Chinese guideline for the diagnosis and management of cerebral amyloid angiopathy 2023

Title: Chinese guideline for the diagnosis and management of cerebral amyloid angiopathy 2023
Edition: Original
Classification: Standard guideline
Field: Diagnosis and Treatment
Countries and regions: China
Guidelines users: The users are clinicians, nurses, technicians and researchers engaged in neurological diseases related work in hospitals at all levels.
Evidence classification 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.
Development unit: Chinese Stroke Society, Chinese Medical Association. Peking Union Medical College Hospital
Registration time: 2023-12-04
Registration number: PREPARE-2023CN797
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.