Autoimmune encephalitis(AE)is a type of brain inflammation caused by the immune system mistaking autoantigens expressed in the central nervous system for foreign antigens,thereby resulting in abnormal immune response that affects brain parenchyma(cortex or deep gray matter and white matter)and may involve the meninges and spinal cord. This disease is not an infectious inflammatory disease caused by pathogen invasion and is fundamentally different from purulent encephalitis and viral encephalitis,and it can occur in different populations such as children,adolescents,and adults. A recent epidemiological study from the United States shows that there might be a higher prevalence rate of AE,and the prevalence rate of AE was 13.7/100 000 in this population-based study,with no significant difference from the prevalence rate of infectious encephalitis. Since the discovery of anti-N-methyl-D-aspartate(NMDA)receptor antibodies,many patients who experience mental symptoms with rapid progression,abnormal behavior,seizures,or unexplained coma have been diagnosed with AE. The onset of symptoms is usually unclear,which might be similar to mental illness,but then the disease often progresses rapidly and causes damage to brain parenchyma and even loss of consciousness and coma,which usually requires intensive care. Therefore,a comprehensive and systematic understanding of AE is of great significance for clinicians to achieve early identification,diagnosis,and treatment and help patients obtain a good prognosis. This article aims to provide a comprehensive overview of existing research findings of AE in terms of its past,present,and future development and from cognitive limitations to the leap towards precision treatment,in order to provide assistance for diagnosis and treatment among clinicians.
Autoimmune encephalitis(AE)is a heterogeneous disorder caused by immune-mediated attack on the central nervous system,and it is characterized by complex clinical manifestations and insufficient diagnostic specificity,which often leads to misdiagnosis and delayed treatment. By integrating expertise from various specialties such as neurology,critical care medicine,psychiatry,and oncology,multidisciplinary treatment(MDT)can provide individualized diagnosis and treatment regimens for AE patients,with significant advantages in patients comorbid with tumor,autonomic dysfunction,or severe complications. This article elaborates on the theoretical rationale for implementing MDT in AE management,the methods for individualized treatment,and existing challenges in clinical practice,in order to provide a reference for optimizing clinical management strategies for AE and promoting the development of precision diagnosis and treatment for complex nervous system diseases.
Autoimmune encephalitis(AE)is a group of inflammatory disorders within the central nervous system(CNS)triggered by autoimmune mechanisms. Most AE patients show good responses to first-line immunotherapies such as corticosteroids and intravenously injected immunoglobulin. Nevertheless,the adverse effects associated with extensive immunosuppression have become a difficult issue in treatment,and some patients with severe conditions or recurrence still require further intensive therapy. Therefore,it is urgently needed to search for novel treatment strategies with high efficiency,precision,and safety. In recent years,targeted immunotherapy and chimeric antigen receptor T-cell immunotherapy that specifically target immune cells or cytokines involved in the pathogenesis of AE have gradually become research hotspots,with good tolerability and safety,which provides new options for the clinical management of AE.
Autoimmune encephalitis(AE)refers broadly to a group of encephalitic disorders mediated by autoimmune mechanisms. AE is characterized by complex pathogeneses and diverse clinical manifestations,and there are still numerous challenges in the diagnosis and treatment of AE. With the development of frontier technologies such as vaccines,gene editing,and big data,new opportunities have emerged for transforming the diagnosis and treatment strategies for AE. Vaccines play a dual role in AE: on the one hand,they can prevent infections; on the other hand,they may trigger autoimmune responses through molecular mimicry. Emerging nanovaccine technologies are expected to achieve safer and more effective immunomodulation. Gene editing techniques,especially the CRISPR-Cas9 system,have shown potential in targeted regulation of the function of immune cells and repair of nervous tissue,which provides new pathways for precise intervention in the treatment of AE. The application of big data technologies,including artificial intelligence,natural language processing,and deep learning,has promoted the early diagnosis of AE and the development of individualized treatment regimens. This article reviews the research advances and clinical prospects of these cutting-edge technologies in AE,in order to provide insights and references for the development of precision medicine,interdisciplinary collaboration,and future treatment strategies for AE.
Autoimmune encephalitis(AE)is a group of antibody-mediated inflammatory disorders that target neuronal antigens,with the main symptoms of mental and behavioral disorders,epilepsy,and cognitive impairment. In recent years,specific antibodies,such as anti-NMDAR antibody and anti-LGI1 antibody,have played an important role in the pathogenesis of AE and have become effective biomarkers,which have significantly improved the early detection rate of AE. As novel antibodies continue to be discovered,the pathological mechanisms of AE are constantly clarified,providing new directions for precision treatment. Nevertheless,there are still challenges in the mechanism of action and clinical application of these emerging antibodies,requiring support from more basic studies and clinical data. This article discusses the role of antibodies in AE,analyzes the types of newly discovered antibodies and their role in disease progression,and explores the potential and challenges of antibodies used as tools for precise diagnosis and targeted therapy.
This article reports the clinical features and prognosis of a patient with anti-N-methyl-D-aspartate receptor(NMDAR)encephalitis comorbid with novel coronavirus infection. After anti-NMDAR encephalitis is comorbid with SARS-CoV-2 infection,the symptoms may worsen,and timely immunoadsorption and second-line immunotherapy can help with disease recovery.
Objective To investigate the clinical manifestations and prognosis of patients with anti-leucine rich glioma inactivated 1(LGI1)antibody encephalitis. Methods A retrospective analysis was performed for the data of patients with anti-LGI1 antibody encephalitis who were hospitalized in Department of Neurology,Xuanwu Hospital,Capital Medical University,from September 2019 to December 2023,including clinical features,treatment,and prognosis. Results A total of 80 patients were enrolled,including 48 male patients and 32 female patients. Of all 80 patients,51(63.8%)had the initial symptom of epilepsy,13(16.3%)had the initial symptom of faciobrachial dystonic seizures,26(32.5%)had the initial symptom of cognitive impairment,and 12(15%)had the initial symptom of mental and behavioral disorders. As for comorbidities,there were 28 patients(35%)with hyponatremia,22(27.5%)with Hashimoto's thyroiditis,and 5(6.25%)with tumor. All 80 patients tested positive for serum LGI1 antibody,and 69(86.3%)tested positive for LGI1 antibody in cerebrospinal fluid. Among the 80 patients,37(46.25%)had abnormalities on magnetic resonance imaging,and 30 had abnormalities on 18F-FDG PET/CT. There were 50 patients with abnormalities on video electroencephalography. A total of 77 patients received first-line immunotherapy,and after follow-up for 1 year,19.4% of the patients had the sequela of seizure,59.7% of the patients had deterioration of the memory,and 48.6% of the patients had mental and behavioral disorders. Conclusion Epilepsy,cognitive impairment,and mental and behavior disorders are the most common manifestations of anti-LGI1 antibody encephalitis. There is generally a good prognosis after immunotherapy,with the sequelae of cognitive impairment and mental and behavioral disorders.
Objective To investigate the clinical features,laboratory findings,and prognosis of patients with autoimmune encephalitis positive for leucine rich glioma inactivated 1(LGI1)antibody. Methods We reviewed the clinical data of 11 patients with anti-LGI1 encephalitis hospitalized in Fu Yang People's Hospital from October 2019 to December 2024. Results All the 11 patients(100%)had cognitive function involvement,9(81.8%)had epileptic seizures,5(45.5%)had mental and behavioral abnormalities,4(36.4%)had sleep disorders,3(27.3%)had autonomic nervous dysfunction,2(18.2%)had faciobrachial dystonic seizures(FBDS),2(18.2%)had facial numbness,and 1(9.1%)had phantosmia and pruritus in both eyes and the neck. LGI1 antibody was positive in the serum of all the cases(100%)and present in the cerebrospinal fluid of 8 cases(72.3%). Seven cases(63.6%)had hyponatremia,and 5 cases(45.5%)also had hypophosphatemia,hypocalcemia,and hypomagnesemia in addition to blood sodium lower than 134 mmol/L. Intracranial abnormalities were detected in 7 cases(63.6%)on magnetic resonance imaging. Electroencephalogram abnormalities were recorded in 6 cases(54.5%). After immunosuppressive treatment,2 cases(18.2%)had recurrent symptoms,and 2 cases(18.2%)had residual mild memory impairment. In terms of prognosis,the modified Rankin Scale scores were generally favorable. Conclusion Anti-LGI1 encephalitis manifests as convulsions,FBDS,memory decline,mental and behavioral abnormalities,autonomic nervous dysfunction,sleep disorders,hyponatremia,and multiple electrolyte disorders such as hypomagnesemia,hypocalcemia,and hypophosphatemia when blood sodium is below 134 mmol/L. The prognosis with immunosuppressive treatment is favorable,but recurrent symptoms may occur.
Objective To investigate the potential risk factors for hemorrhagic transformation(HT)after mechanical thrombectomy(MT)in patients with acute ischemic stroke due to anterior circulation large vessel occlusion(AIS-AC-LVO),and to provide a scientific evidence for reducing post-thrombectomy HT and improving the prognosis of patients. Methods retrospective analysis was performed for the data of 605 patients with AIS-AC-LVO. The Logistic regression analysis was performed for related clinical data to identify independent predictive factors for HT after MT,with a significance threshold of P<0.05. Results A total of 605 patients were included in this study,among whom 207(34.2%)developed HT after MT,while 398(65.8%)did not experience HT. The statistical analysis showed that multiple attempts of thrombectomy(OR=1.340,P=0.003),a prolonged time of operation(OR=1.005,P=0.024),a low ASPECTS score before surgery(OR=0.798,P<0.001),postoperative mTICI≤2a(OR=6.329,P=0.032),a low platelet count before surgery(OR=0.995,P=0.008),and a high level of blood glucose before surgery(OR=1.088,P=0.009)were independent predictive factors for HT. Symptomatic intracranial hemorrhage(sICH)was observed in 68 patients(11.2%),and a history of atrial fibrillation(OR=3.151,P=0.003),a low ASPECTS score before surgery(OR=0.868,P=0.007),a prolonged time of operation(OR=1.009,P<0.001),poor collateral circulation(ASITN/SIR<2)(OR=2.307,P=0.035),a low platelet count before surgery(OR=0.993,P=0.012),a high level of D-dimer before surgery(OR=1.050,P=0.012),and a high level of blood glucose before surgery(OR=1.138,P=0.013)were independent predictive factors for sICH. Conclusion Multiple attempts of thrombectomy,a prolonged time of operation,a low ASPECTS score before surgery,postoperative mTICI≤2a,a low platelet count before surgery,and a high level of blood glucose before surgery are independent predictive factors for HT. A history of atrial fibrillation,a low ASPECTS score before surgery,a prolonged time of operation,poor collateral circulation(ASITN/SIR<2),a low platelet count before surgery,a high level of D-dimer before surgery,and a high level of blood glucose before surgery are independent predictive factors for sICH. Identifying such risk factors is crucial for reducing HT after MT and improving the efficacy of thrombectomy.
Objective To explore the relationship between translocase of outer mitochondrial membrane 40(TOMM40)gene polymorphism and inflammatory status and cognitive function in patients with vascular dementia(VaD). Methods From June 2020 to December 2022,48 patients with VaD and 40 age-matched control participants were enrolled. TOMM40_rs11556505 and IL-10-592 A/C_rs1800872 polymorphisms were measured. The cognitive function of the patients was evaluated by using the Repeatable Battery for the Assessment of Neuropsychological Status(RBANS). Results The main effects of VaD were significant in all the analyses of the sub-scores and total score of RBANS(all P<0.001,all FDR<0.001). TOMM40_rs11556505 polymorphism had a significant main effect on language score(F=4.524,P=0.034,Cohen f=0.08). TOMM40×VaD had significant interaction effects on immediate memory(F=4.682,P=0.031,Cohen f=0.08)and language score(F=4.465,P=0.035,Cohen f=0.08). The two-way multivariate analysis of covariance(IL-10×TOMM40)revealed significant interaction effects between their gene polymorphisms on visual space/structure(F=4.063,P=0.045,FDR=0.080,Cohen f=0.13),language(F=12.031,P<0.001,FDR=0.006,Cohen f=0.23),and delayed memory(F=5.118,P=0.025,FDR=0.075,Cohen f=0.23)in patients with VaD. Conclusion IL-10-592 A/C and TOMM40_rs11556505 gene polymorphisms have interaction effects on cognitive function,with their influence direction depending on each other's genotype.
Objective To investigate the sleep architecture of sleep related bruxism(SB)in adults and the sleep architecture of SB comorbid with obstructive sleep apnea hypopnea syndrome(OSAHS),as well as their correlation with age and other factors. Methods A total of 51 subjects with SB and 67 controls were included in this study to analyze the sleep architecture of SB and compare the sleep architecture of SB comorbid with different severities of OSAHS. Results Compared with the control group,the SB group had a younger age,increases in N1(%TST)and N2(%TST),a reduction in N3(%TST),and an increase in arousal index. The SB group was divided into non-OSAHS group(group 1),mild OSAHS group(group 2),and moderate-to-severe OSAHS group(group 3). Group 1 had a younger age than group 2 and group 3,and group 3 had increases in body mass index(BMI),N1(%TST),oxygen desaturation index(ODI),and arousal index and a reduction in N3(%TST). The Spearman's rank correlation analysis showed that BMI,N1(%TST),arousal index,and ODI increased with the increase in apnea-hypopnea index(AHI),while N3(%TST)decreased with the increase in AHI. The binary logistic regression analysis showed that SB was negatively correlated with age and was positively correlated with arousal index. Conclusion SB may affect sleep architecture by increasing light sleep,reducing deep sleep,and increasing the number of awakenings. There are changes in sleep architecture in case of SB comorbid with different severities of OSAHS. SB is negatively correlated with age and is positively correlated with arousal index.
Objective To establish a model for accurate prediction of hematoma expansion(HE)following spontaneous intracerebral hemorrhage(sICH)based on CT radiomics and different machine learning algorithms. Methods A retrospective analysis was performed for the patients with sICH who were admitted to Sir Run Run Shaw Hospital and Beijing Electric Power Hospital in 2019-2022,and they were established as the training cohort and the external validation cohort,respectively. Radiomic features of hematomas were extracted from CT images and were combined with related clinical risk factors for HE to establish predictive models using four different machine learning algorithms. The receiver operating characteristic(ROC)curve was used to assess the predictive performance of each model,and the area under the ROC curve(AUC)and corresponding confusion matrix metrics were calculated. Results Compared with the other three models,the support vector machine(SVM)model showed the best predictive performance,achieving an AUC of 0.844 in the training cohort and 0.994 in the external validation cohort. In addition,the SVM model achieved the highest F1 scores(0.891 and 0.989),accuracy rates(83.8% and 98.4%),precision rates(94.5% and 99.9%),and specificities(82.4% and 99.9%). Conclusion The radiomics model based on SVM provides a noninvasive tool for accurately predicting the risk of HE,which can help clinicians to identify high-risk sICH patients in the early stage and adjust treatment strategies in a timely manner.
Objective To investigate the association between beat-to-beat blood pressure variability(BPV)and prognosis in patients with acute ischemic stroke(AIS)undergoing mechanical thrombectomy(MT). Methods A retrospective study was conducted among 52 AIS patients who underwent MT in Stroke Center of The First Hospital of Jilin University,and beat-to-beat BPV was monitored during hospitalization. The patients were followed up to observe modified Rankin Scale(mRS)score on day 90 after disease onset,and the patients were divided into good prognosis group(mRS≤1)and poor prognosis group(mRS>1). The two groups were compared in terms of beat-to-beat BPV during hospitalization. A multivariate logistic regression analysis was used to investigate the association between beat-to-beat BPV and prognosis. Results Compared with the good prognosis group,the poor prognosis group had significantly higher beat-to-beat successive variation(SV)and average real variability(ARV)of systolic blood pressure(SBP)[SBP-SV: 2.63(1.84,3.48)vs 3.28(2.87,4.08),P=0.032; SBP-ARV: 2.06(1.30,2.55)vs 2.59(2.23,3.53),P=0.006]. After adjustment for confounding factors such as age,sex,risk factors for stroke,and baseline NIHSS score,the multivariate logistic regression analysis showed that beat-to-beat SBP-SV(OR=2.760,95%CI 1.168-6.522,P=0.021)and SBP-ARV(OR=3.916,95%CI 1.278-12.002,P=0.017)were associated with the poor prognosis of patients. Conclusion Beat-to-beat BPV is independently associated with 90-day poor prognosis in AIS patients undergoing MT,and therefore,it can be used as a predictive factor for prognosis.
Dyskinesia-hyperpyrexia syndrome(DHS)is an acute hyperpyrexia syndrome that is different from Parkinsonism-hyperpyrexia syndrome and serotonin syndrome in patients with advanced Parkinson's disease(PD),with the main symptoms of high fever,disturbance of consciousness,elevated creatine kinase,and dyskinesia. Syndrome of inappropriate secretion of antidiuretic hormone(SIADH)is a clinical syndrome caused by excessive secretion of antidiuretic hormone,which leads to the symptoms of dilutional hyponatremia,water retention,and increases in urine sodium and urine osmotic pressure. DHS has not received widespread attention in clinical practice,and there are rare reports of DHS comorbid with SIADH. This article reports the diagnosis and treatment processes of a case of DHS comorbid with SIADH and reviews the relevant literature,in order to guide the diagnosis and treatment of PD-related critical diseases.
Congenital myasthenic syndrome(CMS)is a group of neuromuscular transmission disorders caused by genetic defects,and it is characterized by an early age of onset,fatigable weakness of skeletal muscle,and a high disability rate,with respiratory failure as a common cause of death. Endplate acetylcholinesterase deficiency(EAD)caused by COLQ gene mutations is a subtype of CMS,and repetitive compound muscle action potential(R-CMAP)in nerve conduction examination is the key evidence for diagnosis. The heterogeneity of clinical phenotypes of CMS often leads to misdiagnosis or missed diagnosis,resulting in delayed treatment. This article reports a case of CMS caused by COLQ mutation and summarizes the pathogenesis,mechanisms,clinical manifestations,electrophysiological characteristics,differential diagnosis,treatment,and prognosis of the disease with reference to relevant literature,in order to improve the understanding of this subtype of CMS among clinicians.
Endovascular interventional therapy is currently the main treatment for acute ischemic stroke,but some patients still have not achieved neurological function independence. Analysis of the predictive value of various factors for postoperative neurological function changes can provide more reference for selecting appropriate patient groups and treatment plans.
Acute large-vessel occlusive ischemic stroke has high disability and mortality rates,causing a great burden to social economy and health care system. Mechanical thrombectomy has become the standard treatment method for this disease,but some patients still have poor prognosis after successful recanalization of blood vessels,and therefore,it is important to explore the factors that can be used to judge the poor prognosis of patients. Quantitative electroencephalography can quantify and objectively assess the changing process of brain function in patients,and RAPID perfusion parameters based on brain CTP can rapidly quantify the local blood perfusion of brain tissue. This combination of perfusion neuroimaging and electrophysiological activity can quantify the severity of ischemia associated with cerebral infarction and functional damage to neurons. This article reviews the application value of these two methods in the prognostic evaluation of stroke.
Benign paroxysmal positional vertigo(BPPV)is a common peripheral vestibular disorder,and at present,otolith shedding and displacement is highly recognized as the main pathological mechanism of BPPV. An increasing amount of evidence has shown that otolith particle shedding is closely associated with vitamin D,and 25-(OH)D is expected to become a potential biomarker for BPPV and an important target for the treatment of BPPV and residual symptoms after successful repositioning. This article reviews the pathophysiological mechanism of vitamin D in BPPV and residual dizziness and summarizes the association of vitamin D with BPPV and residual symptoms based on the treatment methods for vitamin D regulation.
As ultrasound medicine is a clinical specialty characterized by high technological dependency and interdisciplinary integration, the cultivation of research abilities in the standardized residency training for ultrasound medicine specialists is crucial for disciplinary innovation and physicians’ career development. This article systematically analyzes the current status and challenges of research ability training in ultrasound residency programs and proposes core strategies including a hierarchical training model, systematic research thinking training, and mentor system optimization. Additionally, the innovative approaches such as multidisciplinary integration and digital tools are explored. Future efforts should focus on establishing a nationally unified standardized training system, strengthening mental health support, and promoting career sustainability, ultimately achieving a “clinical-research-humanistic” integrated talent cultivation framework and providing compound ultrasound medicine talents for the Healthy China strategy.