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  

Study on clinical features in general paresis of insane,HIV-associated dementia and Creutzfeldt-Jakob disease behaved as dementia

作　　者：刘文彦 吴逸雯 王信义 丁健青[1] 陈生弟[1] 

机构地区：[1]上海交通大学医学院附属瑞金医院神经内科、上海交通大学医学院神经病学研究所,200025

出　　处：《中国现代神经疾病杂志》2012年第3期342-348,共7页Chinese Journal of Contemporary Neurology and Neurosurgery

基　　金：国家重点基础研究发展计划(973计划)(项目编号:2011CB504100);上海市重点学科(项目编号:S30202);上海市基础研究重大(重点)项目(项目编号:10411954500);上海市医学领军人才计划项目(项目编号:LJ06003)~~

摘　　要：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。Objective To investigate the clinical features of general paresis of insane （GPI）, HIV- associated dementia （HAD） and Creutzfeldt- Jakob disease （CJD）. Methods The clinical features, laboratory examination, electroencephalography （EEG）, magnetic resonance imaging （MRI）, treatment and prognosis of 19 patients （GPI, n = 8; HAD, n = 6; CJD, n = 5） were analyzed retrospectively. Results The cases of three groups had cognitive impairment. At the same time, multiple systems （pyramidal system, extrapyramidal system and cerebellar） and multiple cranial nerves were involved. In GPI patients, the results of rapid plasma regain circle card test （RPR） and treponema pallidum particle agglutination assay （TPPA） were all positive in 8 cases, venereal disease research laboratory tests （VDRL） of CSF were positive in 4 cases, and Head MRI showed encephalatrophy in 6 cases. In HAD patients, serologic tests for HIV of all cases were positive, the average protein of CSF was increased significantly and Pandy＇s test was positive in 2 cases, Head MRI were characterized with multiple space occupying lesions or diffuse abnormal density image. In CJD patients, CSF 14-3-3 protein showed positive in 4 patients, EEG showed diffuse slow waves in 5 case, in which 1 case showed typical periodic triphasic wave, Head MRI in 3 sporadic CJD patients （sCJD） showed swelling like changes in sulus of cortex area and 1 varaint CJD （vCJD） patient showed hockey like change in thalamencephalon. Conclusion The clinical characteristics of dementia caused by GPI, HAD or CJD are varied, and the diagnosis mainly depends on clinical features, positive findings in serum, CSF, EEG and MRI detections.

关 键 词：神经梅毒 艾滋病痴呆复合征 朊病毒病 血清学试验 脑脊髓液 脑电描记术 磁共振成像 

分 类 号：R749.16[医药卫生—神经病学与精神病学]