麻痹性痴呆的临床特征与诊断(附2例报告)

目的探讨麻痹性痴呆的临床特征，以及实验室及影像学检查在诊断中的价值。方法回顾性分析2例麻痹性痴呆患者的临床资料，并结合文献进行讨论。结果主要临床表现为隐匿起病，进行性加重性痴呆、精神障碍、癫痫发作等；2例患者均被误诊。血清及脑脊液快速血浆反应素环状卡片试验（RPR）阳性，梅毒螺旋体明胶凝集试验（TPPA）阳性，头颅MRI主要表现为弥漫性脑萎缩。结论麻痹性痴呆的临床表现复杂多变，早期误诊率高；诊断主要根据临床特点、血清学及脑脊液检查综合考虑。     

麻痹性痴呆14例临床特征

目的探讨麻痹性痴呆(GPI)的临床类型、临床表现。方法分析14例麻痹性痴呆患者的临床资料。结果 14例麻痹性痴呆主要表现为精神症状和神经系统症状,门诊与入院诊断的误诊率高达100%,住院后均经实验室检查梅毒螺旋体凝集试验(TPPA)阳性而确诊。结论麻痹性痴呆易被误诊,诊断依据临床流行病学资料、临床表现、血清和脑脊液梅毒抗体检测阳性可确诊。作为一种可治性痴呆,早期的预防和观察识别有着非常重要的意义。     

神经梅毒延迟诊断一例临床分析

目的探讨麻痹性痴呆的临床特征、诊断方法及延迟诊断原因。方法结合1例麻痹性痴呆患者的临床资料进行讨论,阐述新时期神经梅毒的隐蔽性和造成延误诊断的原因。结果麻痹性痴呆中年人发病较多;起病隐匿,进行性加重,主要表现为认知障碍、精神症状、癫疒间发作等;梅毒螺旋体明胶凝集试验(TPPA)阳性;头颅MRI无特征性改变;通过驱梅治疗麻痹性痴呆患者MOCA评分提高。结论神经梅毒临床表现复杂,早期误诊率高;建议梅毒血清抗体检测应作为青壮年卒中或痴呆患者的常规检查项目;麻痹性痴呆是一种可治的痴呆,应及早诊断及早治疗。     

神经梅毒的临床和影像学分析

目的分析神经梅毒的临床、神经影像学和实验室检查特征。方法回顾性分析符合神经梅毒诊断标准的28例患者的临床、神经影像和实验室资料。结果无症状型20例,脑膜型1例,脑膜血管型2例,脊髓结核2例,麻痹性痴呆3例;临床表现复杂多变,易于误诊;脑脊液检查多有异常改变,血清和脑脊液的梅毒特异性抗体检查阳性;神经影像学改变无明显的特异性。结论神经梅毒不同病理阶段有不同的临床表现,应综合分析患者的临床资料,早期诊断和治疗。     

麻痹性痴呆12例误诊原因分析

目的：分析麻痹性痴呆（GPI）误诊原因。方法：回顾性分析12例麻痹性痴呆患者的临床资料，智能检测和各项实验室检查结果。结果：12例患者曾误诊为精神分裂症4例，躁狂发作3例，老年性痴呆3例，多发性硬化和病毒性脑炎各1例。所有患者梅毒螺旋体血液检查及脑脊液检查均为阳性，简易智力状态量表评分均≤20分。经大剂量青霉素治疗后，一般精神症状控制，智力部分恢复，神经体征残留。结论：麻痹性痴呆极易被误诊，忽略了患者的认知障碍和未做细致的神经系统检查是误诊的重要原因。早期诊断和治疗是预后的关键。     

麻痹性痴呆二例的临床特征与诊断

目的分析麻痹性痴呆(GPI)的临床特征及提供早期诊断依据。方法回顾性分析经临床和实验室检查确诊的2例麻痹性痴呆患者的有关临床资料。结果GPI的临床特征(1) 2例患者慢性起病,进行性发展; (2)痴呆是核心症状, 2例患者入院时长谷川痴呆量表评分为4分和17分,均伴夸大妄想、病理性欣快等精神症状; (3) 1例患者的瞳孔左右不等大,光反射迟钝或消失但辐辏反射保持, 2例患者均有构音障碍、肌张力增高及反射异常等; (4) 2例患者血清、脑脊液梅毒抗体反应均为阳性,脑脊液蛋白含量增加、细胞数增多(以淋巴细胞为主); (5)头颅MRI示2例患者均有脑萎缩, 1例患者有脑实质异常信号影,为多发、散在病灶,广泛脑白质纤维变性。结论GPI误诊率高,临床表现、实验室及影像学检查是诊断的重要依据。     

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。     

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。     

曾被误诊的麻痹性痴呆23例分析

目的:研究麻痹性痴呆的诊断与治疗。方法:对曾被误诊的23例麻痹性痴呆,使用简明精神病评定量表(BPRS)及简易精神状态检查(MMSE)评定。并予驱梅毒及抗精神病药治疗。结果:驱梅毒治疗后可显著提高疗效。结论:麻痹性痴呆应及早正确诊断,作驱梅毒及抗精神病治疗。     

脑膜血管型梅毒的临床及影像特点

目的分析脑膜血管型梅毒的临床及影像特征以提供早期诊断依据。方法分析经临床及实验室确诊的2例脑膜血管型梅毒患者的有关临床资料。结果脑膜血管型梅毒早期以突然出现偏瘫、癫痫发作、失语、认知障碍为主要临床表现,影像上主要为病灶散在多发,头颅MRA或CTA可见颅内血管狭窄,实验室检查有特征性改变。结论脑膜血管型梅毒早期临床容易误诊,应重视梅毒血清和脑脊液抗体的检查,结合头颅MRI及MRA或CTA可以诊断,大剂量足疗程青霉素治疗能取得显著疗效。     

麻痹性痴呆的MRI表现(附32例报道)

目的 探讨麻痹性痴呆(GPI)的MRI特征和诊断价值,提高对GPI患者MRI表现的认识.方法 中山大学第三附属医院放射科自2006年5月至2010年11月共诊断GPI患者32例,回顾性分析患者的临床资料并总结GPI的MRI表现.结果 本组患者的MRI表现分为两型:脑萎缩型(30例)和脑肿胀型(2例),其中脑萎缩型GPI的MRI表现为:(1)以颞叶、额叶、海马、胼胝体萎缩为主,脑白质萎缩较脑皮质萎缩明显;(2)杏仁体及海马形态异常及伴T2信号增高;(3)皮层/皮层下"脑回状"T2信号增高,以岛叶多见;(4)双侧豆状核对称性T2信号减低;脑肿胀型GPI的MRI表现为弥漫型或局灶型.结论 GPI的MRI表现多样,但具有一定特征性,结合临床表现和相关实验室检查结果可作出正确诊断.

Abstract：

Objective To evaluate the MRI findings in patients with general paresis of insane (GPI) to enhance the understanding of MRI diagnosis of this disease. Methods The clinical data and MRI findings of 32 patients with GPI, admitted to our hospital from May 2006 to November 2010, were retrospectively analyzed. Results The MRI findings of GPI were mainly divided into 2 types: cerebral atrophy (n=30) and cerebral swell (n=2). The major MRI findings in the type of cerebral atrophy included white cerebral atrophy in the temporal lobe, the frontal lobe, the hippocampus and the corpus callosum, morphological changes and T2 hyperintensity in the amygdaloid body and the hippocampus, gyral T2 hyperintensity in the cortex and subcortex, and T2 hypointensity in the lenticular nucleus. The MRI findings in the type of cerebral swell manifested as suffused and focal types. Conclusion The MRI findings in GPI are multiple with some characteristic manifestations. Diagnosis must be made through the combination of imaging features with clinical data and related laboratory tests.     

麻痹性痴呆(附2例报告)

目的 探讨麻痹性痴呆（GPI）的临床表现、实验室检查、影像学改变和治疗。方法 回顾性分析2例GPI患者的临床资料结果2例患者均为男性,以记忆力下降和精神障碍为主要临床表现,1例出现内脏危象,血清及脑脊液快速血浆反应素环状卡片试验（RPR）、梅毒螺旋体明胶凝集试验（TPPA）均（＋）,MRI检查均有脑室扩大经驱梅治疗后,2例患者病情均明显好转。结论 GPI多见于中年男性,误诊率高,对不明原因的年轻痴呆患者,应想到GPI的可能,及时诊断、积极治疗对其预后有重要意义。     

Evaluation of a new set of automated chemiluminescense assays for anticardiolipin and anti-beta2-glycoprotein I antibodies in the laboratory diagnosis of the antiphospholipid syndrome

Introduction

The laboratory diagnosis of antiphospholipid syndrome (APS) requires the demonstration of antiphospholipid antibodies (aPL): lupus anticoagulant (LAC) measured through coagulation assays, anticardiolipin IgG or IgM antibodies (aCL) and/or anti-β2glycoprotein I IgG or IgM antibodies (aβ2GPI), usually detected by ELISA

Materials and methods

We evaluated the diagnostic value of aCL and aβ2GPI measured by a new automated system using the chemiluminescence principle, the immunoanalyzer Zenit RA (Menarini).

Results

Results of aCL and aβ2GPI were correlated with the clinical background of the patients and with results of ELISA (n = 314). Correlated to the clinical background sensitivity/specificity ranged for aCL IgG between 7.5-45.2% / 54.2-98.8%, for aCL IgM 3.4-5.5% / 89.9-94%, for aβ2GPI IgG 5.5-25.3% / 75.6-100% and aβ2GPI IgM 3.4-4.8% / 89.9-92.3%, depending on the cut-off used. Sensitivity with manufacturer's cut-offs was comparable to ELISA, except for aβ2GPI IgG with a significantly lower sensitivity compared to ELISA (5.5% vs 11.6%).In the APS patient population (n = 30) sensitivity of aCL IgG and aβ2GPI IgG was higher measured by ELISA compared to Zenit RA (46.7% vs 30.0%, and 46.7% vs 26.7%, respectively).Agreement between Zenit RA results and ELISA results for the four parameters was moderate (Kappa-values ranging 0.509-0.565). Sensitivity was 38.5%, 53.3%, 40% and 69.2% for aCL IgG, aCL IgM, aβ2GPI IgG and aβ2GPI IgM, respectively, applying the highest cut-off value for Zenit RA, raising towards 64.3%, 100%, 57.1%, for aCL IgG, aCL IgM, aβ2GPI IgG, respectively, in a APS patient population.

Conclusions

The new technology of chemiluminescense for measuring aPL showed good performance characteristics. Interpretation of results with a cut-off value associated with a good discrimination for disease, resulted in a lower sensitivity for the diagnosis of APS for aβ2GPI IgG measured by Zenit RA assays compared to ELISA; sensitivity for aCL IgG was comparable to ELISA. Specificity for all parameters was high and comparable for aCL and aβ2GPI.     

Contemporary use of glycoprotein IIb/IIIa inhibitors

Platelet glycoprotein IIb/IIIa inhibitors (GPI) are antithrombotic agents preventing the binding of fibrinogen to GP IIb/IIIa receptors. Thus, GPI interfere with interplatelet bridging mediated by fibrinogen. Currently, three generic GPI with different antithrombotic properties are available for intravenous administration: abciximab, eptifibatide, and tirofiban. The development of oral GPI was abandoned, whereas intravenous GPI were introduced in various clinical settings during the 1990s, yielding substantial benefit in the treatment of acute coronary syndromes, particularly during percutaneous coronary interventions. Results of the many randomised trials evidenced the efficacy of this drug class, though these trials were conducted prior to the emergence of modern oral antiplatelet therapy with efficient P2Y(12) inhibitors. Subsequent trials failed to consolidate the strongly favourable impression of GPI, and indications for their use have been more restricted in recent years. Nonetheless, GPI may still be beneficial during coronary interventions among high-risk patients including acute ST-elevation and non-ST-elevation myocardial infarctions, particularly in the absence of adequate pretreatment with oral antiplatelet drugs or when direct thrombin inhibitors are not utilised. Intracoronary GPI administration has been suggested as adjunctive therapy during primary percutaneous coronary intervention, and the results of larger ongoing trials are expected to elucidate its clinical potential. The present review outlines the key milestones of GPI development and provides an up-to-date overview of the clinical applicability of these drugs in the era of refined coronary stenting, potent antithrombotic drugs, and novel thrombin inhibiting agents.     

Prevalence and significance of anti-prothrombin (aPT) antibodies in patients with Lupus Anticoagulant (LA)

Objective

Anti-prothrombin (aPT) antibodies have been found in Lupus Anticoagulant (LA) positive patients. Their prevalence and relative contribution to thromboembolic risk in LA-positive patients is not well defined. The aim of this study was to determine their presence and association with thromboembolic events in a large series of patients with confirmed LA.

Methods

Plasma from LA-positive patients was collected at Thrombosis Centers and sent to a reference central laboratory for confirmation. Positive plasma was tested using home-made ELISA for the presence of aPT and anti-β₂GPI antibodies.

Results

LA was confirmed in 231 patients. Sixty-one of 231 (26%, 95%CI 22-33) LA positive subjects were positive for IgG aPT and 62 (27%, 95% CI 21-33) were positive for IgM aPT antibodies. Clinical features of Antiphospholipid Syndrome (APS) were not associated with the presence of IgG aPT [43 APS in 61 (70%) positive and 109 APS in 170 (64%) negative IgG aPT subjects, p = ns] or IgM aPT. Rate of positivity of IgG and IgM aβ₂GPI was significantly higher than that of IgG and IgM aPT. Clinical events accounting for APS occurred in 97 of 130 (75%) IgG aβ₂GPI positive and in 55 of 101 (54%) IgG aβ₂GPI negative patients (OR 2.4, 95% CI 1.4 to 4.3, p = 0.002). No significant association with clinical events in patients positive for both IgG aPT and IgG aβ₂GPI as compared to those positive for one or another test was found. When patients negative for both IgG aPT and IgG aβ₂GPI (LA positive only) were compared with remaining patients, a significantly lower association with clinical events was found (OR = 0.4, 95% CI: 0.2 to 0.7, p = 0.004).

Conclusions

As compared to IgG aβ₂GPI, the prevalence of IgG aPT in patients with LA is significantly lower and not associated with the clinical features of APS.     

不同时期麻痹性痴呆临床对照研究

目的：分析1947年至1966年和2004年至2007年丽个不同历史时期麻痹性痴呆（GPI）患者的临床差异。方法：回顾性调查1947年6月至2007年6月GPI住院患者临床情况。病例分旧患者组（1947年至1966年时期247例中随机抽取50例），和新患者组（2004年至2007年期间，计12例）两组，进行对照分析。结果：旧患者组收治12．4例／年，新患者组3．42例／年；入院前和入院3d内作血液和脑脊液梅毒抗体检测旧患者组96％，新患者组为0％；临床误诊率旧患者组4％，新患者组100％；两组差异有显著性（P〈0．01）。结论：目前GPI临床少见，患者性病冶游史隐蔽，临床血清梅毒的筛查未列为常规性检查，容易导致误诊。     

Libman-Sacks endocarditis associated with antiphospholipid syndrome and infection

INTRODUCTION: Antiphospholipid syndrome (APS) is a systemic autoimmune disease, associated not only with a hypercoagulable state and recurrent fetal loss but with many diverse clinical manifestations including heart involvement, neurological manifestations, as well as skin, kidney and hematologic abnormalities. Cardiac manifestations include coronary by-pass graft and angioplasty occlusions, cardiomyopathy, cyanotic congenital heart disease, intracardiac thrombus and complications of cardiovascular surgery. The valvular heart disease was defined as Libman-Sacks nonbacterial endocarditis. Previously, we have shown a linear subendothelial deposition of anti-cardiolipin/beta2 glycoprotein I (beta2GPI) antibodies in the valve specimens derived from APS patients. The involvement of complement C3c in the pathogenesis was documented. We assessed the beta2GPI-related target epitope recognized by the anti-beta2GPI Abs on the valves. MATERIALS AND METHODS: In order to find the beta2GPI-related target epitopes recognized by the anti-beta2GPI antibodies on the valves, we used beta2GPI-related synthetic peptides. The presence of anti-beta2GPI Abs on the studied valves was detected by anti-idiotypic antibody, followed by immunoperoxidase analysis. Biotin attached to the N-terminal of beta2GPI-related synthetic peptides and control peptide were used to identify the epitope addressed by the anti-beta2GPI Abs deposited on the patient's valve. The binding was probed by streptavidin-peroxidase and appropriate substrate. The specificity was confirmed by competition assays with control peptide and anti-idiotypic antibody. RESULTS: Among the beta2GPI-related synthetic peptides, two peptides were found in previous studies to mimic common pathogens either bacteriae or viruses, which raised a possible infectious origin for APS. One of these peptides, TLRVYK, is a specific target for anti-beta2GPI Abs deposited on the APS valves. This synthetic peptide was able to displace the anti-anti-beta2GPI anti-idiotypic Abs for binding the anti-beta2GPI Abs on the valve by a competition assay. CONCLUSION: We point to the possibility that Libman-Sacks nonbacterial endocarditis may have an infectious origin.