From the Guidelines
Cerebral amyloid angiopathy (CAA) should be suspected in patients with suspected cerebral amyloid angiopathy, lobar intraparenchymal hemorrhage (IPH), older age, more than 10 cerebral microbleeds on MRI, disseminated cortical superficial siderosis on MRI, poorly controlled hypertension, previous history of spontaneous intracranial hemorrhage (ICH), genetic or acquired coagulopathy, or untreated symptomatic vascular malformation or aneurysm. When evaluating patients for CAA, it is essential to consider the presence of lobar hemorrhages, especially if recurrent or multiple, as well as microbleeds visible on gradient-echo MRI sequences or unexplained cognitive decline 1. Key risk factors that increase suspicion of CAA include:
- Hypertension
- Anticoagulant or antiplatelet therapy
- History of dementia, particularly Alzheimer's disease
- Transient focal neurological episodes, sometimes called "amyloid spells," which can mimic transient ischemic attacks
- Presence of superficial siderosis on MRI CAA results from amyloid-beta protein deposition in cerebral blood vessel walls, causing vessel fragility and increased bleeding risk, making careful consideration of anticoagulants and antiplatelets essential in suspected cases 1. The diagnostic evaluation of CAA may involve brain MRI without contrast to detect microhemorrhage associated with cerebral amyloid angiopathy and to monitor for amyloid-related imaging abnormalities (ARIA) in patients receiving disease-modifying therapies 1. In clinical practice, a multidisciplinary approach is crucial for estimating risk-reward calculus and integrating usual practice, recommendations from other guidelines, and limited evidence to develop an opinion regarding cognitive functional status and syndromic diagnosis.
From the Research
Clinical Presentation of Cerebral Amyloid Angiopathy
Cerebral amyloid angiopathy (CAA) should be suspected in patients with the following clinical presentations:
- Lobar intracerebral hemorrhages (ICH) in elderly patients 2, 3, 4
- Cognitive decline and episodic transient neurological symptoms 2, 5
- Focal neurological deficits, cognitive decline, and transient focal neurological episodes (TFNEs), also known as "Amyloid Spells" 5, 6
- History of recurrent ICH, especially in patients with cortical superficial siderosis (cSS) or cortical subarachnoid hemorrhage (cSAH) 2, 6
Neuroimaging Markers
CAA can be suspected based on the following neuroimaging markers:
- Lobar cerebral microbleeds (CMBs) 4, 6
- Cortical superficial siderosis (cSS) 2, 5, 6
- Lobar ICH 3, 4, 6
- White matter hyperintensities and cerebral edema 5
- Visible centrum semiovale perivascular spaces, lobar located lacunes, and cortical atrophy 2
Patient Characteristics
CAA is more common in: