回顾性比较Poser标准和McDonald标准

目的比较Poser标准和McDonald标准在多发性硬化诊断中的差异。方法对80例新诊断为多发性硬化患者的临床资料进行回顾性分析,分别应用Poser标准和McDonald标准对其进行诊断分类。结果80例患者中48例(60.00%)行脊髓MRI检查,阳性率为83.33%(40/48)。应用Poser标准,41例(51.25%)为临床确诊,10例(12.50%)为实验室确诊,17例(21.25%)为临床可能,余12例(15.00%)未能进行诊断分类。应用McDonald标准,45例(56.25%)为确诊多发性硬化,35例(43.75%)为可能多发性硬化。经Poser标准临床确诊的41例患者中,除4例不完全符合McDonald标准中确诊多发性硬化条件外,其余37例均符合;实验室确诊的10例患者中,4例符合McDonald标准中确诊多发性硬化条件,其余6例为可能多发性硬化。经McDonald标准确诊的45例患者中,37例符合Poser标准中临床确诊条件,4例为实验室确诊,1例为临床可能,余3例为原发进展型多发性硬化。18例患者(33.33%)脑脊液寡克隆区带检测阳性,26例(40.63%)IgG合成率升高。4例患者脑脊液寡克隆区带或IgG检测呈阳性反应且MRI病灶≥2个,经McDonald标准诊断为确诊多发性硬化。结论McDonald标准较Poser标准更为完善,尤其适用于原发进展型多发性硬化及临床孤立综合征患者。同时应用脊髓MRI检查以及脑脊液寡克隆区带和IgG检测有利于提高McDonald标准诊断的敏感性。     

McDonald多发性硬化诊断标准的应用性评价

多发性硬化（multiple sclerosis，MS）的诊断主要基于中枢神经系统病灶在时间上和空间上多发性的临床证据，且需除外可引起这些损害的其他疾病。目前被广泛应用的诊断标准有两种，即1983年提出的Poser标准和2001年提出的McDonald标准。McDonald标准 ^[1]是建立在Poser标准基础上，但更注意利用包括MRI在内的相关实验室检查来证明多发性硬化在时间和空间上的多发性，     

脑脊液寡克隆区带和核磁共振检查对多发性硬化的临床诊断价值

目的 :探讨脑脊液寡克隆区带CSFOB和头颅MRI的变化对诊断多发性硬化 (MS)的临床价值。方法 :对 2 5例怀疑多发性硬化 (MS)的患者进行脑脊液的寡克隆区带和头颅MRI的检查 ,研究两者和MS间的关系。结果 :确诊多发性硬化的患者均有脑脊液的寡克隆带和头颅核磁共振 (头MRI)异常。结论 :脑脊液寡克隆带和头MRI两者结合对诊断发硬化 (MS)有重要临床价值。     

McDonald标准对多发性硬化的诊断意义

目的 研究McDonald标准对多发性硬化(MS)的诊断意义.方法 对47例MS患者,其中视神经脊髓型MS(OSMS)17例、传统型MS(CMS)30例,进行病史收集、查体及MRI,诱发电位、脑脊液检查.运用McDonald标准对MS患者进行诊断.采用Fisher精确检验对两亚型诊断结果 进行比较.结果 OSMS亚组符合McDonald标准确诊MS 16例(94.1%)、可能MS1例(5.9%),CMS亚组符合确诊MS 20例(66.7%)、可能MS 10例(33.3%),两亚组确诊比率的差异有统计学意义(OR=0.1250,95%CI:0.0144～1.083,P<0.05).其中,不需附加证据而符合临床确诊的OSMS患者为16例,而CMS患者为17例,另3例需要结合MRI和脑脊液证据后确诊.结论 McDonald标准诊断OSMS的准确性高于CMS,尤其在确诊MS时更明显.其原因可能与该标准确诊OSMS时主要依赖临床症状和体征,而对CMS需要严格的MRI证据的规定有关.     

诊断为脊髓型多发性硬化的脊髓疾病附68例临床分析

目的 应用新的多发性硬化和视神经脊髓炎诊断标准,回顾分析以往被诊断为“脊髓型多发性硬化”的病例,探讨与主要累及脊髓的脱髓鞘疾病相鉴别的重要疾病类型.方法 应用2010年新修订的McDonald多发性硬化诊断标准,以及2006年Wingerchuk视神经脊髓炎的诊断标准,回顾分析我院1994年～2012年之间曾被诊断为“脊髓型多发性硬化”的患者68例,并进行随访.结果 仅17.65％的患者完全符合McDonald标准,多数脊髓型多发性硬化患者最终转变为视神经脊髓炎,其它疾病如脊髓血管病和系统性自身免疫病也易被误诊为脊髓型多发性硬化.结论 对于孤立的脊髓综合征应该按照一定的诊疗规范进行诊断、鉴别和随访,不建议再使用脊髓型多发性硬化的名称.     

多发性硬化的临床诊断

多发性硬化是中枢神经系统炎性脱髓鞘疾病.目前病因未阐明,发病机制与自身免疫性反应有关.临床表现的显著特点为时间多发性(多次发作)和空间多发性(多个病变部位).常用辅助检查手段包括头部或脊髓MRI、脑脊液免疫学、诱发电位等.最新的诊断标准为2010年McDonald标准.目前认为,多发性硬化与视神经脊髓炎在临床、病理、影像等方面不同,由于该病临床表现的多样性及缺乏特异性生物学诊断指标,其诊断仍然是临床医师面临的最大难题之一.     

脑脊液寡克隆区带和核磁共振检查对多发性硬化的临床诊断价值

目的：探讨脑脊液寡克隆区带CSFOB和头颅MRI的变化对诊断多发性硬化（MS）的临床价值。方法：对25例怀疑多发性硬化（MS）的患者进行脑脊液的寡克隆区带和头颅MRI的检查，研究两者和MS间的关系。结果：确诊多发性硬化的患者均有脑脊液的寡克隆带和头颅核磁共振（头MRI）异常。结论：脑脊液寡克隆带和头MRI两者结合对诊断发硬化（MS）有重要监床价值。     

水通道蛋白4抗体阳性多发性硬化临床特点分析

目的分析水通道蛋白4(AQP4)抗体阳性多发性硬化的临床特征。方法共18例多发性硬化患者,均符合Mc Donald诊断标准(2010年版),分为AQP4抗体阳性组(5例)和AQP4抗体阴性组(13例),并门诊随访。结果 5例AQP4抗体阳性多发性硬化患者,男性2例、女性3例,中位发病年龄43岁,中位病程4个月;脊髓病变和视神经受累常见;MRI显示脑组织广泛多发性长T1、长T2异常信号伴颈胸髓长T1、长T2异常信号(3/5例)或单纯颈胸髓多发性长T1、长T2异常信号(2/5例);脑脊液Ig G指数(4/4例)、24 h Ig G鞘内合成率(3/4例)升高,寡克隆区带阳性(3/4例);血清抗核抗体阳性(2/5例)或合并干燥综合征(1/5例)。结论 AQP4抗体阳性多发性硬化有其特异性,发病机制可能与经典多发性硬化不同,在临床诊断与治疗时需加以重视并区别处理。     

多发性硬化42例临床分析

目的 探讨多发性硬化(MS)的临床特点、诊断和治疗.方法 分析42例多发性硬化患者的一般资料、病变部位、主要症状、重要辅助检查及其治疗方法和效果.结果 42例MS患者中青壮年女性多见,以急性、亚急性起病为主,首发症状以肢体无力最常见,病变部位以脊髓和视神经最常见,实验室检查以脑脊液蛋白水平和IgG指数增高常见.MRI异常率高达85.96%.结论 MS是一种临床表现复杂、累及中枢神经系统白质,多部位、多时相的自身免疫性疾病.根据临床特点、神经电生理、脑脊液免疫学及磁共振成像检查能提高临床确诊率,激素治疗对大多数病人有效.     

伴有周围神经病变的多发性硬化

目的分析5例伴周围神经病变的多发性硬化的临床电生理、实验室检查和MRI改变,探讨多发性硬化合并周围神经损害的临床特点和发病机制。方法回顾性总结5例伴发周围神经病损的多发性硬化的脑脊液生化,免疫学检查、寡克隆带,电生理检查,MRI及其它有助于鉴别诊断的检查。结果5例均为伴有周围神经病变的多发性硬化,电生理提示广泛周围神经病损,既有脱髓鞘改变,又有轴突变性。结论由于中枢神经系统与周围神经系统髓鞘存在着共同的抗原性成分,故MS患者的中枢神经系统与周围神经系统可以同时发生脱髓鞘改变。     

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.     

Summary of Legislation of 1933 of interest to the Department of Mental Hygiene

Psychiatric Quarterly -