脑桥中央髓鞘溶解症1例报告及文献复习

脑桥中央髓鞘溶解症(CPM)是一种罕见的的脱髓鞘疾病．以往仅见于尸检病理诊断被认为是致死性的疾病,随着CT、MRI等影像技术的普及,CPM的生前临床诊断已成为可能,并对CPM的病因、临床表现、影像学检查和治疗都有了进一步的认识。现报告1例经MRI证实的CPM,并结合国内文献报告的17例CPM资料进行分析。     

脑桥中央髓鞘溶解症的临床和MRI特点(8例报告)

目的 探讨脑桥中央髓鞘溶解症及脑桥外髓鞘溶解的发病原因、临床特点、治疗、疾病预后及急性期、恢复期MRI影像学特点.方法 对2002年～2006年吉林大学中日联谊医院收治的8例CPM患者的病因、临床表现、MRI影像学特点及治疗转归进行回顾性分析.结果 8例患者均有基础疾病.患者表现不同程度的意识障碍、肢体瘫痪、球麻痹;其中1例无相应的临床症状.6例患者在发病早期行头部MRI检查,表现脑桥基底部对称分布的病灶;其中的3例伴有脑桥外的髓鞘溶解,病灶主要分布在丘脑、壳核;其中的2例在发病后的1个月复查头部MRI,发现脑桥病灶有不同程度的缩小,表现T1W1低信号、T2W2高信号;丘脑、壳核的病灶表现T1 W1高信号、T2W2高信号.除1例无临床症状及1例肝移植术后患者外,其余6例一经明确诊断,均立即行激素治疗.6例患者基本恢复正常,生活可以自理.结论 肝功能损伤、慢性肾功能不全的患者容易出现脑桥中央及脑桥外的髓鞘溶解症;患者激素治疗有效;头部MRI是最有效的检查方法,是确诊的重要依据;疾病恢复期丘脑、壳核病灶的短T1、长T2的影像学改变考虑与髓鞘溶解后的脂类堆积有关.     

脑桥中央髓鞘溶解症(附一例报告并文献复习)

脑桥中央髓鞘溶解症(CPM)是一种临床少见的继发性脱髓鞘疾病,早期诊断较困难.本文报道经MRI确诊的CPM 1例.     

肝硬化、高血糖合并脑桥中央髓鞘溶解症1例报告

正脑桥中央髓鞘溶解症(central pontine myelinolysis,CPM)是一种累及脑桥的渗透性髓鞘溶解,CPM病例最早见于1959年Adams报道,随着医学影像学的发展,使得该病得以更多的在临床中被报道~([1])。现将我院收治的1例在发病1 m及46 d分别进行过头颅核磁共振(magnetic resonance imaging,MRI)的病例报道如下,分析CPM头颅MRI的动态演变。     

脑桥中央髓鞘溶解症1例报道

目的提高脑桥中央髓鞘溶解症(CPM)的认识,总结CPM的临床特点及治疗方法。方法对收治的1例CPM患者的临床资料进行回顾性分析,并复习相关文献。结果患者女性,因饮食欠佳导致低钠血症,补钠过程中出现CPM。头颅MRI检查示病灶为以脑桥中央病变为主的对称性长T1、稍长T2信号影。经激素等治疗后患者预后良好。结论对于电解质紊乱,尤其低钠血症的患者补钠过程中需注意补钠速度,MRI对于CPM诊断具有较高价值,激素治疗可能有一定的疗效。     

肝豆状核变性合并中央髓鞘溶解症的临床和影像学特点(附2例报告)

目的 探讨肝豆状核变性(WD)合并脑桥中央髓鞘溶解症(CPM)的临床及头颅MRI特点.方法 回顾性分析2例出现脑桥中央髓鞘溶解的WD患者的临床资料,总结其临床特征并进行文献复习.结果 2例WD患者基础状态均较差,在妊娠反复呕吐及严重吞咽困难后,出现急性的延髓麻痹加重及四肢瘫痪;MRI在T2序列上有脑桥中央部大致对称圆形...     

脑桥中央髓鞘溶解症(附2例报告)

<正>脑桥中央髓鞘溶解症(centralpontinemyelinoly-sis,CPM)是一种少见的脱髓鞘疾病,以往仅见于尸检病理诊断,常为致死性疾病。随着CT、MRI应用的普及和对此症的认识加深,CPM生前临床诊断已成为可能,预后亦有改观。现报告2例经头部MRI证实的CPM,并结合国内文献报道的临床资料进行讨论。     

脑桥中央髓鞘溶解症MRI与临床

目的：观察MRI在脑桥中央髓鞘溶解症（CPM）早期诊断中的作用。方法：报告4例CPM,对其MRI、临床诊断、治疗、预后及发病机制进行回顾和分析。结果：4例CPM均有慢性消耗疾病、电解质紊乱或营养不良的基础;具有闭锁综合征表现,MRI示脑桥基底部对称分布的T_1长T_2信号的脱髓鞘改变仅1例,桥脑与脑其他部位同时存在脱髓鞘改变3例。经缓慢矫正电解质紊乱,给激素、脱水剂等治疗,3例好转,1例死亡。结论：CPM好发于有电解质紊乱或（和）营养不良的疾病,矫正电解质紊乱宜缓慢。脑MRI对本病早期诊断是最有效的检查手段,病因和发病机制未阐明。     

橄榄-脑桥-小脑萎缩与“十字征”八例

目的探讨MRI脑桥十字征的橄榄-脑桥-小脑萎缩(OPCA)患者临床与影像学特点。方法总结8例经临床诊断为OPCA,分析其临床特点及影像学特征表现。结果 8例患者的临床表现均以小脑性共济失调和脑干功能受损伴有自主神经功能障碍为主,MRI在脑桥轴位T2WI上均出现典型的"十字征"。结论以小脑性共济失调和脑干功能受损为主要表现的OPCA患者,多伴有自主神经功能障碍,脑桥十字征是MRI特征性表现之一,有助于该病的诊断。     

原发性干燥综合征引起脑桥中央髓鞘溶解症的临床与影像学特征分析

目的：探讨原发性干燥综合征（pSS）引起的脑桥中央髓鞘溶解综合征（CPM）的临床及影像学特征。方法：分析1例pSS引起的CPM临床资料并与国外报道的1例pSS患者进行比较。结果：pSS患者表现为吞咽困难、构音障碍及四肢瘫痪，MRI示桥脑异常信号；给予甲泼尼龙冲击冶疗后，症状明显缓解。国外报道的1例表现为四肢瘫痪和缄默症，MRI示桥脑异常信号，给予甲泼尼龙及静脉注射丙种球蛋白治疗后，症状缓解。结论：pSS引起的CPM是极罕见疾病，经免疫抑制治疗可获得良好预后。     

Formalin-induced astrocyte glutamate-glutamine cycle involved in rat spinal cord central sensitization

BACKGROUND： Central sensitization, a state of increased excitability of nociceptive neurons in the spinal dorsal horn following peripheral tissue injury and/or inflammation, is an important mechanism underlying hyperalgesia and neuropathic pain. Participation of the glutamate-glutamine cycle in central sensitization of the spinal cord remains poorly understood. OBJECTIVE： To determine whether the astrocyte-neuronal glutamate-glutamine cycle is involved in formalin-induced central sensitization in the spinal cord. DESIGN, TIME AND SETTING： A randomized, controlled, animal experiment was performed at the Institute of Orthopedics, Second Hospital, Lanzhou University, China from September 2007 to August 2008. MATERIALS： Methionine sulfoximine （MSO, 0.1 mmol/L）, glutamine （0.25 mmol/L）, and formalin were used for this study. METHODS： A total of 43 male, Sprague Dawley rats, aged 4 months, were randomly assigned to a sham operation group （n = 6） and a model group （n = 37）. Rats in the model group received intrathecal infusion in the spinal cord. 7 days later, 37 model rats were randomly divided into PBS, MSO, glutamine, MSO ＋ glutamine and formalin subcutaneous injection alone groups. The PBS, MSO, glutamine, MSO ＋ glutamine groups were respectively intrathecally injected with PBS, MSO, glutamine, MSO ＋ glutamine （50 μL each）, and then infused with 10 μL of saline. Rats from the sham operation group were not subjected to intrathecal infusion in the spinal cord. At 15 minutes after intrathecal injection, a rat model of formalin-induced inflammatory pain was established by subcutaneous injection of 5% formalin （50 μL） in the left hindpaw. MAIN OUTCOME MEASURES： Changes in spontaneous nociceptive behavior （licking/biting or flinching） were observed following formalin injection into the rat hindpaw. RESULTS： Compared with the PBS group, duration of licking/biting was significantly shortened, and flinching frequency was significantly diminished in the MSO group （P 〈 0.05）. Compared with the MSO group, duration of licking/biting was significantly prolonged, and flinching frequency was significantly increased in the MSO ＋ glutamine group （P 〈 0.05）. There was no significant difference in inflammatory pain behaviors among the sham operation, PBS, glutamine, MSO ＋ glutamine, and formalin subcutaneous injection alone groups （P 〉 0.05）. CONCLUSION： The astrocyte-neuronal glutamate-glutamine cycle in the spinal cord was shown to be involved in central sensitization induced by formalin subcutaneous injection into the hindpaw.     

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.     

正常成人吞咽时的脑激活区域：功能磁共振初步研究

BACKGROUND： While brain-imaging studies in healthy adults have indicated that multiple cortical regions are involved in swallowing, these functional imaging techniques have not been extensively applied to the complete understand neurophysiology of swallowing in China. A full understanding of normal swallowing neurophysiology is important for improving functional outcomes for dysphagia due to neurologic disorders or damage with increasing age. Thus the interpretations of the functional contributions of various brain areas in swallowing should be scientifically researched. OBJECTIVE： To identify the activation and characteristic of swallowing center in healthy adults using functional magnetic resonance imaging. DESIGN, TIME AND SETTING： An uncontrolled neuroimaging study was performed at the Outpatient Clinic, Department of Radiology, West China Hospital of Sichuan University between March and November 2008. PARTICIPANTS： Ten healthy right-handed volunteers, aged over 20 years with a mean age of （34.2 ＋ 8.1） years, a range of 25-45 years and including five males and five females participated. A medical history was obtained from all potential subjects and all subjects were free of systemic diseases and neurological disorders. METHODS： The healthy volunteers were examined with event-related functional magnetic resonance imaging of blood oxygenation level-dependent while laryngeal swallow-related movements were recorded. Subjects were scanned during voluntary saliva swallowing and water bolus swallowing activation tasks. Data was processed using the General Linear Model. A voxel by voxel group comparison was performed using random effect analysis. Any cluster with a corrected P 〈 0.05 for spatial extent was considered significant. MAIN OUTCOME MEASURES： The cerebral cortical activation maps of voluntary swallowing of saliva and swallowing of water bolus in healthy adults were observed. RESULTS： A multifocal cortical representation of swallowing was in the precentral gyrus, postcentral gyrus, insula, anterior cingulate gyrus, thalamus, basal ganglia and cerebellum, in a bilateral and asymmetrical manner, predominantly on the left hemisphere in the volunteers （P 〈 0.05）. CONCLUSION： Activation of the cortex during normal swallowing tasks may be functionally linked to basal nuclei, thalamus, and cerebellum, greatly appearing in the left hemisphere.     

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。     

Total saponins of Panax ginseng effects on proliferation and differentiation of human embryonic neural stem cells and in a Parkinson’s disease mouse model

BACKGROUND： Total saponins of Panax ginseng （TSPG） exhibits neuroprotection against Parkinson＇s disease in the substantia nigra. OBJECTIVE： To investigate the effects of TSPG on human embryonic neural stem cells （NSCs） proliferation and differentiation into dopaminergic neurons using in vitro studies, and to observe NSC differentiation in a mouse model of Parkinson＇s disease, as well as behavioral changes before and after transplantation. DESIGN, TIME AND SETTING： In vitro neural cell biology trial and in vivo randomized, controlled animal trial were performed at the Institute of Basic Medical Sciences, Chongqing Medical University between September 2004 and December 2007. MATERIALS： TSPG （purity 〉 95%） was isolated, extracted, and identified by Chongqing Academy of Chinese Materia Medica. Recombinant human basic fibroblast growth factor （bFGF） and recombinant human epidermal growth factor （EGF） were purchased from PeproTech, USA. A total of 25 C57/BL6J mice, aged 18-20 weeks were included. Twenty were used to establish a Parkinson＇s disease model with i.p. injection of MPTP （1-methyl-4-phenyl-1, 2, 3, 6-tetrahydropyridine） and TSPG alone or combined with interleukin-1 （IL-1）-treated NSCs prior to transplantation into the corpus striatum. The remaining five mice were pretreated for 3 days with TSPG prior to MPTP injection, serving as the TSPG prevention group. METHODS： Primary NSCs were isolated, cultured and purified from embryonic cerebral cortex. Immunocytochemistry was employed to detect specific antigen expression in the NSCs. In vitro experiment： （1） to induce proliferation, NSCs were treated with TSPG, EGF＋bFGF, or TSPG＋EGF＋bFGF, respectively; （2） to induce dopaminergic neuronal differentiation, NSCs were treated with TSPG, IL-1, or TSPG＋IL-1, respectively. MAIN OUTCOME MEASURES： In vitro experiment： the effects of TSPG on NSCs proliferation were evaluated with flow cytometry and MTT assay. Tyrosine hydroxylase expression was determined by immunocytochemistry assay to observe effects of TSPG on dopaminergic neuronal differentiation. In vivo experiment： differentiation of grafted NSCs in the mouse brain was determined by immunohistochemical staining. Behavioral changes were evaluated by spontaneous activity frequency, memory function, and score of paralysis agitans. RESULTS： （1） NSCs were cultured and passaged for more than three passages. Immunocytochemistry revealed positive nestin staining, as well as neurofilament protein and glial fibrillary acidic protein. （2） TSPG significantly increased NSC proliferation, in particular when combined with EGF and bFGF, which was twice as effective as FGF or bFGF alone. TSPG also induced dopaminergic differentiation in NSCs, in particular when TSPG was added together with IL-1, resulting in an effect five times greater than that of IL-1 alone. （3） At day 30 following transplantation, most NSCs in the TSPG prevention group differentiated into dopaminergic neurons, and the scores of paralysis agitans, spontaneous activity, and memory function were significantly increased compared with TSPG alone or TSPG＋IL-1 groups （P 〈 0.05）. CONCLUSION： TSPG stimulated NSC proliferation, in particular when combined with FGF and bFGF. TSPG significantly induced dopaminergic neuronal differentiation of NSCs, and the effect was greater when combined with IL-1. In addition, TSPG greatly improved behavior in the Parkinson＇s disease mouse model following NSC transplantation. Following NSC transplantation, TSPG pretreatment exhibited superior efficacy over either TSPG alone or TSPG in combination with IL-1, in terms of behavioral improvements in the Parkinson＇s disease mouse model.     

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.     

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.     

Wnt信号通路与骨髓间充质干细胞增殖及分化的关系

骨髓间充质干细胞（bonemarrow—derived mesenchymal stem cells,BMSCs）是骨髓中不同于造血干细胞的一类细胞,其来源丰富,取材简便,易分离、纯化、培养,在一定的条件下可以迅速体外扩增,具有多向分化潜能,可以通过不同的方法被诱导分化成骨细胞、软骨细胞、肌细胞、神经胶质细胞、神经元细胞等,而且它具有低免疫源性,向病变部位迁移的能力,     

Monoaminergic and catecholaminergic activation of the central pattern generator for locomotion following spinal cord injury

The development of secondary health complications following spinal cord injury has been increasingly recognized by healthcare professionals as a major concern. These problems most specifically affect complete or near-complete spinal cord injury patients （e.g., those with minimal mobility）, who are not typically rehabilitated with treadmill training approaches, because motor control and leg movements are largely impaired. However, recent pharmaceutical advances in central pattern generator activation may provide new therapeutic hopes for these spinal cord injury patients. This article provides a comprehensive overview, for the non-specialist, of the most recent advances in this field.     

墨蝶呤还原酶基因与精神分裂症相关性的研究进展

墨蝶呤还原酶（SPR）催化四氢生物蝶呤（BH4）从头合成途径的最后一步反应。SPR基因遗传缺陷或突变可导致BH。的合成紊乱,影响单胺类神经递质（如多巴胺、5-羟色胺及谷氨酸等）的合成或释放,进而参与包括精神分裂症在内的多种神经精神系统疾病的发生发展过程。此外,SPR基因敲除小鼠表现出持续增强的自主活动等类精神分裂症症状,说明该基因在精神分裂症的发病中扮演重要的角色。进一步研究SPR基因及其单核苷酸多态性的功能,可为阐明精神分裂症的发病机制提供重要的线索,也为新一代抗精神病药物的研制及开发开拓新的视野。现对SPR基因与精神分裂症的相关研究做一综述。