应明确β淀粉样蛋白在脑淀粉样血管病与阿尔茨海默病发病机制中的作用

脑淀粉样血管病和阿尔茨海默病常常共存,二者在临床表现上存在着很多相似之处,β淀粉样蛋白在这两种疾病的发病机制中都起到了关键的作用。明确Aβ的作用有助于认清疾病的本质,从而更好地把握这两种疾病不同的临床特征,更好地作出诊断和治疗。     

脑淀粉样血管病相关疾病的临床特征研究进展

脑淀粉样血管病(CAA)是β-淀粉样蛋白(Aβ)沉积在软脑膜和大脑皮质血管壁中引起的一种脑小血管疾病.随着人口的老龄化,其在老年患者中很常见,发病率较高,临床上常与多种类型的脑损伤有关,但发病形式隐匿、临床表现多样且不具有特异性,因此识别这种疾病以避免高风险药物的使用至关重要.文章就CAA的定义、流行病学及CAA相关疾...     

脑淀粉样血管病的研究进展

脑淀粉样血管病(CAA)与脑血管淀粉样蛋白沉积有关。脑血管Aβ沉积伴随脑血管的功能和病理变化。CAA相关的血管改变导致出血性病变[脑叶出血,皮质微出血和皮层浅表铁血黄素沉积(cSS)/局灶性凸面蛛网膜下腔出血(SAH)]、缺血性病变(皮质梗死和白质缺血性改变)及CAA相关的炎症/血管炎引起的亚急性脑白质病。随着神经影像学发展,如梯度回波T2~*成像和磁敏感加权成像,可用于检测皮质微出血和cSS。另外,脑脊液的标记物对诊治也起到了一定作用,包括CAA的Aβ40水平和CAA相关炎症抗Aβ抗体的水平。本文综述了CAA和CAA相关疾病的流行病学、病理生理学、临床表现、生物标志物、诊断和治疗。     

脑淀粉样血管病的免疫组化研究

目的：研究脑淀粉样血管病的脑膜及皮层动脉细胞外间质淀粉样淀物成份，探讨该病理改变与脑出血的关系。方法：研究样本取自于１０例生前患有脑淀粉样性血管病死亡者（９例阿尔茨海默病，１例先天性愚型）。另设对照组８例，均为非脑病病故者。利用免疫组化ＡＢＣ法，将针对细胞外间质各种胶元成份以及平滑肌和内皮细胞的单克隆，多克隆抗体与组织切片进行组化染色，显微镜下观察。结果：血管内皮细胞层葡萄糖转运酶减少，中层ＥＣＭ     

脑淀粉样血管病并脑出血的CT诊断与临床分析

目的:探讨脑淀粉样血管病所致脑出血的CT表现特点及临床价值。方法:总结15例脑淀粉样血管病(CAA)引起的脑出血患者的CT表现,结合国内外文献资料及最新病理学研究进展进行综合分析。结果:CAA性脑出血有以下特征:多病灶,多形态,多脑叶,多次反复发作(新旧病灶交替),多位于脑皮质或皮髓质交界区,以颞顶枕额叶多见,血肿可破入脑室、蛛网膜下腔等。结论:CAA性脑出血有一定的CT表现特点及临床特点,总结其特点,旨在提高对本病的认识与影像诊断。     

血管淀粉样变相关脑出血的初步研究

脑淀粉样血管病(CAA)是脑出血的主要病因之一,因脑组织的取材困难、病理诊断要求的组织特异性和诊断的阳性率低等因素限制,使临床确诊率低。我们分析62例脑出血患者脑组织的刚果红染色、免疫组化检测淀粉样前体(Aβ)蛋白和HE染色结果,并结合临床特点,旨在探讨血管淀粉样变相关的脑出血的病因临床特点、和引发再出血的因素。     

脑淀粉样血管病并脑出血的CT诊断与临床分析

目的：探讨脑淀粉样血管病所致脑出血的CT表现特点及临床价值。方法：总结15例脑淀粉样血管病（CAA）引起的脑出血患者的CT表现，结合国内外文献资料及最新病理学研究进展进行综合分析。结果：CAA性脑出血有以下特征：多病灶，多形态，多脑叶，多次反复发作（新旧病灶交替），多位于脑皮质或皮髓质交界区。以颞顶枕额叶多见，血肿可破入脑室、蛛网膜下腔等。结论：CAA性脑出血有一定的CT表现特点及临床特点，总结其特点。旨在提高对本病的认识与影像诊断。     

脑淀粉样血管病相关炎症6例临床诊治的回顾性分析

脑淀粉样血管病相关炎症（cerebral amyloid angiopathy-related inflammation, CAA-ri）是β淀粉样蛋白（Aβ）沉积于脑血管壁引起的炎症反应，是CAA中较为罕见的临床亚型。以头痛、癫痫发作、认知和行为改变等为主要临床表现，但无特异性。头部磁共振（MRI）提示单发或多发白质高信号病灶，磁敏感加权序列（SWI）上存在超过１处皮质或皮质下出血性病灶，包括脑出血、脑微出血、皮质表面铁沉积等。本研究回顾性分析了2017年1月至 2019年9月于我院按照2016年新修订的诊断标准诊治的6例很有可能的CAA-ri病例，发现5例患者在常规T2及FLAIR序列也能检测出微出血和皮质表面铁沉积病灶，提示如果患者病程较长、年龄较大、微出血负荷重者常规核磁检查即能发现病灶，这一特点是诊断CAA-ri的线索，临床医生遇到这一现象应该高度重视，可进一步行SWI检查验证。     

脑淀粉样血管病相关性脑出血的临床研究现状

脑淀粉样血管病相关性脑出血(CAAH)是导致自发性脑出血的第二常见病因,仅次于高血压脑出血,占自发性脑出血的15%～40%。目前研究认为β淀粉样蛋白(Aβ)沉积于软脑膜和皮质的动脉、微动脉以及毛细血管壁内,损害血管壁的完整性,并增加受累血管的脆性是导致脑出血的重要机制。尽管CAAH术中止血困难,但随着治疗方法的深入研究,明确可外科治疗的安全性及有效性。本文对近期有关CAAH的机制、病理特点、影像学表现、治疗方法的相关文献进行梳理,对目前CAAH的研究现状进行综述,以改变人们对CAAH的传统认识。     

头颅CT检出脑淀粉样血管病相关性脑出血与高血压脑出血的危险因素对比分析

目的：通过观察我院脑出血患者临床资料,探讨分析头颅CT检出脑淀粉样血管病相关性脑出血与高血压脑出血的危险因素。方法：对我院收治的49例脑淀粉样血管病相关性脑出血患者（CAAH组）与43例高血压脑出血患者（HICH组）,进行头颅CT检查,对比分析两组的临床资料与检查结果。结果：两组患者在年龄、高血压患病史、脑卒中家族史、发病前痴呆以及癫痫表现率等临床资料对比差异显著（P〈0.05）;经头颅CT检查后,两组患者在出血部位、出血量（〈30mL与〉60mL）、多发性出血以及再次出血等方面对比差异显著（P〈0.05）。结论：年龄、高血压患病史、脑卒中家族史、发病前痴呆以及癫痫表现率等指标可能是引起HICH患者与CAAH患者出现脑出血的危险因素,采用头颅CT检查,能有效定位不同患者的具体出血部位,提供出血量等相关信息,有利于疾病的临床诊断与治疗,值得临床上进一步研究与推广。     

Cerebral amyloid angiopathy with dementia: clinicopathological studies of 17 cases

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血管性痴呆动物模型研究概况

血管性痴呆是由于血管源性的脑部病灶所导致的严重认知功能障碍,它不是一种单一的疾病,而是由于多种发病原因引起的多类型综合征,皮质下小动脉病变、多发梗塞、脑慢性低灌注以及脑血管淀粉样改变等都是引起血管性痴呆的不同病因。针对不同的病因制备血管性痴呆动物模型是研究血管性痴呆发病机制、治疗药物等的重要条件．本文就近几年来血管性痴呆动物模型的制备做以综述。     

Cerebrospinal Fluid Biomarkers in Dementia Patients with Cerebral Amyloid Angiopathy

Objective To study the changes of biomarkers in cerebrospinal fluid (CSF) in cerebral amyloid angiopathy (CAA) dementia and Alzheimer's disease. Methods Levels of amyloid proteinβ (Aβ42, Aβ40) and phosphorylated Tau-protein (P-tau) in CSF and ratio of Aβ42/Aβ40 were tested in 5 cases with CAA dementia and 20 cases with Alzheimer's disease collected at Peking Union Medical College Hospital from December 2001 to March 2011. Results The levels of Aβ42, Aβ40, and P-tau in CSF and ratio of Aβ42/Aβ40 were (660.4±265.2) ng/L, (7111.0±1033.4) ng/L, (71.8±51.5) ng/L, and 0.077±0.033, respectively in CAA dementia and (663.6±365.6) ng/L, (5115.0±2931.1) ng/L, (47.7±38.8) ng/L, and 0.192±0.140, respectively in Alzheimer's disease patients. There were no statistically significant differences between CAA dementia and Alzheimer's disease in terms of these CSF biomarkers (allP>0.05). Conclusion Measurements of CSF biomarkers may not be helpful in differential diagnosis of CAA and Alzheimer's disease.     

Senile dementia of the Binswanger type. A vascular form of dementia in the elderly

Computed tomography and magnetic resonance imaging in the elderly have demonstrated the common occurrence of deep white-matter lesions in the aging brain. These radiologic lesions (leukoaraiosis) may represent an early marker of dementia. At autopsy, an ischemic periventricular leukoencephalopathy (Binswanger's disease) has been found in most cases. The clinical spectrum of Binswanger's disease appears to range from asymptomatic radiologic lesions to dementia with focal deficits, frontal signs, pseudobulbar palsy, gait difficulties, and urinary incontinence. The name senile dementia of the Binswanger type (SDBT) is proposed for this poorly recognized, vascular form of subcortical dementia. The SDBT probably results from cortical disconnection most likely caused by hypoperfusion. In contrast, multi-infarct dementia is correlated with multiple large and small strokes that cause a loss of over 50 to 100 mL of brain volume. The periventricular white matter is a watershed area irrigated by long, penetrating medullary arteries. Risk factors for SDBT are small-artery diseases, such as hypertension and amyloid angiopathy, impaired autoregulation of cerebral blood flow in the elderly, and periventricular hypoperfusion due to cardiac failure, arrhythmias, and hypotension. The SDBT may be a potentially preventable and treatable form of dementia.     

淀粉样脑血管病相关脑出血的临床研究

目的：研究淀粉样脑血管病(erebral amyloid angiopathy，CAA)相关脑出血(CAAH)的临床和病理特点。方法：通过分析5例高血压性脑出血患者的临床表现、影像学和脑组织局部解剖，总结CAAH的临床特点。结果：CAAH的发病情况和临床表现类似于高血压性脑出血。CAAH可并存高血压病，高血压病可加重CAA改变。典型CAA脑出血以皮质、皮质下脑叶出血多见，血肿形态多不规则，占位效应明显。但常见的高血压脑出血部位，如基底节、丘脑出血，并不能排除CAA脑出血的可能。结论：高血压病为脑出血的常见病因，CAA是脑出血的重要病因，并具有重要的临床意义。     

Cerebral amyloid angiopathy-related inflammation: current status and future implications

Cerebral amyloid angiopathy-related inflammation (CAA-RI) is a rare but increasingly recognized subtype of CAA. CAA-RI consists of two subtypes: inflammatory cerebral amyloid angiopathy and amyloid β (Aβ)-related angiitis. Acute or subacute onset of cognitive decline or behavioral changes is the most common symptom of CAA-RI. Rapid progressive dementia, headache, seizures, or focal neurological deficits, with patchy or confluent hyperintensity on T2 or fluid-attenuated inversion recovery sequences and evidence of strictly lobar microbleeds or cortical superficial siderosis on susceptibility-weighted imaging imply CAA-RI. The gold standard for diagnosis is autopsy or brain biopsy. However, biopsy is invasive; consequently, most clinically diagnosed cases have been based on clinical and radiological data. Other diagnostic indexes include the apolipoprotein E ε4 allele, Aβ and anti-Aβ antibodies in cerebral spinal fluid and amyloid positron emission tomography. Many diseases with similar clinical manifestations should be carefully ruled out. Immunosuppressive therapy is effective both during initial presentation and in relapses. The use of glucocorticoids and immunosuppressants improves prognosis. This article reviews the pathology and pathogenesis, clinical and imaging manifestations, diagnostic criteria, treatment, and prognosis of CAA-RI, and highlights unsolved problems in the existing research.     

Diagnosis and management of Alzheimer's disease--an update

Alzheimer's disease (AD) has become recognised as a major cause of morbidity and mortality in the ageing population worldwide. Over 20 million people worldwide are affected by AD, which ensures that the disease imposes a major economic burden. Alzheimer's disease is a progressive neurodegenerative disorder with characteristic clinical and neuropathological features. Neurofibrillary tangles, neuritic plaques and amyloid angiopathy occur in varying severity in brains of patient's with Alzheimer's disease. Biological markers of AD allowing an early definitive premorbid diagnoses are currently not available. Memory loss for recent events is invariable and often the earliest prominent symptom. Language disorders, difficulties with complex tasks, depression, psychotic symptoms and behavioral changes are other common manifestations of AD. Diagnosis involves the early detection of cognitive decline and ruling out other causes of dementia like vascular dementia, Lewy body dementia, fronto-temporal degeneration or reversible causes like hypothyroidism. Acetylcholinesterase inhibitors have shown to be effective in mild to moderate AD in improving the cognitive function of patients in clinical trials. Caregiver intervention programs have considerable potential to improve both the caregiver and patient quality of life.     

Pathological study on cerebral amyloid angiopathy.

The cerebral amyloid angiopathy (CAA) was pathologically confirmed in 26 cases in which there were 20 cerebral hemorrhages, 2 subarachnoid hemorrhages, 3 cerebral infarctions, and 1 subdural hematoma. The CAA mainly involved the media and extima of small and medium-sized arteries as well as the capillaries of the brain tissue. Half of all cases in our series had the CAA associated vasculopathies (CAA-AV), including microaneurysms, "double barreling", onion skin-like intima, fibrinoid necrosis, clusters of arterioles and perivascular lymphocytic infiltrations. The CAA was often accompanied with the senile plaques and neurofibrillary tangles.