Creutzfeldt—Jakob病1例报告

Ｃｒｅｕｔｚｆｅｌｄｔ Ｊａｋｏｂ病 (ＣＪＤ)自 2 0世纪 80年代首例报告以来 ,至今我国已报道 4 5例 ,我科 1991年收治 1例经尸解病理证实的ＣＪＤ患者。现报告如下。1　病例　女 ,6 0岁 ,因智力减退、运动障碍 5个月于 1991年4月 18日入院。患者于 1990年 10月末与家人生气后 ,出现少言寡语 ,反应迟钝、表情淡漠、精神不振 ,逐渐问话不答 ,常突发大笑、不自主皱眉、奴嘴、反复摆弄手指、尿裤子等 ,生活不能自理。 1991年 1月患者出现双下肢无力 ,四肢抽动 ,每日 3～ 4次 ,每次数分钟。逐渐四肢僵硬 ,卧床不起 ,不知饮食 ,进入昏迷。曾经…     

Creutzfeldt－Jakob病临床、病理、免疫组化及动物传递的实验研究

经病理证实为Ｃｒｅｕｔｚｆｅｌｄｔ－Ｊａｋｏｂ病（ＣＪＤ）９例，急性发病４例，亚急性４例，慢性１例。平均病程８．８个月。４例脑切片免疫组化染色阳性。另有３例脑活检组织匀浆接种于Ｃ５７ＢＬ／６０ｌａ，Ｂａｌｂ／ｃ和Ｗｉｓｔａｒ鼠脑内。分别于第二、三代发病。资料提示：（１）国人ＣＪＤ急性发病较多；（２）蛋白酶抗性蛋白免疫组化十分敏感；（３）ＣＪＤ脑组织活检可以成功地进行动物接种。     

国人Creutzfeldt—Jakob病PrP基因表达变化的研究

目的探讨国人朊病毒病PrP基因变异与临床病理变化的关系及同欧亚病例的对比。方法取5例散发型Creutzfeldt－Jakob病（CJD）患者静脉血提取DNA，以多聚酶联反应（PCR）扩增PrP基因，用7种限制性内切酶酶切密码子102、105、129、145、178、180、200、219和232位点及DNA序列测定，并设14例非CJD痴呆和16例健康人为对照。结果发现3组中各1例具密码子129多态性，为缬氨酸／甲硫氨酸杂合子，其余均为甲硫氨酸纯合子，无缬氨酸129纯合子。结论国人CJD患者PrP基因变异及其相关的临床特征与欧洲患者不同。     

Creutzfeldt—Jakob病与Alzheimer病并存

本文报告１例病程长达１２年，经组织病理与免疫组化染色方法证实的Ｃｒｅｕｔｚｆｅｌｄｔ－Ｊａｋｏｂ病与Ａｌｚｈｅｉｍｅｒ病并存病例。     

Creutzfeldt-Jakob病的免疫组织化学研究

４例Ｃｒｅｕｔｚｆｅｌｄｔ－Ｊａｋｏｂ病（ＣＪＤ）的新鲜脑组织石腊切片用改进的免疫组织化学染色，均呈突触型阳性。不仅证实诊断正确，而且为研究国人ＣＪＤ的分子生物学与临床病理的联系提供了依据。     

Creutzfeldt—Jakob病一例报道

CJD为Creutzfeldt—Jakobdisease的简称，又称皮质纹状体脊髓变性，是由具有感染性的蛋白质一朊蛋白（prionprotein，PrP）引起。CJD多在中年以后发病，以进行性痴呆、肌阵挛、锥体束征或锥体外系症状为主要临床表现，病理表现为大脑海绵状变性、神经细胞脱失及星形胶质细胞增生，免疫组化PrPsc阳性。本病预后极差，死亡率100％。临床较少见，且容易误诊，现将我院收治的1例病例报道如下。     

中国与奥地利Creutzfeldt-Jakob病的比较研究

目的 为更准确认识中国人Creutzfeldt-Jakob病（CJD）特殊性，比较中国和奥地利两国CJD在临床、病理等方面异同点。方法 对中国人CJD24例，奥地利人CJD79例，通过组织病理、免疫组化，实验动物传递、临床类型、病程经过等多方面进行比较。结果 （1）两国CJD报道例数有逐年增多趋势；（2）奥地利人绝大多数为剖检证实，中国人多为脑活检确定；（3）异常PrP沉积形式分突触型、空泡周围型和斑块型三种，奥地利人分别为71％、38％和10％；中国人分别为92％、8％和0％；（4）奥地利人突触型多在灰质密集分布，而中国人则为松散分布；（5）两国均发现年轻人和中年人发生的散发性CJD，但均无新变异型CJD。结论 两国CJD确实存在若干异同处，认清此点，对早期发现CJD，减少医源性传播有积极意义。     

Creutzfeldt-Jakob病(附国内45例临床病理分析)

目的:进一步认识Creutzfeldt-Jakob病(CJD)的临床和病理特征,探讨诊断方法。方法:对1例患者的临床症状、脑电图表现、脑活检病解所见进行观察,并复习了国内已报道的CJD的临床病理特点。结果:CJD患者临床表现以进行性痴呆和肌阵挛为特征,脑电图呈典型的发作性周期性高波幅双相和三相波,组织学所见为海绵状变性,星形细胞增生、肥胖变性及神经细胞脱失。结论:临床和脑电图检查结合,可帮助对该病的拟诊,对临床疑诊的病例,应做脑活检电镜观察以明确诊断。     

新变异型Creutzfeldt-Jakob病的诊断

新变异型Ｃｒｅｕｔｚｆｅｌｄｔ－Ｊａｋｏｂ病,是人类一种新的可传染性海绵状脑病,平均病程１４个月,平均死亡年龄２９岁。本文阐述新变异型Ｃｒｅｕｔｚｆｅｌｄｔ－Ｊａｋｏｂ病的临床特征、神经病理学特征、辅助检查、诊断标准及鉴别诊断。     

Creutzfeldt Jakob病1例报告

患者，男，67岁，以“糊涂40d”为主诉，于2007年6月21入院。患者40d年前无明显诱因出现头晕、颈部不适，在当地医院按脑供血不足治疗，未见好转，随之出现恐惧感，感觉自己在荒郊野外，上厕所回来找不到床，尚能与家人交流，言语肢体活动及饮食均正常，对自己的症状有自知力，     

Concomitant Creutzfeldt-Jakob and Alzheimer diseases

Summary We report a 69-year-old female with cerebral and cerebellar symptomatology of 15-month duration. At autopsy, both panencephalopathic Creutzfeldt-Jakob and plaque-predominant Alzheimer diseases were found. Plaque amyloid was exclusively of the /A4 type, but abundant abnormal protease-resistant protein was identified by Western blot analysis of brain extracts.     

Coexistence of Alzheimer-type neuropathology in Creutzfeldt-Jakob disease

Creutzfeldt-Jakob disease (CJD) and Alzheimer’s disease (AD) share clinical, neuropathological, and pathogenetic features. To investigate eventual mutual influences, we screened prominently affected neocortex from 110 neuropathologically proven CJD patients for Alzheimer-type pathology with anti-β/A4, Bielschowsky and anti-tau (immuno)stains. The neuropathological classification of Alzheimer-type pathology was made according to the CERAD criteria. Results were controlled by comparison with Alzheimer-type changes in sections from the same cortical areas in 110 sex- and age-matched non-demented control patients. For comparison, the control patients were also classified according to the CERAD neuropathology criteria as if they had been demented. Alzheimer-type tissue changes as in definite and probable CERAD AD occur in 10.9% of the CJD patients and 19.1% of control patients (P = 0.11). The median age of CJD and control patients with CERAD AD is 72 and 68 years, respectively, which differs significantly from the median ages of 64 and 63 years, respectively, in the non-AD/CJD and non-AD control patients. Since CERAD criteria include “presence of other neuropathological lesions likely to cause dementia”, an AD diagnosis in CJD patients (all of whom are demented) is solely based on densities of neuritic plaques. Similar Alzheimer-type changes in even higher frequency, however, are also present in elderly non-demented controls. Thus, the coexistence of Alzheimer-type pathology in CJD most likely represents an age-related change. Deposits of prion protein (PrP) frequently accumulate at the periphery of β/A4 plaques. The presence of β/A4 amyloid in the brain may influence PrP morphogenesis. Received: 18 November 1997 / Revised, accepted: 11 February 1998     

Ocular Dipping in Creutzfeldt-Jakob Disease

Background

Ocular dipping (OD), or inverse ocular bobbing, consists of slow, spontaneous downward eye movements with rapid return to the primary position. It has been mainly reported following hypoxic-ischemic encephalopathy, but has also been described in association with other types of diffuse or multifocal encephalopathies and structural brainstem damage.

Case Report

We report the case of a previously asymptomatic 66-year-old woman who presented with confusion, recent memory disturbances, and abnormal involuntary movements, followed by a coma. Abnormal spontaneous vertical eye movements consistent with OD developed from the fourth day after admission, and the patient died 20 days later. The pathological examination of the brain confirmed the diagnosis of Creutzfeldt-Jakob disease.

Conclusions

The precise location of damage causing OD is unknown. In contrast to ocular bobbing, OD has no localizing value itself, but structural brainstem damage is likely when it appears combined with other spontaneous vertical eye movements.     

国人Creutzfeldt-Jakob病的可能危险因素

目的 研究国人Creutzfeldt—Jakob病(CJD)的可能危险因素。方法 对30例已确诊的CJD进行地域、职业、颅脑外伤与手术、角膜手术、脑深部电极检查、输血及血液制品、生长激素注射、饮食习惯、遗传以及家族息神经系统其他变性疾病等10项回顾性研究。结果 (1)国内未发现硬膜、角膜移植、生长激素注射、脑深部电极检查所致的CJD；(2)未发现此病与职业相关以及接触牛、羊肉较多的人易患CJD；(3)未发现CJD有高发区；(4)白内障手术、颅脑外伤及开颅手术、遗传及家族中有患神经系统其他变性疾病者，可能为国人CJD的危险因素。结论 国人CJD的可能危险因素，既不同于西方，也有别于日本，充分认识这些事实，对减少医源性传播有积极意义。     

吸烟与阿尔茨海默病

目的 探讨吸烟与阿尔茨海默病(AD)的关系。方法 以社区为基础进行1：1配对病例对照研究。结果 共282对患者和对照者纳入研究，与≥50岁以后不吸卷烟者相比，50岁以后吸烟量为0．1～9．9包年者的OR值为0．748(95％CI：0．536～1．045)，吸烟量≥10包年者的OR值为0．560(95％CI：0．287～1．091，P=0．0884)。被动吸烟与AD无显著性联系(P=0．9568)。结论 ≥50岁时吸卷烟可能对AD有保护作用，且两者间的联系有边缘统计学意义，因此值得扩大样本量或选取暴露率更高的目标人群进一步研究。     

Serial Polysomnograms in Creutzfeldt-Jakob Disease

Abstract: This is a report of the case of a patient with Creutzfeldt-Jakob disease, whose electroencephalograms and polysomnograms were repeatedly recorded throughout the course of the illness with details of the alterations of periodic synchronous discharges. In the advanced stage of the disease, the appearance of peculiar paroxysms was noted, predominantly in the early morning. Furthermore, apnea of the central type was observed during the same time period. Discussions were held on the mechanisms inducing the EEG paroxysms and apneas.     

Creutzfeldt-Jakob病5例临床研究

目的　通过临床病例和实验室资料的总结 ,提高对CJD的认识和诊断水平。方法　对 5例CJD病患者的临床表现、影像学特点、脑电图改变、病理学变化、动物接种和基因检测等资料进行分析和总结。结果5例均为亚急性起病 ,有迅速进展的痴呆和肌阵挛 ,1例MRI显示双侧尾状核长T2 信号 ,脑电图均明显异常。 3例病理检查均符合典型CJD的病理学特点。结论　应当提高临床医师对本病的认识 ,根据典型的临床和EEG表现 ,特别是MRI特点以及CSF中 14- 3- 3蛋白的检测 ,争取实施无创性检查 ,以早期作出诊断     

克-亚二氏病的周围神经损害

目的 探讨克-亚二氏病(CJD)肌电罔与临床的关系。方法 分析5例CJD患者，2例为病理诊断，3例为临床诊断，对5例患者进行肌电图检查，其中1例病人在病程的不同阶段进行肌电图观察，并将肌电图改变与临床症状和体征进行分析总结。结果 5例病人中，1例出现束颤电位，1例出现神经性受损，其余3例为阴性。结论 在散发的CJD中，周围神经系统的损害应看作是疾病的一部分。