实验性轴索型GBS坐骨神经病理学研究

目的：探讨轴索型格林巴利综合征（GBS）动物模型坐骨神经的病理学改变。结果：对发病兔坐骨神经进行分离单神经、LFB镀银染色、HE染色及电镜检查。结果：分离单神经显示严重Wallerian样变性，串珠样改变；HE染色炎性细胞浸润少见；LFB镀银染色坐骨神经轴索肿胀、增粗、粗细不均、扭曲、部分破坏、断裂、消失，呈严重的轴索变性；电镜检查坐骨神经轴索固缩，体积变小，微丝、微管致密、集聚成团、线粒体固缩。严重者可见轴索膜破坏溶解，呈大小不等、形态不规则的空泡样结构、轴索移向一边或消失，髓鞘相对完整。结论：本实验证实了有轴索型GBS亚型的存在，同时也证实AMAN型GBS为一种原发性轴索变性疾病。     

急性运动性轴索型GBS的免疫发病机制研究进展

急性运动性轴索型GBS(Acute motor axonal form Guillain-Barre syndrome)的病因与前驱的空肠弯曲菌(Campylobacter jejuni,Cj)感染有密切关系,但确切机理仍不十分清楚。我国是GBS病前感染Cj的高发国家,国内急性运动性轴索型GBS(即AMAN型GBS)病人血清显示抗体达峰值期的抗Cj抗体阳性率为91．4％。目前认为Cj感染和体液免疫与AMAN型GBS的发病密切相关。现将近年来国内外对AMAN型GBS免疫学及免疫发病机制的研究进展作如下综述。     

运动轴索型格林-巴利综合征患者血清对神经轴索损害作用的研究

应用含有高效价空肠弯曲菌抗体的急性运动性轴索型格林-巴利综合征病人的血清浸泡8只在体新西兰兔坐骨神经。14天后病理检查显示4只兔的坐骨神经均有20%～30%的纤维发生轴索变性,而髓鞘相对正常。而其他神经疾病组血清、健康成人组血清做同样实验获阴性病理结果。本实验表明:AMAN 患者血清中含有致病因子,此致病因子可能是抗空肠弯曲菌抗体并介导了神经轴索的损害。     

吉兰-巴雷综合征患者人类白细胞抗原A、B的基因分型

目的 研究经典吉兰－巴雷综合征（GBS）－－急性炎性脱髓鞘性多神经根神经病（AIDP）和其亚型－－急性运动轴索型神经病（AMAN）易患性与人类白细胞抗原（HLA）－A、B等位基因分型的关系，从免疫遗传学角度探讨自身内在因素在GBS不同亚型发病中的可能作用。方法 以改良快速盐析法自静脉血中抽提基因组DNA，采用聚合酶链反应－顺序特异性引物法（PCR－SSP），结合琼脂糖凝胶电泳对31例AIDP患者、33例AMAN患者和132名健康对照进行HLA－A、B位点等位基因分型。结果 AIDP组HLA－A33频率（22．6％）较对照组（3．5％）升高，相对危险度（RR）为6．1，校正概率值Pc＝0．011；AMAN组HLA－B35、B15频率（34．5％，51．7％）较对照组（6．9％，20．8％）高，RR分别为7．1、4．1、Pc分别为0．0008、015。结论 AIDP和AMAN与健康对照比较，有着不同的HLA等位基因分布。HLA－A33与AIDP易患性相关联，HLA－B15、B35与AMAN易患性相关联。推测AIDP和AMAN发病机制、病理改变的差异与在免疫应答、调控等各环节有关键作用的HLA的型别有关。     

细胞因子在格林-巴利综合征发病机制中的研究进展

<正> 淋巴细胞和巨噬细胞参与了经典格林—巴利综合征(GBS)的发病过程。目前研究表明,巨噬细胞(MΦ)可能在GBS的新亚型—AMAN型中起重要作用,而这些炎性细胞又是细胞因子(Cytokine,CK)的主要来源。因此,CK可能参与了对雪旺细胞(SC)、髓鞘、轴索的损害。此外,越来越多的研究表明,一些CK包括一些通常引起损害的炎性CK,也可能保护外周神经系统的细胞,并帮助其修复。笔者复习近年来的文献,综述如下。     

急性运动性轴索型GBS的流行病学、病因学及临床研究进展

急性运动性轴索神经病 (Acute m otor axonal neuropa-thy,AMAN)是 Feasby等 [1 ]于 1986年首先报告并命名的。此后一些国外学者曾一度认为在我国北方发生的 GBS为中国麻痹综合征 (Chinese paralytic syndrome,CPS) [2 ] ,后又改称为 AMAN[3 ,4] ,其流行病学、病因学及临床特征与经典型GBS有不同之处 [2～ 5 ] 。目前国内外学者公认 AMAN是 GBS的一种亚型。本文复习近年来有关 AMAN型 GBS的国内外文献 ,对其流行病学、病因学、临床特征作如下综述。1.　流行病学研究进展AMAN型 GBS主要发生于中国北方 ,尤以河北省农村为高…     

急性运动轴索型多发性神经病神经电生理异常分布的研究

吉兰-巴雷综合征(Guillain-Barre syndrome．GBS)是临床上急性迟缓性瘫痪的常见病因。根据其电生理和神经病理表现．可分为急性炎症性脱髓鞘性多发性神经病(acute inflammatory demyelinating polyradiculoneuropathy．AIDP)和轴索型多发性神经病。轴索型GBS进一步分为急性运动轴索型多发性神经病(acute motor axonal neuropathy．AMAN)和     

脱髓鞘型和轴索型吉兰-巴雷综合征的临床及电生理研究

<正>根据电生理学和病理学表现,吉兰-巴雷综合征(GBS)主要分为两种亚型:(1)脱髓鞘型,即以节段性脱髓鞘为主的急性炎性脱髓鞘性多发性神经病(AIDP);(2)轴索型,即以轴索损害为主的急性运动轴索型神经病(AMAN)[1]。这两种GBS亚型的发病率不仅存在地理分布差异,而且具有不同     

不同类型Guillain-Barre’综合征患者血清中TNF-α、IL-6水平的研究

目的：探讨肿瘤坏死因子（TNF－a）、白细胞介素6（IL－6）与不同类型Guillain－Barre’综合征（GBS）发病机制的关系及临床意义。方法：采用ELISA法检测GBS组（69例）．其中AIDP组（3例）、AMAN组（38例）和其它神经系统疾病组（26例）、正常对照组（26名）的血清中TNF－α、IL－6的水平。结果：急性期AIDP组、AMAN组患者血清中TNF－α、IL-6水平明显高于两个对照组（P＜0．05）,且与病情轻重显著相关。随着临床症状的恢复．其TNF－α、IL—6水平明显下降。AIDP和A-MAN患者血清中TNF－α、IL—6水平无显著差异（P＞0．05）。结论：TNF－α、IL－6在GBS免疫发病机制中可能起重要作用,但在两种不同类型GBS发病中的作用是否存在差异尚不完全清楚。     

空肠弯曲菌抗体对兔周围神经致病作用的研究

目的探讨空肠弯曲菌（Ｃｊ）及其抗体在急性运动性轴索型格林－巴利综合征（ＡＭＡＮ）中的免疫致病作用。方法经外科手术将含与不含Ｃｊ抗体的血清滴注、浸泡两组各８只兔的坐骨神经。１４ｄ后取浸泡处的坐骨神经．进行病理检查。结果Ｃｊ抗体阳性血清致局部神经损害,５只兔的坐骨神经有２０～３０％的纤维发生轴索变性。而Ｃｊ抗体阴性血清未引起上述病变。结论Ｃｊ抗体可能是ＧＢＳ的致病因子之一并介导了神经轴索的损害。     

Denervation study of synapses of organ of corti of old world monkeys

Fine structural characteristics of synapses in the spiral organ of Corti were examined, with reference to differences between inner and outer haircell systems, and to location of neurons of origin of efferent axons. Surgical interruption of crossed olivocochlear bundle, of vestibular nerve, of facial nerve, and excision of superior cervical ganglia were used to determine the pathways of efferent axons. Interruption of the vestibular nerve near the brainstem results in degeneration of all efferent terminals on outer hair cells. Mid-line lesions at, and caudal to, the facial colliculus result in degeneration of about half of these efferent terminals. Efferent synaptic bulbs to the inner hair-cell system are small, of the order of one micron, and form type 2 junctions with afferent dendrites. They tend to have more large dense-core vesicles (about 80 nm) than the large efferent terminals of the outer hair-cell system, and appear to be the terminals of axons in the habenula perforata, which exhibit varicosities laden with large dense core vesicles. The varicosities are unaffected by excision of the superior cervical ganglia. So far as our material can reveal, it appears that the varicosities in the habenula perforata do not survive vestibular root interruption, nor do the efferent processes in the internal spiral bundle or at the base of inner hair cells. Most interestingly, the afferent processes of the inner hair-cell system, as identified for example by their relation to pre-synaptic bodies in the inner hair cells, are subject to a trans-synaptic reaction after severance of the vestibular root. They undergo a dramatic cytological transformation, characterized by increase of volume, engorgement with microtubules, microfilaments, microvesicles of various sizes, and clusters of lysosomes. Thus, both the efferent and afferent terminals of the inner hair-cell system show marked cytological differences from the corresponding terminals of the outer hair cell system.     

Mechanism of action of tubocurarine on nicotinic cholinoreceptors of neurons of the sympathetic ganglia of rats

Tubocurarine (Tc) effect on membrane currents elicited by acetylcholine (ACh) was studied in isolated superior cervical ganglion neurons of rat using patch-clamp method in the whole-cell recording mode. The "use-dependent" block of ACh current by Tc was revealed in the experiments with ACh applications, indicating that Tc blocked the channels opened by ACh. Mean lifetime of Tc-open channel complex, tau, was found to be 9.8 +/- 0.5 s (n = 7) at -50 mV and 20-24 degrees C. tau exponentially increased with membrane hyperpolarization (e-fold change in tau corresponded to the membrane potential shift by 61 mV). Inhibition of the ACh-induced current by Tc (3-30 microM/1) was completely abolished by membrane depolarization to the level of 80-100 mV. Inhibition of ACh-induced current was augmented at increased ACh doses. It is concluded that the open channel block produced by Tc is likely to be the only mechanism for Tc action on nicotinic acetylcholine receptors in superior cervical ganglion neurons of rat.     

The effect of age of onset of PD on risk of dementia

Background Dementia occurs in the majority of patients with Parkinson’s disease (PD). Late onset of PD has been reported to be associated with a higher risk for dementia. However, age at onset (AAO) and age at baseline assessment are often correlated. The aim of this study was to explore whether AAO of PD symptoms is a risk factor for dementia independent of the general effect of age. Methods Two community-based studies of PD in New York (n = 281) and Rogaland county, Norway (n = 227) and two population-based groups of healthy elderly from New York (n = 180) and Odense, Denmark (n = 2414) were followed prospectively for 3–4 years and assessed for dementia according to DSM-IIIR. All PD and control cases underwent neurological examination and were followed with neurological and neuropsychological assessments. We used Cox proportional hazards regression based on three different time scales to explore the effect of AAO of PD on risk of dementia, adjusting for age at baseline and other demographic and clinical variables. Findings In both PD groups and in the pooled analyses, there was a significant effect of age at baseline assessment on the time to develop dementia, but there was no effect of AAO independent of age itself. Consistent with these results, there was no increased relative effect of age on the time to develop dementia in PD cases compared with controls. Interpretation This study shows that it is the general effect of age, rather than AAO that is associated with incident dementia in subjects with PD. Received in revised form: 22 December 2005     

Limits of deinstitutionalization--perspectives of relatives of psychiatric patients

After a hopeful beginning, the social process of the reintegration of those with severe mental illness has come to a standstill. I am led to wonder whether "the community" really wants to live together with people suffering from severe mental illness, and if so, how closely? As long as the medical treatment of mental illness provided by the general practitioners is fundamentally deficient, as they are not able to prescribe the necessary interventions--such as out-patient psychiatric nursing, and service providers in the out-patient sector are content with offering increasingly intensive forms of care for the less seriously ill at the cost of the Social Welfare System--the reintegration of those with serious mental illness remains an illusion--which is mainly to the benefit of providers of residential care in homes and hostels.