多发性硬化与特发性炎性脱髓鞘疾病诊断的若干问题

多发性硬化(multiple sclerosis,MS)是中枢神经系统(CNS)特发性炎性脱髓鞘疾病(idiopathic inflammatory demyelinating diseases,IIDD)家族的代表和最重要的成员,此家族还包括MS变异型(如Marburg型急性MS、Bal6病和Schilder病)、视神经脊髓炎(NMO)、急性播散性脑脊髓炎(ADEM)、临床孤立综合征(CIS)和瘤样炎性脱髓鞘病(TIDD)等[1].     

重视中枢神经系统炎性脱髓鞘疾病的神经保护治疗

正中枢神经系统炎性脱髓鞘疾病(idiopathic inflammatory demyelinating disorders,IIDDs)是一类以累及中枢神经系统的无菌性炎性反应及其导致的组织损伤为主要特征的疾病~([1])。复发-缓解型多发性硬化(multiple sclerosis,MS)及视神经脊髓炎谱系疾病(neuromyelitisoptica spectrum disorders,NMOSD)是我国最常见的IIDDs,其他较少见的类型包括进展型MS、Marburg型MS、同心圆硬化、Schilder病及急性播散性脑脊髓炎(acute     

中枢神经系统炎性脱髓鞘病的诱发电位应用进展

正中枢神经系统炎性脱髓鞘病是在炎症基础上,一类免疫分子介导的髓鞘脱失而轴索相对保留的神经系统疾病。临床常见的有多发性硬化和视神经脊髓炎或视神经脊髓炎谱系疾病等。多发性硬化(multiple sclerosis,MS)为中枢神经系统广泛的白质脱髓鞘,累及大脑半球、小脑、脑干、脊髓和视神经等,表现为时间和空间的多发性,病程呈缓解复发。视神经脊髓炎(neuromyelitis optica,NMO)或视神经脊髓炎谱系疾病(neuromyelitis optica spectrum disorder,NMOSD)主要表     

应早期鉴别和区别治疗视神经脊髓炎与多发性硬化

视神经脊髓炎(NMO)是主要累及视神经和脊髓的中枢神经系统炎性脱髓鞘疾病。1884年首先由Devic报告,故又称为Devic’s病。在中枢神经系统炎性脱髓鞘疾病中,视神经脊髓炎在亚洲人群较为多见,而欧美人群则以经典型多发性硬化(MS)更常见。近年研究发现,中枢神经系统水通道蛋白aquaporin4(AQP4)抗体(NMOIgG)为视神经脊髓     

实验性变态反应性脑脊髓炎发病初期血脑屏障的形态学变化

<正> 多发性硬化(multiple sclerosis,MS)是以脑脊髓白质损伤为主的中枢神经系统炎性脱髓鞘疾病。实验性变态反应性脑脊髓炎(experimental allergic enceph-     

PD-1/PD-L1通路与多发性硬化

正多发性硬化(multiple sclerosis,MS)是一种以中枢神经系统(central neurology system,CNS)白质炎性脱髓鞘为主要病理特点的自身免疫病,以反复复发、缓解为主要特征,其发病受诸多因素的影响,并有多种内源性调节因子和途径参与其中。实验性自身免疫性脑脊髓炎(experimental autoimmune encephalomyelitis,EAE)是目前研究MS的经典动物模型。程     

假瘤型炎性脱髓鞘病的临床及病理

目的 :探讨假瘤型炎性脱髓病的临床病理特点。方法 :分析 5例假瘤型炎性脱髓鞘病的临床、影像及病理特点。结果 :本组临床特点 :1亚急性或慢性起病 ,逐渐进展。 2症状、体征及 MRI均提示为单一病灶。 3MRI所见病灶比 CT大 ,可有占位效应 ,也可呈周边花边样或环形强化 (例 2呈均匀强化 )。病理呈炎性脱髓鞘改变 ;白质内大片脱髓鞘区 ,血管周围淋巴细胞浸润 ,呈袖套样 ,伴有胶质细胞增生。也可主要累及灰质。结论 :1假瘤型炎症性脱髓鞘病可能是多发性硬化 (MS)中的一种特殊类型 ,或者是 MS与急性播散性脑脊髓炎之间的过渡类型。 2假瘤型炎性脱髓鞘病与胶质瘤不易鉴别 ,可试用肾上腺皮质激素治疗或者进行组织活检 ,不可急于手术。     

提高对少见中枢神经系统免疫相关疾病影像特点的综合辨别能力

中枢神经系统免疫相关疾病种类较多,多数在影像方面有自己的特点,认识和掌握这些影像学特点,对临床相应疾病的诊断与鉴别诊断有较大帮助。某些中枢神经系统免疫相关疾病,如多发性硬化、视神经脊髓炎、播散性脑脊髓炎等免疫介导的脱髓鞘病,以及结缔组织病(系统性红斑狼疮、干燥     

结缔组织疾病伴发的脱髓鞘病变

结缔组织疾病或系统性自身免疫疾病常侵及中枢神经系统(脑、脊髓),导致脱髓鞘的病理改变,其临床症状和影像特征与中枢神经系统特发性炎性脱髓鞘疾病(如多发性硬化、横贯性脊髓炎等)极为相似,从而造成诊断上的困难。因此详细询问每一位脱髓鞘病变患者有否系统性自身免疫疾病症状,行相关检查并进行随访,以早期识别并进行针对性治疗无疑具有重要的临床意义。     

蠕虫对多发性硬化动物模型的保护作用

一、多发性硬化(MS)和实验性自身免疫性脑脊髓炎多发性硬化是中枢神经系统(CNS)白质炎症脱髓鞘性自身免疫性疾病,临床主要表现为缓解复发和阶梯进展的视力、运动和平衡障碍。MS按疾病的病程分为以下几种类型:缓解复发型、原发进展和继发进展型。MS病情的进展是髓鞘抗原特异性T细胞,以髓鞘为靶,导致CNS的炎症浸润,最后导     

视频脑电图在小儿癫痫诊断中的应用

目的评价视频脑电图(video-EEG)在小儿癫诊断中的应用价值。方法对126例具有发作性症状的患儿进行连续8h的包括清醒、睡眠、诱发试验及必要的认知测验的视频脑电图监测。结果经发作期视频脑电图证实,39例初诊为癫性发作的患儿中14例(35%)为非癫性发作;15例其他症状发作中13例(86%)为非癫性发作。64例样放电患儿中51例(80%)确定发作类型,22例(34%)确定癫类型。视频脑电图可发现短暂轻微的癫发作及样放电引起的一过性认知损伤。结论视频脑电图在排除非癫性发作、确定癫性发作的类型、评价脑电-临床关系方面可提供准确可靠的证据,进一步提高癫的临床诊断水平。     

Role of glutathione in repair of free radical damage in hippocampus in vitro.

Depletion of glutathione (GSH), an intrinsic antioxidant, increases vulnerability to free radical damage in a number of cell systems. This study investigates the role of GSH in limiting electrophysiological damage and/or recovery from free radical exposure in slices of guinea pig hippocampus. Synaptic potentials (PSPs) and population spikes (PSs) were recorded from field CA1. Free radicals were generated from 0.006% peroxide through the Fenton reaction. Analysis of the input-output curves showed that peroxide treatment decreased PSPs and impaired ability of the PSPs to generate PSs as previously reported. Recovery was nearly total within a half hour. Treatment with 5 mM buthionine sulfoximine (BSO) for 2 h depleted hippocampal GSH to 79.2% of control values. The extent of free radical damage was not increased. Recovery, however, was only partial. GSH was further depleted by oxidation with diamide or covalent bonding with dimethyl fumarate (DMF) immediately before and during the peroxide treatment. Neither diamide nor DMF treatment in BSO-incubated tissue enhanced peroxide-induced electrophysiological deficits. Following these treatments, however, tissue showed little recovery from free radical damage. We conclude that glutathione is essential for repair processes in hippocampal neurons exposed to oxidative damage.     

Neuroprotective properties of memantine in different in vitro and in vivo models of excitotoxicity

The pathogenesis of stroke, trauma and chronic degenerative diseases, such as Alzheimer's disease (AD), has been linked to excitotoxic processes due to inappropriate stimulation of the N-methyl-D-aspartate receptor (NMDA-R). Attempts to use potent competitive NMDA-R antagonists as neuroprotectants have shown serious side-effects in patients. As an alternative approach, we were interested in the anti-excitotoxic properties of memantine, a well-tolerated low affinity uncompetitive NMDA-R antagonist presently used as an anti-dementia agent. We explored in a series of models of increasing complexity, whether this voltage-dependent channel blocker had neuroprotective properties at clinically relevant concentrations. As expected, memantine protected neurons in organotypic hippocampal slices or dissociated cultures from direct NMDA-induced excitotoxicity. However, low concentrations of memantine were also effective in neuronal (cortical neurons and cerebellar granule cells) stress models dependent on endogenous glutamate stimulation and mitochondrial stress, i.e. exposure to hypoxia, the mitochondrial toxin 1-methyl-4-phenylpyridinium (MPP+) or a nitric oxide (NO) donor. Furthermore, memantine reduced lethality and brain damage in vivo in a model of neonatal hypoxia-ischemia (HI). Finally, we investigated functional rescue (neuronal capacity to migrate along radial glia) by memantine in cerebellar microexplant cultures exposed to the indirect excitotoxin 3-nitropropionic acid (3-NP). Potent NMDA-R antagonists, such as (+)MK-801, are known to block neuronal migration in microexplant cultures. Interestingly, memantine significantly restored the number of neurons able to migrate out of the stressed microexplants. These findings suggest that inhibition of the NMDA-R by memantine is sufficient to block excitotoxicity, while still allowing some degree of signalling.     

Storage of lipofuscin in neurons in mucopolysaccharidosis

Summary A histochemical and ultrastructural study was made on the brain of a 23-year-old man with Sanfilippo's syndrome. In accordance with previous reports the cortical nerve cells contained a PAS-positive lipid storage substance. This showed intense autofluorescence in UV-light and was positive with various stains for lipofuscin. The storage material appeared ultrastructurally as inclusion bodies composed of short lamellated membranes, granular material, and vacuoles. In addition, concentrically and transversely lamellated membranous cytoplasmic bodies were observed in the nerve cells. It is concluded that the PAS-positive lipid storage material in the neurons was composed partly of lipofuscin in addition to other lipids presumably glycosphingolipids.Supported by a grant from the Expressen Prenatal Research Foundation