Iodine-131-meta-iodobenzylguanidine ( 131 I-MIBG) cardiac scintigraphy is a nuclear medicine technique used to assess the function of the sympathetic nervous system in the heart. Decreased myocardial 131 I-MIBG uptake has been reported in some patients with neurodegenerative diseases. The purpose of this study was to compare sympathetic denervation in the myocardium across various neurodegenerative diseases. We recruited 222 patients with Parkinson's Disease (PD), 37 with Parkinson's Disease Dementia (PDD), 10 with Dementia with Lewy Bodies (DLB), 116 with Multiple System Atrophy (MSA), 14 with Pure Autonomic Failure (PAF), 9 with Progressive Supranuclear Palsy (PSP), and 63 with Parkinsonian Syndrome (PDS). The heart-to-mediastinum ratio (H/M ratio) at 15 minutes and 4 hours post-injection of 131 I-MIBG cardiac scintigraphy was calculated and compared among the neurodegenerative disease groups mentioned above. Patients with PD, PDD, and DLB had significantly lower H/M ratios for both early and delayed images than those with PAF, MSA, PSP, and PDS ( p  < 0.05). Moreover, patients with PDD and DLB demonstrated significantly reduced H/M ratios in both imaging sessions compared to those with PD ( p  < 0.05). However, no significant differences were observed between the PDD and DLB groups. 131 I-MIBG cardiac scintigraphy is a valuable research tool for enhancing our understanding of autonomic nervous system involvement in neurodegenerative diseases. Patients with PDD and DLB showed lower cardiac MIBG uptake than those with PD. Our findings indicate a novel perspective on the potential differences in pathological changes among PD, PDD, and DLB.

Background:Tabes dorsalis is a late manifestation of neurosyphilis, characterized by progressive ataxia, lightning pains, loss of proprioception, and urinary incontinence. The absence of a definitive diagnostic standard and the non-specific clinical manifestations have led to a significant rate of misdiagnoses. Methods:Hospitalized patients with tabes dorsalis at Peking Union Medical College Hospital between January 2010 and December 2023 were reviewed. Results:A total of 13 patients were included, with 10 males and 3 females. The median age was 50 years (range, 34-64). The most frequent initial symptoms were limb numbness (30.8%) and lightning pains (30.8%). Eleven patients (84.6%) received misdiagnoses prior to the final diagnosis. The most frequently observed physical sign was positive Romberg's sign (84.6%). Notably, Argyll Robertson pupil was presented in 7 subjects (53.8%). Serological tests revealed positive rapid plasma regain (RPR) and Treponema pallidum particle agglutination (TPPA) for all patients. All CSF samples were TPPA-reactive. Intramedullary hyperintensity on T2-weighted imaging of spinal MRI was found in 5 patients (38.5%). All patients received anti-syphilitic treatment, with effective treatment recorded in five cases. Conclusion:This study underscores the importance of neurological symptoms and signs in diagnosing tabes dorsalis. Individuals with progressive ataxia and positive Romberg's sign should be closely monitored for potential neurosyphilis. Integrating clinical features, laboratory tests, and neuroimaging could reduce misdiagnosis and expedite the initiation of anti-syphilitic therapy.

Purpose:General paresis is a common type of neurosyphilis featuring progressive cognitive deterioration. The lack of a golden standard of diagnosis and its nonspecific clinical manifestations resulted in a high rate of misdiagnoses. This study aims to investigate the clinical, laboratory and radiological presentations of general paresis and enrich its knowledge for timely diagnoses.Patients and methods:The study collected hospitalized patients admitted for general paresis from September 2002 to November 2022. Their socio-demographical and medical status, clinical presentations, cognitive assessments, laboratory and radiographical manifestations and treatment information were collected retrospectively.Results:A total of 20 males and 5 females were included. Patients' ages ranged from 30 to 66 years (average 50.3 years). The average and median time for diagnosing general paresis was 14.1 months and 10.0 months respectively. The most frequent initial symptom is memory deterioration (68.0%). Impaired calculative ability and memory deterioration were the most frequent cognitive anomalies, as found in 50% and 45.4% of subjects during examination. The mean and median scores of MoCA was 16.7 and 17 respectively. Serological tests revealed positive TPPA for all patients and a median RPR titer at 1:64 positive. All CSF samples with TPPA and FTA-ABS results reported positivity. The MRI manifestations of general paresis include patchy or speckled hyperintensities (70.8%) and cerebral atrophy (45.8%). The most common lesioned sites in MRI were the ventricular and paraventricular area (50.0%) and temporal lobes (45.8%). For treatment, penicillin-based anti-syphilitic plans were adopted in 17 patients (68.0%).Conclusion:The clinical features and radiological alternations of general paresis patients often exhibited diverse and nonspecific alternations. However, some specific clinical manifestations and auxiliary examinations can provide meaningful clues for the identification and differential diagnosis of this disease.

To evaluate the efficacy of high-frequency repetitive transcranial magnetic stimulation (rTMS) in patients with primary progressive aphasia (PPA). In this randomized, double-blind trial in a single center, patients who were diagnosed with PPA were randomly assigned to receive either real rTMS or sham rTMS treatment. High-frequency rTMS was delivered to the dorsolateral prefrontal cortex (DLPFC). The primary outcome was the change in Boston Naming Test (BNT) score at each follow-up compared to the baseline. The secondary outcomes included change in CAL (Communicative Activity Log) and WAB (Western Aphasia Battery) compared to baseline and neuropsychological assessments. Forty patients (16 with nonfluent, 12 with semantic and 12 with logopenic variant PPA) were enrolled and randomly assigned to the rTMS or sham rTMS group, with 20 patients in each group. Thirty-five patients (87.5%) completed a 6-month follow-up. Compared to the sham rTMS group, the BNT improvement and WAB improvement in the real rTMS group were significantly higher. These significant improvements could be observed throughout the entire 6-month follow-up. At 1 month and 3 months after treatment, CAL improvements of real rTMS were significantly higher than sham rTMS. The improvements in BNT, CAL and WAB did not significantly differ among PPA variants. No significant improvement in neuropsychological assessments was observed. High-frequency rTMS delivered to DLPFC improved language functions in patients with different PPA variants. The efficacy was still observed after 6 months of treatment. Trial registration: NCT04431401 ( https://clinicaltrials.gov/ct2/show/NCT04431401 ).

Dementia with Lewy bodies(DLB)is the second most common neurodegenerative dementia after Alzheimer's Disease(AD). This article will mainly elaborate the relationship between DLB and blood-brain barrier(BBB)from the following five aspects: (1)The structure and function of BBB; (2)In vivo assessment methods for the blood-brain barrier damage; (3)Evidence for the damage of blood-brain barrier in DLB; (4)The relationship between α-synuclein and the blood-brain barrier; (5)The relationship between APOE and the blood-brain barrier.Future research should focus on the pathogenesis of BBB damage in DLB patients, by which new drug targets for disease diagnosis and treatment may be found.

Abstract Background: Cerebrospinal fluid (CSF) shunt intervention is currently the standard and most effective treatment for idiopathic normal-pressure hydrocephalus (iNPH). However, many patients do not undergo surgery due to various reasons. The study aimed to assess the efficacy and safety of methazolamide (MTZ) in the treatment of iNPH. Methods: A 12-week randomized, double-blind, drug-placebo clinical studywas conducted at the Aviation General Hospital. Participants with a diagnosis of possible or probable iNPH according to Japanese second iNPH guidelines were consecutively recruited from September 2019 to May 2021. All patients were inoperable or had refused surgical treatment due to various reasons. The patients were assigned (2:1) to the MTZ or placebo group via a computer-generated randomization list. The gait and cognition function were assessed, and brain MRI scans were taken before and 12 weeks after drug administration. The primary endpoint was the difference change in the Boon gait sum score relative to baseline between the drug and placebo groups. Secondary endpoints were differences in MMSE and MOCA scores. In addition, the adverse reactions were also monitored. Results: Twenty participants were randomly included in the MTZ group and 12 in the placebo group. A total of 26 patients, 17 in the MTZ and 9 in the placebo group, were included in the final efficacy analysis. The difference in Boon sum score relative to baseline between the groups was -6.06 points (95% CI, -9.37 to -2.75; p =0.001) after 12 weeks of treatment. However, there was no significant difference in the MMSE and MoCA scores between the drug and placebo groups (MMSE 0.36, 95% CI, -2.36 to 3.09; p = 0.78; MoCA 0.001, 95% CI, -1.98 to 1.98; p = 1.00). No serious adverse reactions such as hypokalemia or acidosis occurred in any of the patients. Conclusions: MTZ significantly improved the gait of iNPH patients without any adverse effects. Large sample studies are needed for further confirmation of our results.

Parkinson’s disease (PD) is the second most common neurodegenerative disorder and the heterogeneity of early PD leads to interrater and intrarater variability in observation-based clinical assessment. Thus, objective monitoring of PD-induced motor abnormalities has attracted significant attention to manage disease progression. Here, we proposed a hierarchical architecture to reliably detect abnormal characteristics and comprehensively quantify the multisymptom severity in patients with PD. A novel wearable device was designed to measure motor features in 15 PD patients and 15 age-matched healthy subjects, while performing five types of motor tasks. The abnormality classes of multimodal measurements were recognized by hidden Markov models (HMMs) in the first layer of the proposed architecture, aiming at motivating the evaluation of specific motor manifestations. Subsequently, in the second layer, three single-symptom models differentiated PD motor characteristics from normal motion patterns and quantified the severity of cardinal PD symptoms in parallel. In order to further analyze the disease status, the multilevel severity quantification was fused in the third layer, where machine learning algorithms were adopted to develop a multisymptom severity score. The experimental results demonstrated that the quantification of three cardinal symptoms was highly accurate to distinguish PD patients from healthy controls. Furthermore, strong correlations were observed between the Unified PD Rating Scale (UPDRS) scores and the predicted subscores for tremor ${(R = 0.75,\;P = 1.40e - 3)}$ , bradykinesia ${(R = 0.71,\;P = 2.80e - 3)}$ , and coordination impairments ${(R = 0.69,\;P = 4.20e - 3)}$ , and the correlation coefficient can be enhanced to ${0.88}\,\,{(P = 1.26e - 5)}$ based on the fusion schemes. In conclusion, the proposed assessment architecture holds great promise to push forward the in-home monitoring of clinical manifestations, thus enabling the self-assessment of disease progression.

阿尔茨海默病(Alzheimer's disease,AD)是最常见的进行性神经退行性疾病,其特征是神经元丢失和认知功能障碍,该疾病严重影响老年人的生活,因此,探索AD的治疗具有重要的社会意义.丹酚酸B(salvianolic acid B,SAB)是源自丹参的主要水溶性活性天然化合物,对神经退行性疾病具有预防和治疗作用.体外和体内研究表明,SAB对AD具有改善作用.该研究旨在回顾SAB对AD治疗的实验研究,并阐述总结SAB对AD治疗的潜在分子机制,以期对后续研究起指导作用.

目的 探讨标准化F波潜伏期在糖尿病周围神经病(DPN)中的诊断价值.方法 采用前瞻性研究方法 ,收集神经传导检查正常的DPN患者48例,另选择年龄和性别匹配的健康自愿者48名为对照组.采用肌电图诱发电位仪检测两组正中神经、尺神经、胫神经及腓总神经F波最短潜伏期(FLmin)、F波平均潜伏期(FLmean)、F波潜伏最长期(FLmax),并结合臂长和腿长计算标准化FLmin、标准化FLmean、标准化FLmax.比较两组间F波潜伏期及标准化F波潜伏期的差异,采用ROC曲线分析不同F波潜伏期在DPN中的诊断价值.结果 除尺神经身高标准化FLmax外,DPN组4条神经的肢长和身高标准化F波潜伏期均较对照组延长(P<0.01或P<0.05).ROC曲线分析显示,除尺神经常规F波潜伏期及身高标准化FLmean和FLmax外,4条神经常规F波潜伏期、身高标准化F波潜伏期及肢长标准化F波潜伏期对DPN均有诊断价值,ROC曲线下面积(AUC)为0.631～0.995(P<0.05),其中正中神经、尺神经、胫神经和腓总神经的肢长标准化FLmin的诊断价值最高,其AUC分别为0.995、0.934、0.940、0.969(均P<0.01).结论 针对神经传导检查正常的DPN患者,标准化F波潜伏期优于常规F波潜伏期,其中肢长标准化F波潜伏期的诊断价值优于身高标准化F波潜伏期.标准化F波潜伏期可作为临床诊断DPN的一个参考指标.

Objective: To observe the clinical efficacy of Tuina (Chinese therapeutic massage) plus oxiracetam in treating mild vascular dementia (VD) and seek its underlying mechanism. Methods: Ninety-six patients with mild VD were randomized into an observation group and a control group, with 47 cases in the observation group and 49 cases in the control group. The control group received oral oxiracetam capsules for treatment, and the observation group was given additional Tuina treatment. Before and after treatment, the mini-mental state examination (MMSE) was adopted to assess the patient's cognitive function; the activities of daily living (ADL) scale was used to evaluate their ability to conduct daily activities; changes in the serum inflammatory factors and oxidative stress indicators were also detected. Results: After treatment, the serum content of malondialdehyde (MDA) decreased in both groups (P<0.05) and was lower in the observation group than in the control group (P<0.05); the serum contents of superoxide dismutase (SOD) and glutathione peroxidase (GSH-Px) increased in both groups (P<0.05) and were higher in the observation group than in the control group (P<0.05); the serum contents of interleukin (IL)-1, tumor necrosis factor (TNF)-α, IL-6, and IL-8 declined in both groups (P<0.05) and were lower in the observation group than in the control group (P<0.05). After the intervention, the levels of systolic velocity (Vs) and mean velocity (Vm) of the middle cerebral artery elevated, and the pulsatility index (PI) dropped in patients in the two groups, showing significant intra-group differences (P<0.05); the levels of Vs and Vm in the observation group were higher than those in the control group, and the PI was lower in the observation group than in the control group, showing significant between-group differences (P<0.05). The MMSE and ADL scores increased in both groups after the intervention (P<0.05) and were higher in the observation group than in the control group (P<0.05). Conclusion: In the treatment of mild VD, Tuina plus oxiracetam can improve the cerebral blood supply, ADL, and cognitive function; the mechanism may be associated with the reduction of oxidative stress damages and inflammatory reactions.

阿尔茨海默病(AD)是痴呆的主要原因,约占痴呆发病人数的60％～70％.约90％的AD患者在65岁以后发病,60岁以前发病者在临床上非常少见,称为早发AD(EOAD)[1],一般与家族遗传有关.本文报道1例散发性EOAD.

Objective:To assess the safety and efficacy of Anthocyanins for the treatment of Alzheimer's disease.Methods:From November 2018 to December 2020, a multicenter, double-blind, randomized controlled clinical study was conducted in 6 hospitals.The regular medication for the two groups was memantine, with the addition of a combination preparation containing Anthocyanins for the experimental group and a placebo for the control group.The Mini-Mental State Scale(MMSE), Montreal Cognitive Assessment Scale(MoCA), Alzheimer's Disease Assessment Scale-Cognitive Subscale(ADAS-cog), Activities of Daily Living Scale(ADCS-ADL)and Hamilton Depression Scale(HAMD)were used for assessment at the beginning.After 16 weeks of treatment, MMSE, MoCA, ADCS-ADL, ADAS-cog and the Clinician's Interview-Based Impression of Change Plus Caregiver Input(CIBIC-Plus)Scale were conducted and adverse events were recorded.Results:A total of 66 patients were enrolled, with 33 in the control group and 33 in the experimental group.There were no significant differences in cognitive function scores between the two groups before enrollment.Differences in MMSE scores, MOCA scores and ADAS-cog scores before and after treatment between the control group and the experimental group were 1.9±2.4 vs.3.4±2.0( t=2.62, P=0.011), 1.8±1.9 vs.2.9±1.4( t=2.45, P=0.018)and 3.0±2.3 vs.5.3±4.6( t=2.45, P=0.019), respectively.The differences were statistically significant.Instrumental activities of daily living(IADL)scores before and after treatment in the control group were 21.6±5.7 vs.22.6±6.2( t= 2.09, P= 0.046), and those in the experimental group were 22.7±5.4 vs.23.4±5.4( t= 2.45, P= 0.021). The differences between the two groups before and after treatment were statistically significant. Conclusions:Treatment with Anthocyanins can delay the decline of cognitive function and activities of daily living ability in patients with Alzheimer's disease.Anthocyanins may be a promising therapeutic drug for Alzheimer's disease in the future.

Background: The value of cerebrospinal fluid (CSF) biomarkers for assessing idiopathic normal pressure hydrocephalus (iNPH) must be determined. This prospective study aimed to reveal the correlation between CSF biomarkers and clinical symptoms of iNPH and the predictive value of these biomarkers for tap test responsiveness. Methods: Thirty-nine patients with suspected iNPH were recruited, contributed qualified CSF, and underwent a tap test and unified pre- and post-test evaluations of the neurological function. Results: The analysis of biomarkers from the patients’ CSF showed decreased levels of tau and its phosphorylated form, especially in the tap test (+) group. The responsiveness of the tap test was also related to the number of combined symptoms ( p < 0.01), and a correlation was found between the end pressure or pressure difference in CSF and tap test responsiveness ( p < 0.05). The results of the binary logistic regression analysis showed that P (tap test responsiveness) = 1/1 + e ∧ − (−5.505 + 55.314 * ratio of p/ T -tau − 1.586 * numbers of combined symptoms). The combined indicators (−5.505 + 0.553 * percentage of p/ T -tau − 1.586 * numbers of combined symptoms) resulted in the highest sensitivity and specificity of 94.12% and 72.73%, respectively. Conclusions: CSF biomarkers may be assessed to judge tap test responsiveness, which is beneficial for the feasibility of a clinical application.

氧化应激参与阿尔茨海默病(AD)的发病机理，较重点讨论在AD中的作用。还原型烟酰胺腺嘌呤二核苷酸磷酸(NADPH)氧化酶的激活为活性氧产生的重要来源。本文就NADPH氧化酶在AD中的作用进行了论述，同时对血红素氧化酶-1(HO-1)与NADPH氧化酶的相互关系进行了阐述，提出通过药物上调HO-1进而抑制NOX 2 NADPH氧化酶/活性氧通路抑制Aβ诱导损伤，因此，筛选调控HO-1/NOX2 NADPH氧化酶轴的药物有可能成为治疗AD的潜在靶点。

目的:对影响轻中度阿尔茨海默病患者日常生活能力下降的因素进行分析.方法:对64例轻中度阿尔茨海默病患者进行16周的随访调查,调查开始及16周后均行简易智能状态量表(MMSE)、蒙特利尔认知评估量表(MoCA)、日常生活能力量表(ADCS-ADL)、阿尔茨海默病评价量表-认知分量表(ADAS-cog)等评估.结果:未用药组入组前MMSE评分、MoCA评分、ADCS-ADL评分及ADAS-cog评分分别为20.50±3.53分、18.67±7.01分、17.50±11.73分及45.17±4.28分,16周观察期后分别为18.25±6.23分、17.92±6.96分、18.67±12.23分及43.67±7.72分,均有恶化趋势,但前后差异没有统计学意义;单用药组入组前MMSE评分、MoCA评分、ADCS-ADL评分及ADAS-cog评分分别为19.50±4.50分、17.30±5.47分、16.13±9.24分及46.50±5.59分,16周观察期后分别为22.73±4.88分、17.83±6.34分、14.50±8.20分及48.58±5.63分,在MMSE评分、ADAS-cog评分和ADCS-ADL评分较前有改善,差异有统计学意义;联用药组入组前MMSE评分、MoCA评分、ADCS-ADL评分及ADAS-cog评分分别为18.75±2.63分、18.58±5.02分、19.08±18.00分及42.58±6.59分,16周观察期后分别为24.83±6.22分、19.67±4.10分、16.42±15.54分及48.00±5.83分,16周后的评分较入组前的评分均有改善,差异有统计学意义.多元线性回归分析显示,受教育程度、基础ADCS-ADL评分、抗痴呆治疗方案及照料对于ADCS-ADL评分变化有影响,P<0.05.结论:尽早联合应用抗痴呆类药物及加强照料具有改善AD患者日常生活能力的作用,而受教育程度也在其中起到积极的作用.

早期临床检测阿尔茨海默病和额颞叶变性所存在的轻微认知损害,并提高其鉴别诊断价值至关重要.尽管受试者的症状诉求是最为早期的征象,但需要相对客观的检测手段进行评估,神经心理测试作为一种相对客观的工具,能检测认知损害,监测疾病进展,评估药物疗效,被广泛应用于临床研究,本文就其在阿尔茨海默病和额颞叶变性的应用基础以及价值进行综述.

谵妄和痴呆是两种独立的疾病状态，以记忆和判断受损、困惑、迷失方向为特征，伴有不同程度偏执和幻觉，这些相似之处使临床医生对两者的鉴别极具挑战性，尤其是对于缺乏临床经验的医生。然而，谵妄和痴呆还具有明显和关键的差异，一旦确认便可以协助临床医生作出准确的鉴别诊断，本文重点阐述谵妄和痴呆的鉴别诊断以及如何判定痴呆是否合并有谵妄。

Alzheimer's disease(AD), characterized by a progressive decline in cognitive functions, may manifest differently in disease progression.Many researchers have turned their attention to the role of microbes in AD pathogenesis, with increasing evidence suggesting that microbial infections in the central nervous system and peripheral tissues may trigger the innate immune system via direct and/or indirect inflammatory reaction of the nervous system, then leading to the accumulation of amyloid-β(Aβ)plaques and hyperphosphorylated tau in the brain in AD.This article reviews the pathogenesis of AD concerning inflammatory reaction caused by microbes.

目的:总结经北京协和医院诊断很可能(probable)脑淀粉样血管病(cerebral amyloid angiopathy,CAA)的临床特点.方法:对诊断符合临床很可能(probable)脑淀粉样血管病患者7例的影像学特点和脑脊液痴呆标记物检查进行回顾分析总结.结果:共纳入7例CAA患者,男5例,女2例,平均年龄为70.6±4.8岁.患者起病方式均为隐袭起病,临床症状包括情景记忆障碍、精神行为症状、神经系统定位体征.MRI检查示脑室扩大、脑白质缺血灶、对称性或多发性脑白质缺血灶,脑叶出血病灶,海马MAT分级I级(0例)、II级(2例)、III级(3例)和IV级(2例).2例患者行PET-CT检查,双侧前额、双侧顶叶大部及双侧颞叶皮层代谢下降,其表现与AD不易区别.脑脊液痴呆标志物检查示,与非CAA痴呆疾病对照组患者相比较,CAA合并痴呆患者的脑脊液痴呆标志物Aβ42下降,t-tau及p-tau升高,且差异有显著性.结论:痴呆标志物的检查无法鉴别CAA合并痴呆与阿尔茨海默病.如果阿尔茨海默病患者的影像学检查提示有较多的血管性病灶时,进一步行SWI明确多发微出血,对于疑诊CAA的患者很必要.

目的:检测丁苯酞对路易体病小鼠认知功能的影响,在认知行为学改善的前提下探索其作用机制.方法:实验分为3组,即Con组、α-synuclein A53T突变的转基因小鼠组(A53T组)和NBP组(A53T突变的转基因小鼠+NBP),每组10只12月龄小鼠.NBP组腹腔注射NBP(0.2 ml/天,持续14天),Con组和A53T组腹腔注射生理盐水(0.2 ml/天,持续14天),NBP组腹腔注射NBP(0.2 ml/天,持续14天),通过旷场、条件恐惧、转棒、嗅觉检测等行为学测试.电镜观察小鼠线粒体、突触形态变化,显微镜计数小鼠黑质细胞数目,应用免疫组化检测小鼠纹状体及黑质的TH细胞以及α-synuclein含量变化.Western Blot:蛋白质印记检测线粒体自噬相关蛋白,融合分裂蛋白的变化.