Influence of Cortical Superficial Siderosis in Patients with Mixed Location Cerebral Microbleeds and Intracerebral Hemorrhage
Alvin Das1, Elif Gokcal2, Alessandro Biffi2, Robert Regenhardt2, Avia Abramovitz Fouks2, Anand Viswanathan2, W. Kimberly2, Joshua Goldstein3, Lee Schwamm2, Jonathan Rosand2, Steven Greenberg2, Edip Gurol2
1Department of Neurology, Beth Israel Deaconess Medical Center, 2Department of Neurology, 3Department of Emergency Medicine, Massachusetts General Hospital
Objective:
We aim to test whether the presence of cortical superficial siderosis (cSS), a marker strongly associated with cerebral amyloid angiopathy (CAA), is likely to modify the underlying microangiopathy from hypertensive cerebral small vessel disease (HTN-cSVD) to CAA in patients with a combination of lobar and deep intracerebral hemorrhage (ICH)/cerebral microbleeds (CMBs) (mixed ICH/CMBs).
Background:
HTN-cSVD has been shown to be the predominant microangiopathy in patients with mixed ICH/CMBs.
Design/Methods:

Brain MRIs from a prospective database of consecutive non-traumatic ICH patients admitted to a single referral center (2003 to 2019) were reviewed for the presence of CMBs, cSS, and non-hemorrhagic CAA markers (lobar lacunes, centrum semiovale enlarged perivascular spaces (CSO-EPVS), and multispot pattern of leukoaraiosis). The frequency of CAA markers and left ventricular hypertrophy (LVH), a marker for hypertensive end-organ damage, were compared between mixed ICH/CMB patients with cSS (mixed+cSS) and without cSS (mixed–cSS) in univariate and multivariate models.

Results:

Of 1824 ICH patients, 40 had mixed+cSS and 256 had mixed–cSS. LVH was less common in patients with mixed+cSS compared to those with mixed–cSS (34% vs. 59%, p = 0.01). The frequency of multispot pattern (18% vs. 4%, p < 0.01) and severe CSO-EPVS (33% vs. 11%, p < 0.01) were higher in patients with mixed+cSS compared to mixed–cSS, whereas lobar lacune frequency was similar (23% vs. 20%, p = 0.67). In a logistic regression model, older age (aOR 1.04 per year, 95% CI [1.01–1.08], p = 0.01), presence of multispot pattern (aOR 5.82, 95% CI [1.82–18.60], p < 0.03), severe CSO-EPVS (aOR 4.08, 95% CI [1.73–9.61], p < 0.01), and LVH (aOR 0.43, 95% CI [0.20–0.94], p = 0.03) were independently associated with mixed+cSS after further adjustment for sex and hypertension.

Conclusions:

CAA-related markers are common among patients with mixed ICH/CMBs and cSS, suggesting that CAA may be the underlying microangiopathy in such patients.

10.1212/WNL.0000000000204075