exerting neuroprotective effects within a specific time window; and (3) facilitating axonal regeneration via miR-26a and miR-34a-5p, and modulating the glial microenvironment via miR-494 and miR-145-5p, thereby creating a favorable milieu for neural regeneration. Although exosomal miRNA therapy faces challenges in targeted delivery, standardized production, and safety evaluation, the ongoing development of engineering modification strategies and multi-omics analytics promises to transform this approach into a breakthrough strategy, bridging the gap from fundamental research to clinical application in SCI treatment.
Paraneoplastic neurologic syndromes (PNS) are immune-mediated disorders of the nervous system triggered by cross-immune response induced by tumor-associated antigens. Since high-risk paraneoplastic antibodies are highy associated with malignancies, they have an important clinical value in the early recognition, tumor screening, and prognostic assessment of PNS. The diagnostic criteria updated in 2021 further refined the classification of high-risk antibodies and clarified their value in risk assessment. This article systematically reviews the clinical spectrum, tumor associations, and immunopathogenic mechanisms of neurological disorders associated with the high-risk paraneoplastic antibodies such as anti-Hu, anti-Ri, anti-CV2, anti-Ma2, anti-amphiphysin, anti-Yo, anti-PCA2, anti-Tr, anti-SOX1, and anti-KLHL11 antibodies. Immunotherapy combined with active control of primary tumor is the main treatment strategy for such disease, but with a limited overall therapeutic effect, and early identification is of particular importance. In the era of the wide application of immune checkpoint inhibitors (ICIs), the risk of ICIs in inducing or exacerbating should be taken seriously in clinical practice. Mechanistic research, multimodal diagnostic approaches, and individualized treatment strategies should be enhanced in the future to improve the prognosis of patients.