亚急性炎性脱髓鞘性多发神经根神经病的临床和病理

研究亚急性炎性脱髓鞘多发性神经根神经病的临床和病理特点。方法对６例亚急性炎性脱髓鞘多发性神经根神经病进行临床、腓肠神经活检光镜和电镜研究。结果患者的临床高峰在４～８周。临床表现主要为中等度瘫痪不伴呼吸衰竭，激素治疗有效。３例腓肠神经活检显示为脱髓鞘伴有单核细胞浸润。结论由于临床和病理结果与急性和慢性格林－巴利综合征不同，这些患者应考虑诊断亚急性炎性脱髓鞘多发性神经根神经病，以区别急性炎性脱髓鞘多发性神经根神经病和慢性炎性脱髓鞘多发性神经根神经病。     

炎性脱髓鞘性周围神经病的电生理诊断

炎性脱髓鞘性周围神经病包括一组疾病,其中以急性炎性脱髓鞘性多发性神经根神经病(acute inflammatory demyelinating polyradiculoneuropathy,AIDP)和慢性炎性脱髓鞘性多发性神经根神经病(chronic inflammatory demyelinating polyradiculoneuropathy,CIDP)较为常见.     

慢性炎性脱髓鞘性多发神经根神经病误诊为颈椎病3例临床分析

<正> 慢性炎性脱髓鞘性多发神经根神经病(Chronic inflamma-tory demyelinating polyradicuroneuropathy,CIDP)是以周围神经和神经根病变为主的自身免疫性脱髓鞘疾病,临床表现复杂,早期极易误诊为颈椎病或其它疾病。本文报告3例CIDP入院前都曾被误诊为颈椎病。临床资料 例1,女,53岁。因四肢末端感觉异常1年、无     

慢性炎性脱髓鞘性多发性神经根神经病诊治中国专家共识2022

本共识经该领域内的中国专家反复讨论而成稿, 在前一版慢性炎性脱髓鞘性多发性神经根神经病诊治指南的基础上, 结合近年来的诊断和治疗新进展, 进行了更新。内容包括慢性炎性脱髓鞘性多发性神经根神经病的临床特点、神经电生理、脑脊液、周围神经影像、腓肠神经病理和抗体检测, 以及诊断标准和治疗要点。     

亚急性炎性脱髓鞘性多发性神经根神经病的临床和病理研究(附3例病理报告)

目的 探讨亚急性炎性脱髓鞘性多发性神经根神经病（SIDP）的临床和病理特点。方法 对7例SIDP患者临床资料和腓肠神经活检所见进行分析。结果 患者呈亚急性起病，达到高峰时间均在4周－2个月；运动障碍较感觉障碍明显，上升性呼吸肌麻痹很少发生，对激素治疗有效且无复发。3例腓肠神经活检，病理显示：以脱髓鞘为主要表现，伴有单核细胞浸润，胶原纤维增生，但无洋葱样肥大神经改变。结论 SIDP不论临床表现还是病理特点都不同于急、慢性炎性脱髓鞘性多发性神经根神经病（AIDP、CIDP），而是介于两者之间。     

以拉力参数构建兔腰骶神经牵拉损伤模型***★◆

背景：神经根牵拉损伤是腰椎手术失败综合征的近期发病原因之一，但确切的病理机制尚未经证实。 目的：建立一种不同程度的腰骶神经根牵拉损伤动物模型，探讨脊髓、神经根牵拉损伤后光镜和电镜改变。 设计、时间及地点：随机对照，形态学动物实验，于2004-04/2006-10在南通大学第二附属医院中心实验室和南通大学江苏省神经再生重点实验室完成。 材料：清洁级健康12周龄雄性大白兔40只，随机分为对照组、轻度牵拉组、中度牵拉组、重度牵拉组4组，每组10只。 方法：轻、中、重度牵拉组动物全椎板切除显露双侧S1神经根，用测力神经根拉钩分别以0.4，1和1.8 N的拉力水平牵拉左侧S1神经根造成神经根和脊髓的牵拉性损伤，分别定义为轻、中和重度牵拉，时间持续10 min。对照组不牵拉。 主要观察指标：术后2周对受牵拉节段脊髓，两侧S1神经根行光镜和透射电镜检测神经元和髓鞘。 结果：轻度牵拉组牵拉侧前角个别神经元稍肿胀，神经元及神经纤维形态基本上正常，个别白质小片状脱髓鞘样改变，神经根髓鞘和轴索轻度水肿，脱髓鞘不明显；中度牵拉组白质有脱髓鞘改变，神经纤维排列紊乱，轴索崩解、断裂；灰质神经元水肿明显加重，前角运动神经原细胞尼氏体消失，核固缩、深染，神经根髓鞘和轴索水肿，局部轻度脱髓鞘改变；重度牵拉组牵拉侧白质为不规则的大片脱髓鞘区，前角运动神经元胞体固缩、变形、核深染，神经根有髓神经纤维萎缩、数目减少,重度脱髓鞘改变。 结论：以拉力为参数可以稳定地建立不同程度的腰骶神经根牵拉性损伤动物模型，术后神经根和脊髓的病理分级改变与牵拉损伤程度一致。     

急性发作的复发-缓解型慢性炎性脱髓鞘性多发性神经根神经病1例报告并文献复习

正临床上,急性发作的复发-缓解型慢性炎性脱髓鞘性多发性神经根神经病与复发型格林-巴利综合征的鉴别诊断常较困难。然而,由于治疗方案和预后的不同,许多实验研究仍致力于寻找能够区分二者的诊断标准。本文报道1例病程酷似复发型格林-巴利综合征的多次急性发作的复发-缓解型慢性炎性脱髓鞘性多发性神经根神经病,并复习其与复发型格林-巴利综合征的鉴别诊断。     

直立性低血压为临床表现的吉兰-巴雷综合征个案报道

吉兰-巴雷综合征(Guillain-Balle syndrome,GBS)又称急性炎性脱髓鞘性多发性神经根神经炎,是一种由细胞免疫和体液免疫共同介导的周围神经脱髓鞘疾病.自主神经功能紊乱为GBS常见的及重要的并发症,发生率达2/3[1];但以自主神经功能紊乱为唯一表现的GBS文献中很少报道.我科于2012年5月22日收治1例以直立性低血压为唯一临床表现的GBS,报道如下.     

慢性炎性脱髓鞘性多神经根神经病

本文介绍近年国外对慢性炎性脱髓鞘性多神经根神经病的临床表现、实验诊断、诊断标准、治疗及预后等的研究进展。     

多灶性运动神经病7例分析

目的 探讨多灶性运动神经病MMN的临床特点、神经电生理及病理变化的特异性.方法 结合文献分析7例MMN的临床资料、肌电图、病理特征及治疗结果.结果 MMN的临床表现为慢性、非对称性远端肢体无力;电生理检查以运动神经传导阻滞或部分阻滞为特点;病理学特征为运动神经局灶性脱髓鞘性改变.结论 MMN应与运动神经元病和慢性炎性脱髓鞘性多发性神经根神经病鉴别.可采用免疫球蛋白治疗.     

Environmental factors in the development and progression of late-onset Alzheimer＇s disease

Late-onset Alzheimer＇s disease （LOAD） is an age-related neurodegenerative disorder characterized by gradual loss of synapses and neurons, but its pathogenesis remains to be clarified. Neurons live in an environment constituted by neurons themselves and glial cells. In this review, we propose that the neuronal degeneration in the AD brain is partially caused by diverse environmental factors. We first discuss various environmental stresses and the corresponding responses at different levels. Then we propose some mechanisms underlying the specific pathological changes, in particular, hypothalamic-pituitary adrenal axis dysfunction at the systemic level; cerebrovascular dysfunction, metal toxicity, glial activation, and Aβ toxicity at the intercellular level; and kinase-phosphatase imbalance and epigenetic modification at the intracellular level. Finally, we discuss the possibility of developing new strategies for the prevention and treatment of LOAD from the perspective of environmental stress. We conclude that environmental factors play a significant role in the development of LOAD through multiple pathological mechanisms.     

Disrupted structural and functional brain connectomes in mild cognitive impairment and Alzheimer＇s disease

Alzheimer＇s disease （AD） is the most common type of dementia, comprising an estimated 60-80% of all dementia cases. It is clinically characterized by impairments of memory and other cognitive functions. Previous studies have demonstrated that these impairments are associated with abnormal structural and functional connections among brain regions, leading to a disconnection concept of AD. With the advent of a combination of non-invasive neuroimaging （structural magnetic resonance imaging （MRI）, diffusion MRI, and functional MRI） and neurophysiological techniques （electroencephalography and magnetoencephaJography） with graph theoretical analysis, recent studies have shown that patients with AD and mild cognitive impairment （MCI）, the prodromal stage of AD, exhibit disrupted topological organization in large-scale brain networks （i.e., connectomics） and that this disruption is significantly correlated with the decline of cognitive functions. In this review, we summarize the recent progress of brain connectomics in AD and MCI, focusing on the changes in the topological organization of large-scale structural and functional brain networks using graph theoretical approaches. Based on the two different perspectives of information segregation and integration, the literature reviewed here suggests that AD and MCI are associated with disrupted segregation and integration in brain networks. Thus, these connectomics studies open up a new window for understanding the pathophysiological mechanisms of AD and demonstrate the potential to uncover imaging biomarkers for clinical diagnosis and treatment evaluation for this disease.     

Edema and neuronal apoptosis in the hippocampus and cortex of elderly rats following transient cerebral ischemia/reperfusion injury

BACKGROUND： Previous studies of cerebral ischemia have used young animals, with an ischemic time greater than 5 minutes （safe time limit）. Despite an increased understanding of neuronal apoptosis, it remains uncertain whether brief cerebral ischemic events of 5 minutes or less damage brain tissue in elderly rodents. OBJECTIVE： To investigate the effects of transient cerebral ischemia （5 minutes）/reperfusion injury on brain cortical and hippocampal edema, aquaporin-4 （AQP-4） expression, and neuronal apoptosis in aged rats, and to compare ischemic sensitivity between cortex and hippocampus. DESIGN, TIME AND SETTING： A randomized, controlled, animal experiment was performed at the Institute of Cerebrovascular Disease, Qingdao University Medical School from April 2008 to March 2009. MATERIALS： Rabbit anti-AQP-4 polyclonal antibody, TUNEL kit, and SABC immunohistochemistry kit were purchased from Wuhan Boster Bioengineering, China. METHODS： A total of 160 healthy, male, aged 19-21 months, Wistar rats were randomly assigned to 4 groups： sham-surgery, and ischemia 1-, 3-, and 5-minute groups, with 40 rats in each group. The global cerebral ischemia model was established using the Pusinelli four-vessel occlusion, and the three cerebral ischemia groups were subdivided into reperfusion 12-hour, 1-, 2-, 3-, and 7-day subgroups, with 8 rats in each subgroup. The sham-surgery group was subjected to exposure of the first cervical bilateral alar foramina and bilateral common carotid arteries. MAIN OUTCOME MEASURES： The dry-wet weight assay was used to measure brain water content and histopathology of the cortex and hippocampus was observed following hematoxylin-eosin staining. In addition, cortical and hippocampal AQP-4 expression was detected by streptavidin-biotin complex immunohistochemistry, and neuronal apoptosis was detected by the TUNEL method. RESULTS： There was no significant difference in brain water content or AQP-4 expression in the cortex and hippocampus between ischemia 1- and 3-minute groups and the sham-surgery group or brain water content or AQP-4 expression in the cortex between ischemia 5-minute group and sham-surgery group （P 〉 0.05）. However, brain water content and AQP-4 expression in the hippocampus after 5 minutes of cerebral ischemia were significantly increased compared with the sham-surgery group （P 〈 0.05 or P 〈 0.01）. Several TUNEL-positive cells were observed in the cortex and hippocampus of the sham-surgery group and ischemia 1-minute group, as well as in the cortex of the ischemia 3-minute group. In addition, the number of apoptotic neurons in the hippocampus of ischemia 3-minute group and in the cortex and hippocampus of ischemia 5-minute group was significantly increased （P 〈 0.05 or P 〈 0.01 ）. Neuronal apoptosis was increased after 12 hours of ischemia/reperfusion, and it reached a peak by 2 days （P 〈 0.01）. CONCLUSION： Transient cerebral ischemia （5 minutes） resulted in increased hippocampal edema, AQP-4 expression, and neuronal apoptosis. Moreover, cerebral ischemia had a greater effect on neuronal apoptosis than brain edema or AQP-4 expression, and the hippocampus was more sensitive than the cortex.     

雷公藤内酯对癫痫大鼠神经元P13K／Akt／mTOR蛋白的影响

目的通过检测癫痫大鼠海马神经元P13K、Akt和mTOR蛋白表达,探讨雷公藤内酯抑制癫痫大鼠神经元凋亡的分子机制。方法30只大鼠随机分为对照组、海人酸组、雷公藤内酯干预组,免疫组化法检测各组大鼠海马神经元P13K、Akt和mTOR蛋白的表达情况。结果海人酸组神经元胞体皱缩,形态不规则,数量减少,而雷公藤内酯干预组神经元的数量和形态与对照组相似,海人酸组海马神经元P13K、Akt、ITITOR蛋白表达与对照组比较均减少,而雷公藤内酯干预组海马神经元的P13K、Akt、mTOR蛋白表达均较海人酸组增加,差异均有统计学意义（P〈0．05）。结论雷公藤内酯可能通过上调P13K／Akt／mTOR信号通路蛋白表达对癫痫大鼠海马神经元发挥保护作用。     

Dysfunctional autophagy in Alzheimer＇s disease： pathogenic roles and therapeutic implications

Neuronal autophagy is essential for neuronal survival and the maintenance of neuronal homeostasis. Increasing evidence has implicated autophagic dysfunction in the pathogenesis of Alzheimer＇s disease （AD）. The mechanisms underlying autophagic failure in AD involve several steps, from autophagosome formation to degradation. The effect of modulating autophagy is context-dependent. Stimulation of autophagy is not always beneficial. During the implementation of therapies that modulate autophagy, the nature of the autophagic defect, the timing of intervention, and the optimal level and duration of modulation should be fully considered.     

高血压脑出血的显微外科治疗进展

高血压脑出血（Hypertensive intrac-rebral hemorrhage,HICH）是具有高发病率、高病死率、高致残率的急性脑血管疾病,占所有脑卒中患者的10%-20%,早期病死率可高达49.4%。随着人口老龄化,其发病率逐年提高;而外科手术的干预,使其病死率有所下降,但致残率居高不下。如何提高手术疗效和患者生存质量,一直是神经外科医师努力的方向。微侵袭血肿清除术因其手术创伤小,恢复快,是目前国内治疗高血压脑出血的重要手段。     

Oxidative stress in Alzheimer＇s disease

Oxidative stress plays a significant role in the pathogenesis of Alzheimer＇s disease （AD）, a devastating disease of the elderly. The brain is more vulnerable than other organs to oxidative stress, and most of the components of neurons （lipids, proteins, and nucleic acids） can be oxidized in AD due to mitochondrial dysfunction, increased metal levels, inflammation, and β-amyloid （Aβ） peptides. Oxidative stress participates in the development of AD by promoting Aβ deposition, tau hyperphosphorylation, and the subsequent loss of synapses and neurons. The relationship between oxidative stress and AD suggests that oxidative stress is an essential part of the pathological process, and antioxidants may be useful for AD treatment.     

神经内镜联合亚低温治疗高血压基底节区脑出血

目的 探讨神经内镜联合亚低温在治疗高血压基底节区脑出血中的临床应用价值.方法 回顾性分析我院神经内镜治疗高血压基底节区脑出血患者40例的临床资料,并对治疗结果进行分析.结果 神经内镜治疗组22例(甲组),神经内镜联合亚低温治疗组18例(乙组),术后3个月根据GCS评分,甲组恢复良好1例,中残4例,重残6例,植物生存6例,死亡5例;乙组恢复良好4例,中残8例,重残3例,植物生存1例,死亡2例,两组比较差异有统计学意义(P＜0.05).两组颅内压比较第1天两者差异不明显,但第2、3天亚低温组颅内压明显降低.结论 神经内镜是治疗高血压基底节区脑出血较为有效的手术方式,联合亚低温治疗能有效降低颅内压,改善术后神经功能恢复,具有较好的临床应用价值.     

Targeting 13-secretase with RNAi in neural stem cells for Alzheimer＇s disease therapy

There are several major pathological changes in Alzheimer＇s disease, including apoptosis of cho- linergic neurons, overactivity or overexpression of 13-site amyloid precursor protein cleaving enzyme 1 （BACE1） and inflammation. In this study, we synthesized a 19-nt oligonucleotide targeting BACE1, the key enzyme in amyloid beta protein （AI3） production, and introduced it into the pSilenCircle vector to construct a short hairpin （shRNA） expression plasmid against the BACE1 gene. We transfected this vector into C17.2 neural stem cells and primary neural stem cells, resulting in downregulation of the BACE1 gene, which in turn induced a considerable reduction in reducing AI3 protein production. We anticipate that this technique combining cell transplantation and gene ther- apy will open up novel therapeutic avenues for Alzheimer＇s disease, particularly because it can be used to simultaneously target several pathogenetic changes in the disease.