Marchiafava-Bignami病1例报告

Marchiafava-Bignami病(MBD)是一种罕见的大脑白质脱髓鞘性疾病,以胼胝体受累为突出表现,是导致胼胝体变性、渐进性脱髓鞘和坏死的中毒性疾病,大多见于慢性酒精中毒的患者.     

合并皮质受累的胼胝体变性1例报道及国内报道荟萃分析

该文对1例合并皮质受累的胼胝体变性进行了报道,同时将该病例与国内文献检索出的27篇共50例胼胝体变性病例一起作临床特点的分析。在51例胼胝体变性病例中,49例有长期大量饮酒史。胼胝体变性临床表现复杂,难以定位,其中急性发病者19例,多表现为癫痫发作、意识水平下降;亚急性起病者13例,表现为精神异常、反应迟钝、行走不稳、尿便障碍等;慢性发病者19例,表现为言语不清、精神障碍、反应迟钝、肢体无力等。影像学表现为特征性胼胝体脱髓鞘改变,CT表现为胼胝体膝部或压部低密度灶,磁共振表现为相应部位脱髓鞘改变。胼胝体变性如能早期诊断与治疗,大部分患者预后良好。     

复发型伴胼胝体压部可逆性病变的轻度脑炎/脑病Ⅱ型一例

伴胼胝体压部可逆性病变的轻度脑炎/脑病临床、影像表现特异。根据病变累及范围分为Ⅰ型和Ⅱ型,临床以Ⅰ型多见,Ⅱ型少见。文中报道1例罕见的复发型伴胼胝体压部可逆性病变的轻度脑炎/脑病Ⅱ型患者。该例患者首次发病表现为典型的Ⅱ型伴胼胝体压部可逆性病变的轻度脑炎/脑病,累及胼胝体及深部脑白质,短期复查病变消失;2年后病变复发,病变累及范围与第一次相似,短期复查病变消失。结合相关文献对其临床及影像学表现进行分析,以加深对该病的认识,提高诊治水平。     

成人可逆性胼胝体压部病变综合征临床特点及预后的比较性分析

目的对成人可逆性胼胝体压部病变综合征(RESLES)的成因,病程,MRI表现及预后特点进行分析,以系统性总结评估成人RESLES临床特点及预后。方法回顾性分析成人RESLES患者的临床及影像资料。参照改良牛津残障量表(MOHS)对患者的临床结局进行评分,并据此将患者分为预后良好组(MOHS≤2)及预后不良组(MOHS≥3),比较性分析两组临床影像特点。结果共纳入8例患者,RESLES的疾病谱包括轻度脑炎/脑病,Marchiafava-Bignami病,抗癫痫突然撤药相关脑病及代谢性脑病等。MRI特征性表现为胼胝体压部高信号,伴或不伴胼胝体外,其他胼胝体部位受累,复查MRI提示胼胝体压部高信号完全消失或明显减弱。发病过程中伴有严重意识障碍及胼胝体外病灶等因素在两组的差异有统计学意义(P0.05)。结论成人RESLES是一临床少见的临床影像综合症,其涉及疾病谱广泛。虽然RESLES患者的临床症状均趋于缓解,但病程中有昏迷症状及伴有胼胝体外病灶的患者预后不良。     

治疗后迅速缓解的Marchiafava-Bignami病1例报告

正Marchiafava-Bignami病(MBD)是一种酒精中毒相关的疾病,可导致严重的神经系统症状,例如:快速进展的痴呆,癫痫,精神症状等~([1])。MBD的病理改变主要是中枢神经系统脱髓鞘及坏死,主要累及胼胝体部位。该病最典型的特征是核磁共振影像表现,在急性期,病变部位在T2WI/FLAIR/DWI像上都能表现为高信号~([2])。MBD的典型病灶位于胼胝体,除此之外还能累及脑内其他部位如:脑叶,皮质下,脑白质及基底节区~([3])。当病灶广泛累及胼胝体以外的其他部位时,常常提示病情严重和预后不良~([4])。本文介绍了1例核磁共振提示广泛病灶的MBD,经过治疗以后临床症状迅速改善,影像学病灶也相应缓解。     

药物致急性脱髓鞘脑病3例临床分析

目的探讨药物致急性脱髓鞘脑病的各项临床特征及其可能相关原因.方法报道3例病前1-3 d服用不明成份药物,且通过影像学检查临床诊断为急性脱髓鞘脑病的病例,综合相关研究文献,分析其临床表现、辅助检查、治疗及预后等情况.结果药物致急性脱髓鞘脑病患者轻者可出现多项精神状态检查异常,重者在短时间内出现意识障碍,缺乏明确的中枢神经系统定位体征;脑电图及地形图表现为弥漫性低功率慢波;头颅核磁共振成像MRI表现为大脑白质区域广泛对称的长或略长T1信号、长T2信号,相应的弥散加权成像显示高信号;激素治疗后部分患者好转,部分患者预后较差,长期随访无复发.结论药物致急性脱髓鞘脑病呈单向病程,突出表现为高级中枢功能障碍,头颅MRI检查对于明确诊断及预后判断较为重要,脑电图及地形图检查在一定程度上反映出弥漫性脑功能的减退,预后可能与个体差异、病变范围及程度、并发症等因素有关.     

胼胝体变性六例影像学特点和临床表现

目的 探讨胼胝体变性(Marchiafava-Bignami disease,MBD)的影像学特点和临床表现.方法 对我院诊治的6例MBD患者进行回顾性分析,包括影像学检查(如头颅CT、MRI)、实验室检查、临床症状和随访情况.结果 6例患者MRI检查均表现为胼胝体肿胀及T1WI等或低信号,T2WI高信号,液体衰减反转恢复序列高信号,弥散加权成像示弥散受限.其中例1、例4和例6以急性意识障碍起病,CT和MRI检查除累及整个胼胝体外双侧半卵圆区也受累,预后较差.例2和例3以反应迟钝、记忆力下降起病,CT和MRI检查病变主要累及胼胝体膝部和压部,经积极治疗,患者基本恢复正常.例5以双下肢麻木无力、反应迟钝起病,MRI检查病变累及胼胝体压部及体部,经治疗后也恢复正常.结论 MBD临床表现复杂,但具有特征性影像学表现,其起病形式和影像学特征可作为反映其预后的重要因素之一.     

急性播散性脑脊髓炎的临床、MRI表现及治疗

目的研究急性播散性脑脊髓炎（ADEM）的临床特点。方法对2例临床确诊为ADEM患者进行回顾及随访．并对临床和影像学表现等进行分析。结果神经系统症状多样,影像学表现：主要累及脑白质的脱髓鞘病变,应用激素治疗有效。结论ADEM是一种中枢神经系统炎性脱髓鞘疾病,临床表现多样。MRI表现有一定的特征性,MRI检查有利于早期诊断,也是评价疗效的重要手段。     

临床常见脑病的临床及影像学特点分析

目的探讨临床常见的脑病,包括代谢、中毒及放射性脑病等临床常见脑病的临床及影像学表现特点。方法收集作者医院收治的临床常见脑病患者43例,其中中毒性脑病17例(包括装修苯中毒1例、鼠药氟乙酰胺中毒1例、化疗药物中毒5例、有机磷农药中毒4例、一氧化碳中毒5例、海洛因中毒1例)、放射性脑病5例及代谢性脑病21例(包括Wernick脑病2例、低血糖脑病4例、肝性脑病4例、尿毒症性脑病5例、桥本脑病3例、甲基丙二酸尿症1例、卟啉病1例、伴有囊肿及钙化的白质脑病1例),总结分析临床常见脑病的临床及影像学特点。结果 (1)临床表现:不同病因脑病患者多表现为不同程度的认知功能障碍、精神行为异常、言语含糊及计算力减退等,部分患者表现为肢体力弱或无力,少数患者表现为语音障碍、眼球固定、发作性肢体抽搐及腹痛等。(1)表现为认知功能障碍:包括不同程度的反应迟钝伴记忆力减退(脑病中苯中毒1例、有机磷农药中毒2例、化疗药物中毒5例、一氧化碳中毒2例、放射性脑病5例、尿毒症性脑病5例、桥本脑病3例),以及不同程度的意识障碍(有机磷农药中毒2例、一氧化碳中毒3例、低血糖脑病2例、肝性脑病4例);(2)精神行为异常(桥本脑病3例、甲基丙二酸尿症1例);(3)表现为言语含糊伴计算力减退(海洛因脑病1例);(4)部分患者表现为肢体力弱或无力,少数患者表现为语音障碍、眼球固定、发作性肢体抽搐及腹痛等,其中包括放射性脑病3例、低血糖脑病2例、甲基丙二酸尿症1例、卟啉病1例、伴有囊肿及钙化的白质脑病1例表现为肢体力弱,Wernick脑病2例表现为进行性声音嘶哑伴双下肢力弱及眼球固定,苯中毒及鼠药氟乙酰胺中毒各1例表现为发作性肢体抽搐,卟啉病1例表现为发作性腹痛、肢体无力伴言语不清。(2)影像学表现:影像学表现病变主要累及白质、基底节核团,少数可累及脑室旁及导水管区域,仅1例表现为广泛性脑萎缩。病变表现以白质脑病为主者包括苯中毒、鼠药氟乙酰胺中毒、化疗药物中毒、有机磷农药中毒、一氧化碳中毒、海洛因中毒、尿毒症性脑病及桥本脑病患者;伴钙化与囊变的脑白质病患者表现为双侧大脑半球脑白质区多发大小不等囊状病灶;以基底节核团病变为主者包括低血糖性脑病、肝性脑病、卟啉病、有机磷农药中毒性脑病,一氧化碳中毒迟发性脑病及海洛因中毒性脑病;Wernick脑病患者病灶多位于三脑室旁、侧脑室旁及导水管周围;甲基丙二酸尿症1例表现为广泛脑萎缩。结论临床上常见类型的脑病病因多样,均有不同程度的认知功能障碍,影像学表现包括广泛的白质脱髓鞘、皮层萎缩及基底节区病变等,诊断应结合临床、影像学特点及相应的病史进行诊断。     

双侧丘脑病变:1例病例报告与综述

目的：提高对以双侧丘脑病变为主要表现的神经系统疾病的临床认知水平。方法：报道1例以“行走不稳伴言语异常”为主要临床表现,具有双侧丘脑病变患者的诊疗经过,同时结合文献,重点针对其病因及临床表现等方面的相关研究进展进行综述。结果：该例丘脑病变患者以意识障碍、言语行走功能障碍为主要表现,经过相关影像学检查在双侧丘脑发现对称病灶,最终诊断为后循环脑梗死,基底动脉尖综合征。结论：双侧丘脑病变临床上并不罕见,临床表现受病变部位、其他大脑组织部位有否合并受累等影响。其病因众多,包括肿瘤、感染或脱髓鞘疾病、代谢或中毒性疾病以及需要及时干预的血管性疾病。因缺乏具特征性的临床症状表现,为临床诊断及鉴别带来一定困难。因此,早期明确丘脑病变病因尤为重要,以期能够早诊断早治疗,本文在报导病例的同时就其病因和临床表现等方面的研究进展进行综述。     

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 -