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Meta-Analysis
. 2022 Jan;18(1):10-28.
doi: 10.1002/alz.12366. Epub 2021 May 31.

Prevalence of cerebral amyloid angiopathy: A systematic review and meta-analysis

Lieke Jäkel  1 , Anna M De Kort  1 , Catharina J M Klijn  1 , Floris H B M Schreuder  1 , Marcel M Verbeek  1   2
Affiliations
Meta-Analysis

Prevalence of cerebral amyloid angiopathy: A systematic review and meta-analysis

Lieke Jäkel et al. Alzheimers Dement. 2022 Jan.

Abstract

Reported prevalence estimates of sporadic cerebral amyloid angiopathy (CAA) vary widely. CAA is associated with cognitive dysfunction and intracerebral hemorrhage, and linked to immunotherapy-related side-effects in Alzheimer's disease (AD). Given ongoing efforts to develop AD immunotherapy, accurate estimates of CAA prevalence are important. CAA can be diagnosed neuropathologically or during life using MRI markers including strictly lobar microbleeds. In this meta-analysis of 170 studies including over 73,000 subjects, we show that in patients with AD, CAA prevalence based on pathology (48%) is twice that based on presence of strictly lobar cerebral microbleeds (22%); in the general population this difference is three-fold (23% vs 7%). Both methods yield similar estimated prevalences of CAA in cognitively normal elderly (5% to 7%), in patients with intracerebral hemorrhage (19% to 24%), and in patients with lobar intracerebral hemorrhage (50% to 57%). However, we observed large heterogeneity among neuropathology and MRI protocols, which calls for standardized assessment and reporting of CAA.

Keywords: Alzheimer's disease; Boston criteria; MRI; amyloid; cerebral amyloid angiopathy; immunotherapy; intracerebral hemorrhage; meta-analysis; microbleeds; neuropathology; prevalence; systematic review.

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Figures

FIGURE 1
FIGURE 1
Flow chart of study selection
FIGURE 2
FIGURE 2
Forest plots showing the prevalence in Alzheimer's disease patients of moderate‐to‐severe cerebral amyloid angiopathy (CAA) pathology (A), strictly lobar cerebral microbleeds (B), and cortical superficial siderosis (C). CAA, cerebral amyloid angiopathy
FIGURE 3
FIGURE 3
Forest plots showing the prevalence in the general population of moderate‐to‐severe cerebral amyloid angiopathy (CAA) pathology (A), strictly lobar cerebral microbleeds (B), and cortical superficial siderosis (C). CAA, cerebral amyloid angiopathy
FIGURE 4
FIGURE 4
Forest plots showing the prevalence in cognitively normal elderly of moderate‐to‐severe cerebral amyloid angiopathy (CAA) pathology (A), probable CAA according to the (modified) Boston criteria (B), strictly lobar cerebral microbleeds (C), and cortical superficial siderosis (D). CAA, cerebral amyloid angiopathy
FIGURE 5
FIGURE 5
Forest plots showing the prevalence in patients with intracerebral hemorrhage of moderate‐to‐severe cerebral amyloid angiopathy (CAA) pathology (A), probable CAA according to the (modified) Boston criteria (B), strictly lobar cerebral microbleeds (C), and cortical superficial siderosis (D). CAA, cerebral amyloid angiopathy
FIGURE 6
FIGURE 6
Forest plots showing the prevalence in patients with lobar intracerebral hemorrhage of moderate‐to‐severe cerebral amyloid angiopathy (CAA) pathology (A), probable CAA according to the (modified) Boston criteria (B), and cortical superficial siderosis (C). CAA, cerebral amyloid angiopathy
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