易误诊的脑淀粉样血管病分析并文献复习

目的 根据脑淀粉样血管病的临床表现及磁共振影像资料探讨脑淀粉样血管病的临床特征及诊断方法。方法 回顾性分析我院就诊的3例脑淀粉样血管病患者的临床表现、MRI及磁敏感加权成像(SWI)特点。结果 3例脑淀粉样血管病可以以复发性和(或)多发性脑叶出血、痴呆、认知和神经功能减退、暂时性局灶性神经症状发作为主要表现形式,早期难以诊断及鉴别诊断,行SWI检查显示皮质及皮质下多发点状微出血灶,给予对症治疗后症状改善显著。结论 脑淀粉样血管病的早期诊断需要结合临床表现及影像学检查,磁敏感成像(SWI)示皮质及皮质下多发点状微出血灶为主要诊断依据,早期诊断及治疗,减少误诊和漏诊。     

淀粉样脑血管病相关性脑出血的病理研究

目的 研究新疆少数民族地区自发性脑出血患者中淀粉样脑血管病相关性脑出血(CAAH)的比例及其病理特点.方法 经头颅CT证实为自发性脑出血的124例患者来自于有代表性的南北疆6家三级甲等医院,入组患者接受开颅手术,标本取白血肿腔周围,通过HE染色、刚果红染色偏振光显微镜观察、β淀粉样蛋白(Aβ)免疫组化检测明确是否存在脑血管淀粉样变性.结果124例患者中11例为CAAH,占8.9％,其中4例为嗜刚果红血管病,1例表现为斑样血管病,6例为混合型.结论 新疆少数民族地区手术治疗的自发性脑出血患者中8.9％与淀粉样脑血管病(CAA)相关,其比例随年龄增加;CAA表现为受累的血管壁增厚,血管壁正常结构消失,淀粉样物质在血管被膜中层和外膜中沉积;部分患者脑实质内可见β淀粉样蛋白沉积.     

淀粉样变脑出血的临床特点及诊断方法

目的 分析淀粉样变脑出血的临床特点及诊断方法.方法 对55例自发性脑出血患者的临床表现及预后进行总结,对病理标本进行刚果红特殊染色和β淀粉样蛋白免疫组化染色,以明确病理诊断.结果 55例中6例首诊为可疑性淀粉样变脑出血,1例通过病理染色予以排除;2例术前诊断为可疑高血压性脑出血的患者经病理染色确诊为淀粉样变脑出血.结论 淀粉样变脑出血术前可以根据临床特点做出初步诊断;但是对于临床表现不典型者,诊断上还有一定的困难,病理分析仍然是诊断的金标准.目前对于此病的临床诊断方法需进一步研究改进.     

短期内反复多灶性脑出血为表现的脑转移癌1例

自发性多灶性脑出血约占所有脑实质出血3%~([1]).其病因包括高血压病、血管炎、脑淀粉样血管病、凝血功能障碍、原发和继发的脑肿瘤、脑静脉窦血栓形成等~([2]).由于自发性多灶性脑出血相对少见,以上各种病因发病率未有详细报道.短期内反复多灶性脑出血更为罕见.本文报告1例短期内反复多灶性脑出血为表现的脑转移癌,分析其临床特征和影像学特点,希望有助提高临床医师对这一疾病的认识.     

手术联合重组活化凝血因子Ⅶ治疗脑淀粉样血管病相关性脑出血1例并文献复习

目的 观察开颅血肿清除术联合凝血因子Ⅶ治疗脑淀粉样血管病相关性脑出血的疗效,探讨脑淀粉样血管病相关性脑出血的治疗方案.方法 分析1例脑淀粉样血管病相关性脑出血患者的临床表现、影像学、治疗经过及预后等资料,并复习相关文献.结果 患者男性,78岁,以头晕起病,病情快速进展至左侧肢体偏瘫及嗜睡,头部CT显示右侧额叶脑出血,予开颅血肿清除术清除血肿.术后血肿脑组织病理显示脑血管壁可见淀粉样蛋白沉积,显示为脑淀粉样血管病,予重组活化凝血因子Ⅶ2 mg静脉注射1次.术后90 d患者无明显功能障碍,能完成日常工作及生活,改良Rankin量表评分1分.结论 手术联合凝血因子Ⅶ治疗可能对脑淀粉样血管病相关性脑出血患者有效.     

脑淀粉样血管病相关出血(附6例分析)

探讨脑淀粉样血管病（CAA）相关出血的临床特征,以期引起对该病的认识与重视。对6例CAA相关出血的临床资料进行分析,并结合文献讨论本病病因、诊断及治疗。6例病人均为急性起病,既往均有高血压病史;头颅CT显示以脑叶为主的出血量较大的高密度灶;病理检查显示病史以软脑膜为主,有CAA－AV改变4例,经刚果红染色均为典型的苹果绿双折光现象。本病病灶多发生于脑叶,常呈多发性和反复性;CSS-AV改变为本病原因之一,病理标本应尽可能包括软脑膜,病灶处及切片其他区域均有血管淀粉样蛋白染色阳性即可确诊;治疗方案的选择应考虑到病人年龄、意识障碍程度及病变范围等因素。     

脑淀粉样血管病的病理学机制及影像学研究进展

脑淀粉样血管病（cerebral amyloid angiopathy，CAA）是淀粉样物质沉积于大脑皮质和软脑膜中、小动脉壁而引起的血管病变，以痴呆、精神症状、进行性反复多病灶出血为主要临床表现。脑淀粉样血管病的发病率国内外报道相仿，在无症状老年人群中的发生率〉30％，并随着年龄的增加而逐渐升高，与性别无明显相关性。脑淀粉样血管病为非高血压老年脑出血的重要原因之一，其所引起的脑出血占原发性脑出血的5％～10％，且可能参与不同类型老年痴呆的发病机制。笔就近年来散发性脑淀粉样血管病的发病机制和影像学研究进展进行综述。[第一段]     

脑淀粉样血管病相关炎症研究进展

脑淀粉样血管病相关炎症(CAA-I)是脑淀粉样血管病的罕见亚型,逐渐受到临床医师的关注。CAA-I组织病理学兼具脑淀粉样血管病和血管炎的特点;临床主要表现为认知功能减退、癫■发作、头痛等,具有较大的临床异质性;头部MRI检查是临床诊断及评估疗效的重要手段,可见T_2WI和FLAIR成像脑白质高信号病灶和多发脑微出血灶;免疫抑制治疗反应良好,与脑淀粉样血管病有一定差异。本文拟就CAA-I病因和病理生理学机制、病理学特点、临床表现、辅助检查、治疗等方面研究进展进行综述。     

脑淀粉样血管病研究进展

脑淀粉样血管病(cerebral amyloid angiopathy,CAA)是脑小血管疾病中的一类[1],主要特征是刚果红和硫磺素染色阳性的淀粉样物质进行性地沉积于脑血管,导致病变血管扩张、破裂,最终产生临床症状.     

脑淀粉样血管病研究进展

脑淀粉样血管病(cerebral amyloid angiopathy,CAA)是脑小血管疾病中的一类[1],主要特征是刚果红和硫磺素染色阳性的淀粉样物质进行性地沉积于脑血管,导致病变血管扩张、破裂,最终产生临床症状.     

淀粉样脑血管病相关性脑出血与高血压脑出血的临床研究

目的 探讨淀粉样脑血管病相关性脑出血(CAAH)与高血压脑出血(HICH)的临床、影像、手术预后的特点.方法 收集3年来6家三级甲等医院经手术治疗的101例自发性脑出血患者的临床资料,所有标本经送我院病理科诊断后分为CAAH组11例,HICH组90例.对两组临床表现、影像学特点、术后病死率进行统计分析.结果 CAAH组发病平均年龄高于HICH组,而术前偏瘫的发生率低于HICH组.CAAH组出血部位多在脑叶,而HICH组以基底节区最常见.CAAH组年龄≥70岁患者术后病死率高于HICH组.随访期内CAAH组2例再次出血,其中1例死亡,4例出院后因长期卧床并发肺部感染,1例出现左眼视力颞侧偏盲,生活基本自理.结论 CAAH在临床和影像学上具有一定的特征,年龄较大者预后较差.外科手术干预近期有较好的效果,术后肺部感染是导致预后不良的常见并发症.     

Cerebral amyloid angiopathy-related hemorrhage. Interaction of APOE epsilon2 with putative clinical risk factors.

BACKGROUND AND PURPOSE: Current evidence suggests that the apolipoprotein E (APOE for gene; apoE for protein) epsilon4 allele predisposes to cerebral amyloid angiopathy (CAA) whereas epsilon2 is associated with CAA-related hemorrhage (CAAH). The clinical risk factors for other forms of intracranial hemorrhage are a less-frequent feature of CAAH. In this study we examined potential clinical risk factors in patients with CAAH and assessed these with respect to APOE genotype. METHODS: Thirty-six patients were identified with a pathological diagnosis of CAAH. Clinical notes were reviewed to document age of hemorrhage onset, history of dementia, antiplatelet/anticoagulant medication, hypertension, minor head trauma, or transient neurological events. In a review of reported cases of CAAH, the frequency of these clinical features was also recorded. APOE genotypes were determined with use of polymerase chain reaction techniques. RESULTS: There were 24 women and 12 men; the mean age was 70.3 years. One third (n=12) had been taking antiplatelet medication, and a similar number were demented. Nine patients were hypertensive, and 4 had a history of recent minor head trauma. The relative frequency of each of these clinical features was similar to that in previous reports. Forty-four percent (16 of 36) possessed an epsilon2 allele. Antiplatelet or anticoagulant medication, hypertension, or minor head trauma were significantly more frequent antecedents of CAAH in epsilon2 carriers than in non-epsilon2 carriers (81% versus 35%, P=0.008), antiplatelet/anticoagulant medication in particular (P=0.038). CONCLUSIONS: Our findings suggest that antiplatelet or anticoagulant medication, hypertension, or minor head trauma are most likely to precipitate cerebral hemorrhage in patients with CAA who are also epsilon2 carriers. This may result from isoform-specific effects of apoE on the structure of amyloid-laden blood vessel walls.     

Apolipoprotein E (APOE) gene polymorphism and risk and prognosis in cerebral amyloid angiopathy-related haemorrhage

The authors present current opinions about the role of APOE (apolipoprotein E gene) genotype as a factor modifying risk, course and prognosis in haemorrhagic stroke of cerebral amyloid origin. The search for the role of genetics in haemorrhagic stroke has been ongoing for more than 15 years. One of the most frequently investigated genotypes in the context of intracerebral haemorrhages is the APOE genotype. Alleles APOE e2 and e4 have been established as risk factors for cerebral amyloid angiopathy (CAA), as well as for cerebral amyloid angiopathy-related haemorrhage (CAAH). Moreover, APOE genotype seems to determine prognosis in CAAH in terms of early mortality, as well as risk of recurrence. Current findings related to the association between different isoforms of apoE and haemorrhagic stroke due to CAA do not allow us to formulate any clinical recommendations yet.     

Cerebral amyloid angiopathy presenting as a brain tumor: case report

We describe the unusual case of a 45-year-old male patient harboring an intracranial mass due to cerebral amyloid angiopathy whose clinical and radiological features were those of a low grade glioma. Biopsy revealed cerebral amyloid angiopathy. The clinical, radiological and pathological findings are discussed as we review the available literature.     

Clinical Neuropathological Analysis of 10 Cases of Cerebral Amyloid Angiopathy-Related Cerebral Lobar Hemorrhage

ObjectiveThe clinical and pathological characteristics of 10 cases of cerebral amyloid angiopathy (CAA)-related cerebral lobar hemorrhage (CLH) that was diagnosed at autopsy were investigated to facilitate the diagnosis of this condition.MethodsThe clinical characteristics of 10 cases of CAA-related CLH were retrospectively reviewed, and a neuropathological examination was performed on autopsy samples.ResultsThe 10 cases included two with a single lobar hemorrhage and eight with multifocal lobar hemorrhages. In all of the cases, the hemorrhage bled into the subarachnoid space. Pathological examinations of the 10 cases revealed microaneurysms in two, double barrel-like changes in four, multifocal arteriolar clusters in five, obliterative onion skin-like intimal changes in four, fibrinoid necrosis of the vessels in seven, neurofibrillary tangles in eight, and senile plaques in five cases.ConclusionCAA-related CLHs were located primarily in the parietal, temporal, and occipital lobes. These hemorrhages normally consisted of multiple repeated CLHs that frequently bled into the subarachnoid space. CAA-associated microvascular lesions may be the pathological factor underlying CLH.     

Low avidity and level of serum anti-Aβ antibodies in patients with cerebral amyloid angiopathy-related cerebral hemorrhage

Cerebral amyloid angiopathy (CAA) is characterized by the deposition of the beta-amyloid protein (Aβ) in small cerebral vessels, which is considered a common cause of intracerebral hemorrhage (CAAH) in elderly people. Little is known about the properties of serum naturally occurring anti-Aβ antibodies in patients with CAAH. We investigated the avidity and levels of anti-Aβ antibodies in 20 patients and 20 age-matched healthy controls by an enzyme-linked immunosorbent assay with thiocyanate elution. Our study revealed that both the levels and the avidity of these endogenous anti-Aβ antibodies were lower in patients with CAAH than in controls, which may be a new mechanism for the impaired clearance of cerebral Aβ and have important implications for the development of immune-based therapeutic strategies for CAA.     

急性多灶性脑出血的病因、发病机制及临床分析

目的 探讨包性多灶性脑出血（ＡＭＣＨ的病因、发病机制及临床特点。方法 通过ＣＴ、病理和临床检查,对４７例病人进去观察分析。结果 ＡＭＣＨ占同期脑出血病人的３％。主要病因为高血压（７７％）,淀粉样脑血管病（４％）,脑血管畸形（４％）,瘤卒中（２％）,白血病（２％）,原因不明５例（１１％）,出血发限部位依次为基底节（５１％）,脑叶（３９％）,脑干和小脑（各５％）。按出血部位将其上脑蚌 型、小脑幕下型和     

载脂蛋白E基因多态性和脑淀粉样血管病相关脑出血的临床相关性研究

目的探讨载脂蛋白E（APOE）基因多态性与脑淀粉样血管病相关脑出血（CAAH）的相关性。 方法收集自2015年3月至2018年1月高密市人民医院神经外科收治的36例CAAH患者、51例高血压脑出血（HICH）患者,与同期50例健康体检者比较,检测其APOE基因多态性,比较APOE等位基因与CAAH的关系。 结果CAAH组等位基因ε2频率显著高于HICH组及健康对照组（P<0.05）,其余组间差异无统计学意义（P>0.05）。 结论APOEε2等位基因与CAAH有关联,可增加脑叶出血的风险。     

Cerebral amyloid angiopathy and thrombolysis-related intracerebral haemorrhage

Intracerebral haemorrhage is a complication of thrombolytic therapy for acute myocardial infarction, pulmonary embolism, and ischaemic stroke. There is increasing evidence that cerebral amyloid angiopathy (CAA), which itself can cause haemorrhage (CAAH), may be a risk factor for thrombolysis-related intracerebral haemorrhage. CAAH and thrombolysis-related intracerebral haemorrhage share some clinical features, such as predisposition to lobar or superficial regions of the brain, multiple haemorrhages, increasing frequency with age, and an association with dementia. In vitro work showed that accumulation of amyloid-beta peptide causes degeneration of cells in the walls of blood vessels, affects vasoactivity, and improves proteolytic mechanisms, such as fibrinolysis, anticoagulation, and degradation of the extracellular matrix. In a mouse model of CAA there is a low haemorrhagic threshold after thrombolytic therapy compared with that in wild-type mice. To date only a small number of anecdotal clinicopathological relations have been reported; neuroimaging advances and further study of the frequency and role of CAA in patients with thrombolysis-related intracerebral haemorrhage are required.     

The incidence of cerebral amyloid angiopathy in Alzheimer's disease.

The incidence and forms of cerebral amyloid angiopathy were studied in 15 cases of Alzheimer's disease using Congo red staining and polarization. Thirteen cases showed slight to severe involvement; two contained no amyloid vascular degeneration. There was a correlation between the presence of amyloid-rich plaques and cerebral amyloid angiopathy (especially the plaque-like angiopathy) but no correlation with "amyloid-poor" senile plaques or Alzheimer's neurofibrillary degeneration.