青年额颞叶痴呆1例报告

<正>额颞叶痴呆(FTD)是中老年患者缓慢出现人格改变、言语障碍及行为异常,影像学上表现为额颞叶萎缩的一种痴呆综合征。我科收治1例青年额颞叶痴呆患者,报告如下。1病例男,32岁,医生,因"性格、行为及言语异常2年"于2009年7月18日入院。2年前患者家人发现其性格孤僻,寡言少语,行为懒散,逐渐进展为放纵、鲁莽行为。1年前开车冲上人行道撞死1人,但自认为无过错,表情冷漠。同年11     

阿尔茨海默病的昨天、今天和明天:痴呆研究的历史、现状与展望

1906年11月3日,德国病理学家Alois Alzheimer首次在图宾根(Tübingen)举行的第37届德国西南精神病学年会上报告了一例56岁患严重记忆障碍女性患者的病理所见,即在其脑组织中发现大量"神经炎性斑[NPs,即老年斑(SPs)]"和"神经原纤维缠结(NFTs)",4年后德国著名精神病学家Emil Kraepelin将由此种病理改变引起的临床症状与体征命名为"阿尔茨海默病(AD)",从此在医学及社会学的字典中增加了一个沉重的名词,揭开了人类研究阿尔茨海默病的序幕[1-3].     

痴呆-肌萎缩侧索硬化1例报道

额颞痴呆(frontotemporal dementia,FTD)是一种隐袭起病、逐渐进展的非Alzheimer型早老性痴呆,其病理特征为额叶和颞叶前部的进行性变性;肌萎缩侧索硬化(amyotrophic lateral sclerosis,ALS)是最常见的成人起病的运动神经元病(motor neuron diease,MND),其病理特征为上、下运动神经元的进行性消失。国外已有许多这一疾病组合(FTD-ALS)的临床与病理报告。现将我们见到的1例报告如下。病例资料患者男性,49岁。住院号:432965。因“智力下降9个月,言语含糊2个月,肌肉萎缩1个月”于2004年10月12日入院。2004年1月起患者简单计算出错。2月…     

国产与进口奥氮平治疗痴呆患者精神行为症状的对照研究

<正>本研究比较国产(商品名:欧兰宁)与进口奥氮平(商品名:再普乐)治疗阿尔兹海默病(AD)及血管性痴呆(VD)患者的精神行为症状(BPSD)的疗效、安全性,报告如下。1对象和方法为2012年6月至2013年12月我院老年病房住院的痴呆患者60例,诊断符合中国精神障碍分类与诊断标准第3版。入组前患者痴呆病理行为评定量表(BEHAVE-AD)总     

精神行为异常合并癫痫发作的女性青年麻痹性痴呆1例

以精神行为异常起病的麻痹性痴呆需要与相关精神疾病鉴别。本文报告1例女性青年患者,以间断性精神行为异常合并癫痫发作的麻痹性痴呆临床资料及其诊疗过程,供临床医生参考。患者女,29岁因性格改变、行为异常就诊于精神专科医院,病情反复,后因合并癫痫发作,就诊于神经内科,行血液及脑脊液梅毒相关抗体检查、简易智力状态评定量表检查、头部MRI等相关检查,诊断为麻痹性痴呆,予青霉素治疗,症状有所好转。本病例发病年龄轻,神经系统症状出现早,提示临床医生要注意年轻患者梅毒的早期筛查,做到早发现、早治疗。     

多动秽语综合症

一、历史回顾早在1885年法国 Georges Gilles ae laTourelte 医生(1857—1904)因报告9例多动秽语综合征(以下简称 GTS)而一举成名,其中有1例系法国贵族妇女 Marguise de Dampiene,她七岁发病,因奇特的发音和姿势难以进行社会交往,被迫幽居,直到85岁死去。其实她的症状早在60年之前,由法国另一位医生 Ttard 于1825     

中国26个常染色体显性遗传性脑动脉病伴皮质下梗死和白质脑病家系的临床和影像学特点及与其他国家患者的对比

目的 分析我国常染色体显性遗传性脑动脉病伴皮质下梗死和白质脑病(CADASIL)患者的临床和影像学特点,并与其他国家的患者进行对比.方法 收集2003年1月至2009年10月在我院经Notch3基因和超微病理检查证实的26个CADASIL家系中102例患者的发病年龄、首发症状和临床表现特点,与德国、日本、沙特、法国患者的症状用x2检验进行比较;分析35例患者头颅MRI改变特点并与英国以及法国患者进行对比.结果 102例患者发病年龄在22～80岁,平均(43.9±11.0)岁,缺血性脑卒中或TIA出现率(79.41%)与其他国家患者差异无统计学意义;痴呆出现率(50.00%)高于沙特(21.05%,x2=5.513,P=0.020)和法国(31.11%,x2=4.517,P=0.034)患者;精神异常出现率(14.71%)较德国患者低(30.39%,x2=7.185,P=0.007);偏头痛出现率(13.73%)和法国患者类似,明显少于日本(40%,x2=12.658,P=0.000)、德国(38.24%,x2=15.932,P=0.000)和沙特患者(42.11%,x2=6.869,P=0.009).35例患者头MRI均存在异常改变,没有发现延髓损害,腔隙性梗死在基底节的发生率(82.86%)明显较法国患者(60%,x2=5.663,P=0.017)高;大脑白质疏松发生率与法国患者相似;颞极白质受累率(68.57%)明显少于英国患者(95%,x2=5.211,P=0.022).结论 不同国家CADASIL的临床表现和MRI改变存在差异.双颞极白质异常信号对国人CADASIL的诊断不是一个敏感指标.     

Alzheimer病3例报告：附1例病理报告

Alzheimer 病过去称为早老性痴呆,以后经大量的临床与病理研究认为,老年性痴呆多数亦属于此病。随着我国人口寿命不断增长,此病亦将成为老年医学的一个重要问题。现将我们遇见的3例(包括经尸检证实1例)报告如下:病例报告例1:男性,63岁,干部,因精神异常9年、肺部炎症入院。患者于12年前开始记忆力减退,智力下降,9年来经常无目的游走,回答不切题。5年前开始不认识熟人,说不准     

利培酮口服液治疗老年期痴呆患者病理性行为对照研究

目的：对具有病理性行为的老年期痴呆患者分别给予利培酮和氟哌啶醇治疗的疗效进行比较。方法：65例伴有病理性行为的老年期痴呆患者随机分为研究组33例和对照组32例,分别给予利培酮口服液和氟哌啶醇针剂治疗。疗程2周。于治疗前及治疗2h、24h、72h、1周和2周采用痴呆病理行为评定量表（BEHAVE-AD）和治疗中出现的症状量表（TESS）评定疗效及不良反应,并观察服药依从性。结果：利培酮口服液与氟哌啶醇针剂疗效相仿（P〉0.05）,但利培酮不良反应更小,依从性更好（P〈0.05或P〈0.01）。结论：利培酮口服液更适用于老年期痴呆患者的病理性行为的治疗。     

以痴呆为主要表现的麻痹性痴呆、HIV相关性痴呆和克雅病的临床特征分析

目的探讨并分析梅毒所致麻痹性痴呆(麻痹性痴呆)、HIV相关性痴呆和克雅病等中枢系统感染性疾病所致痴呆的临床特征。方法检索19例患者(麻痹性痴呆8例、HIV相关性痴呆6例、克雅病5例)临床资料,回顾分析其临床表现、实验室检查、脑电图、神经影像学及治疗转归特点。结果 3组患者临床表现均以认知损害为主,并广泛累及多系统(锥体系、锥体外系、小脑)及多组脑神经。辅助检查显示,麻痹性痴呆患者快速血浆反应素环状卡片试验和苍白密螺旋体抗体明胶颗粒凝集试验阳性(8例),脑脊液美国性病研究实验室试验阳性(4例),MRI呈现不同程度脑萎缩(6例);HIV相关性痴呆患者血清HIV抗体筛选试验及Western blotting检测阳性(6例),脑脊液平均蛋白定量明显升高(2例)、潘氏试验阳性(2例),MRI以脑内多发占位病变或大片异常密度影为特征;克雅病患者脑脊液Western blotting检测1433蛋白阳性(4例),脑电波呈弥漫性慢波(4例)伴典型三相波(1例),散发型患者MRI脑叶皮质区沟、回呈肿胀样改变(3例),变异型患者可伴丘脑"曲棍球样"改变(1例)。结论麻痹性痴呆、HIV相关性痴呆及克雅病等中枢系统感染性疾病所致痴呆临床表现复杂多样,诊断时应结合患者病史、实验室血清学和脑脊液指标,以及脑电图和神经影像学表现等综合考虑,明确诊断。     

ApoE与阿尔茨海默病

载脂蛋白E（ApoE）基因型是现在所知的散发型和迟发型阿尔茨海默症的最大的危险因子,它是中枢神经系统中主要的胆固醇运载体,同时又是极低密度脂蛋白（VLDL）的组成成分。ApoE对脑内胆固醇代谢异常和β-淀粉样蛋白沉积等阿尔茨海默症的典型特征有重要作用,对于ApoE在阿尔茨海默症中生化作用机制的研究对于我们对其发病机理的深入认识及治疗有重要意义。     

What is ‘early onset dementia’?

There are two types of dementia with early onset: (i) presenile dementias; and (ii) senile dementias with early onset. Most patients who develop dementia before 65 years of age have Alzheimer's disease (AD). The remainder are likely to have vascular dementia (VaD), frontotemporal dementia, head injury, alcohol intoxication, or metabolic disorder. Presenile dementias, caused by frontotemporal lobar degeneration, progressive supranuclear palsy, and corticobasal degeneration, usually occur in patients of presenile and are rarely seen in patients of senile age. Although the factors responsible for the accelarted onset of the illness are not fully known, genetic abnormalities appear to be important in some types of presenile dementia, such as frontotemporal dementia with parkinsonism linked to chromosome 17. Conversely, senile dementias such as sporadic AD and VaD commonly occur in patients of senile age. These disorders may also occur in patients of presenile age, although less frequently. Alzheimer's disease was originally classified as a ‘presenile dementia’. Since the 1980s, ‘senile dementia of Alzheimer type’ (SDAT) and ‘Alzheimer's disease’ have been considered to belong to the same pathological entity and both are now known as ‘dementia of Alzheimer's type (DAT)’ or merely ‘Alzheimer's disease’. Rapid progression of cognitive impairment with neuropsychological syndromes and neurological symptoms has been considered a characteristic of early onset AD. However, recently, neurological symptoms such as spastic paraparesis, seizures, and myoclonic convulsions have been reported to occur infrequently in early onset AD, although language problems and visuospatial dysfunctions are common. There are at least three dominant genes that have been identified in cases of familial Alzheimer's disease with early onset, namely the amyloid precursor gene (APP), and the genes encoding presenilin 1 (PSEN1) and presenilin 2 (PSEN2). Therefore, genetic abnormalities are important factors contributing to the earlier onset of the illness. It is also important to investigate the pathophysiological mechanism in relation to genetic abnormalities, environmental factors, physical illnesses, and metabolic disturbances to understand the processes underlying the development of dementia with early onset.     

脑老化及相关神经疾病新进展

与脑老化相关的主要疾病包括阿尔茨海默病、帕金森病及淀粉样脑血管病。随着人口老龄化趋势的进展,此类疾病已经成为危害我国老年人群健康的重大疾病之一。尽管,目前脑老化机制及相关神经疾病的发病原因尚不十分清楚,但其蛋白质变性、异常聚集的病理学研究,以及轻度认知损害、痴呆和慢性进行性运动障碍等疾病的临床前期或早期功能影像学和生物学标志研究已成为老年神经科学领域的主要焦点之一。     

Dissociation of neuropathologic findings and cognition: case report of an apolipoprotein E epsilon2/epsilon2 genotype

BACKGROUND: The apolipoprotein E (APOE) epsilon2 allele has been suggested as having a protective effect and delaying the age at onset of Alzheimer disease. OBJECTIVE: To describe a dissociation between neuropathologic findings with normal cognition in a woman with severe Alzheimer disease with the APOE epsilon2/epsilon2 genotype. DESIGN: Case report from a community-based prospective study of persons 90 years or older (The 90+ Study). PARTICIPANT: A 92-year-old woman without dementia with the APOE epsilon2/epsilon2 genotype who lived independently without significant cognitive or functional loss and was a participant in The 90+ Study. She died in December 2004, and postmortem examination of her brain was performed. INTERVENTION: Neurologic examination and a battery of neuropsychological tests were performed 6 months and 1 month before death. Neuropathologic examination included Braak and Braak staging for senile plaques and neurofibrillary tangles. RESULTS: Neuropathologic examination of the brain revealed advanced senile plaque and neurofibrillary tangle disease consistent with a high likelihood of Alzheimer disease. At clinical evaluation, the participant demonstrated no dementia and only mild cognitive deficits. CONCLUSIONS: The APOE genotype may have contributed to maintenance of cognition despite advanced neuropathologic findings of Alzheimer disease. This case suggests that the APOE epsilon2 isoform may have a protective effect against cognitive decline in Alzheimer disease that may be independent from senile plaques and neurofibrillary tangles.     

PET显像在阿尔茨海默病的应用进展

阿尔茨海默病是老年期痴呆之临床常见类型,随着发病率的逐年升高,受到社会各界更多的关注。PET功能显像可通过各种显像剂敏感、特异性地反映阿尔茨海默病的病理生理学改变,笔者将重点介绍18F-FDG显像剂、淀粉样蛋白和神经原纤维缠结tau蛋白显像剂在阿尔茨海默病中的应用,详细阐述PET显像在阿尔茨海默病病因学研究、早期诊断、鉴别诊断、疾病转归和药物治疗中的作用。     

Mild cognitive impairment

Great interest is now devoted to elderly people with memory or other cognitive complaints who are not demented. The determination of this impairment from normality is difficult, because memory performance may decline slowly along the lifetime of the individual. On the other hand, the identification of dementia depends on the criteria used for dementia (DSM-IV or ICD-10). Furthermore, cognitive deterioration of the elderly appears to be heterogenous and may forerun not only Alzheimer’s disease but also other forms of dementia. By applying a set of criteria for frontotemporal mild cognitive impairment, it was possible to identify, retrospectively, a series of patients with behavioral, affective, or speech symptoms suggestive of frontotemporal dysfunction and deficits in frontal lobe-dependent neuropsychological tests, but who have maintained activities of daily living and are not demented. These patients appear to have a high probability of progressing subsequently to dementia of the frontotemporal type. Several potential neuroprotective compounds are now being subjected to clinical trials. Should they be effective in delaying the progression to dementia, the need to detect and treat elderly people with cognitive impairment will become very important.     

A historical review of the concept of vascular dementia: lessons from the past for the future

The history of senile dementia begins in the Greco-Roman period with basic concepts of senility by Pythagoras and Hippocrates. During the Middle Ages, the main contribution was by Roger Bacon in 1290. The first textbook of neurology, De cerebri morbis, by Jaso de Pratis (1549), included a chapter on dementia ("De memoriae detrimento"). In the 17th century, Thomas Willis recognized intellectual loss with aging. In the 19th century, Philippe Pinel removed chains from the mentally ill; his student Esquirol wrote the first modern classification of mental disease, including senile dementia. In 1860, Morel recognized brain atrophy with aging. The modern history of vascular dementia began in 1896, when Emil Kraepelin in his textbook Psychiatrie included "arteriosclerotic dementia" among the senile dementias, following the ideas of Otto Binswanger and Alois Alzheimer, who had differentiated clinically and pathologically arteriosclerotic brain lesions from senile dementia and from neurosyphilitic general paresis of the insane. Binswanger's and Alzheimer's contributions are reviewed in detail.     

阿尔茨海默病神经影像学研究进展

阿尔茨海默病是临床最常见的老年期痴呆类型。在当今老龄化社会中,阿尔茨海默病发病率呈逐年升高之趋势,且治疗效果较差,给社会带来巨大的经济负担。建立在早期诊断基础上的临床干预可以延缓阿尔茨海默病进展,改善患者预后,本文拟对阿尔茨海默病早期诊断的神经影像学进展进行综述。     

农村老年痴呆患者精神行为症状及相关因素调查

目的:探讨农村老年痴呆患者精神行为症状(BPSD)的特点及其相关因素。方法:应用阿尔茨海默病病理行为评分量表对77例上海青浦区农村地区老年痴呆患者的病理性行为进行评定,并分析其相关因素。结果:老年痴呆患者BPSD发生率为89.6%,其中以行为紊乱、攻击性行为、日夜节律紊乱和偏执与妄想多见。BPSD与性别、年龄、文化程度和病程无相关,轻、中度痴呆患者偏执和妄想症状较重度明显,阿尔茨海默病和血管性痴呆BPSD相似。结论:农村老年痴呆患者BPSD发生率较高。