| 标题： | 中国脑淀粉样血管病诊治指南（2023版） |
| title： | Chinese guideline for the diagnosis and management of cerebral amyloid angiopathy 2023 |
| 版本： | 原创版 |
| version： | Original |
| 分类： | 标准指南 |
| classification： | Standard guideline |
| 领域： | 诊疗 |
| field： | Diagnosis and Treatment |
| 国家和地区： | 中国 |
| Country and region： | China |
| 指南使用者： | 使用人群为各级医院从事神经系统疾病相关工作的临床医师、护士、技术人员及科研人员。 |
| Guide users： | The users are clinicians, nurses, technicians and researchers engaged in neurological diseases related work in hospitals at all levels. |
| 证据分级方法： | Grade分级表 1. 高级证据：定义为非常确信真实的效应值接近效应估计。包括RCT、质量升高二级的观察性研究。 2. 中级证据：定义为对效应估计值有中等程度的信心，真实值有可能接近估计值，但仍存在二者大不相同的可能性。包括质量降低一级的RCT、质量升高一级的观察性研究。 3. 低级证据：定义为对效应估计值的确信程度有限，真实值可能与估计值大不相同。包括质量降低二级的RCT证据分级、观察性研究。 4. 极低级证据：定义为我们对效应估计值几乎没有信心，真实值很可能与估计值不大相同；包括质量降低三级的RCT、质量降低一级的观察性研究、系列病例观察、个案报道 |
| Evidence grading method： | GRADE approach 1. High: We are very confident that the true effect value is close to the estimated effect, including RCTs, observational studies with 2 elevated levels. 2. Moderate: We have moderate confidence in the estimated effect, where the true value is likely to be close to the estimate but there is still the possibility that the two are substantially different. This includes RCTs with 1 deducted level and observational studies with 1 elevated level. 3. Low: We have limited confidence in the estimated effect, with the possibility that the true value is likely to be substantially different from the estimate. It includes RCTs with 2 deducted level and observational studies. 4. Extremely low: We have little confidence in the estimated effect, and the true effect value is different from the estimated effect. It includes RCTs with 3 deducted level, observational studies with 1 deducted level, case-series and case report. |
| 制定单位： | 中华医学会神经病学分会脑血管病学组；中国医学科学院北京协和医院 |
| Formulating unit： | Chinese Stroke Society, Chinese Medical Association. Peking Union Medical College Hospital |
| 注册时间： | 2023-12-04 |
| Registration time： | |
| 注册编号： | PREPARE-2023CN797 |
| Registration number： | |
| 指南制订的目的： | 脑淀粉样血管病（cerebral amyloid angiopathy，CAA）是一类β淀粉样蛋白沉积在大脑皮层和柔脑膜小血管导致的年龄相关脑小血管病。淀粉样蛋白沉积继发血管壁完整性破坏可导致临床上复发性脑叶出血及年龄相关认知功能障碍和步态障碍。此外，CAA亦可表现为皮层凸面孤立蛛网模下腔出血相关淀粉样发作（TFNE）、炎性CAA相关亚急性认知或精神异常、无症状影像学标志物包括局限脑叶的微出血、皮层表浅含铁血黄素沉积/孤立皮层蛛网膜下腔出血、白质高信号、血管周围间隙和脑萎缩等。在CAA认识的早期，诊断主要依赖病理，目前公认的波士顿诊断标准综合了临床、影像及病理的特征。由于迄今没有针对CAA的疾病修饰治疗方法，因此部分复发性脑叶出血或严重认知功能损伤的CAA患者预后不良，及时识别和诊断对于预防抗栓相关脑出血非常重要。 |
| Purpose of the guideline： | Cerebral amyloid angiopathy (CAA) is one of the age-related cerebrovascular diseases caused by deposition of Amyloid-β on the cerebral cortex and leptomeningeal small vessels. The disruption of vascular wall integrity secondary to amyloid deposition may lead to recurrent lobar hemorrhage and age-related cognitive impairment and gait disorders in clinical practice. In addition, CAA may also present as transient focal neurologic events (TFNE) caused by isolated cortical/convexity subarachnoid hemorrhage, psychiatric abnormalities related to CAA-related inflammation as well as asymptomatic radiological markers including strict lobar microbleeds, cortical superficial siderosis/isolated cortical convexity subarachnoid hemorrhage, white matter hyperintensity, enlarged perivascular spaces, and cerebral atrophy. In the early time, the diagnosis of CAA mainly relied on pathology. The widely recognized Boston diagnostic criteria integrates the clinical, imaging, and pathological features. Due to the lack of disease modifying therapy for CAA, the prognosis of CAA patients with recurrent lobar hemorrhages or severe cognitive impairment is poor. So it is important to timely identify and diagnose CAA to reasonably prevent cerebral hemorrhage. |
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