Clinical and radiologic distinctions between familial cavernous malformation syndrome and cerebral amyloid angiopathy.

ABSTRACT:

Purpose

Familial cerebral cavernous malformation syndrome (FCCM) is characterized by multiple hemorrhagic lesions and is sometimes mistaken for cerebral amyloid angiopathy (CAA).

Methods

We compared clinical and radiologic characteristics in patients with definite (N = 32) and presumed FCCM (n = 76) to patients with definite (N = 29) and probable CAA (N = 21).

Results

Patients with CAA were older (78.6 years CAA vs. 43.4 FCCM; p < 0.0001), had cognitive complaints (66.0% CAA vs. 8.3% FCCM; p < 0.0001), and less likely to have a family history (4.0% CAA vs. 50.9% FCCM; p < 0.0001). FCCM patients were more likely to have at least 1 Zabramski type 2 lesion (0 CAA vs. 79.6% FCCM; p < 0.0001). Presence of any subcortical white matter hemorrhagic lesion (23.0% CAA vs. 99.1%

SUBMITTER: Flemming KD 

PROVIDER: S-EPMC11666656 | biostudies-literature | 2024 Dec

REPOSITORIES: biostudies-literature