Abstract
We investigated the prevalence and clinical-radiologic associations of cortical superficial siderosis (cSS) in patients with probable cerebral amyloid angiopathy (CAA) compared to those with intracerebral hemorrhage (ICH) not attributed to CAA. We conducted a retrospective multicenter cohort study of 120 patients with probable CAA and 2 comparison groups: 67 patients with either single lobar ICH or mixed (deep and lobar) hemorrhages; and 22 patients with strictly deep hemorrhages. We rated cSS, ICH, white matter changes, and cerebral microbleeds. cSS was detected in 48 of 120 (40%; 95% confidence interval [CI]: 31.2%-49.3%) patients with probable CAA, 10 of 67 (14.9%; 95% CI: 7.4%-25.7%) with single lobar ICH or mixed hemorrhages, and 1 of 22 (4.6%; 95% CI: 0.1%-22.8%) patients with strictly deep hemorrhages (p < 0.001 for trend). Disseminated cSS was present in 29 of 120 (24%; 95% CI: 16.8%-32.8%) patients with probable CAA, but none of the other patients with ICH (p < 0.001). In probable CAA, age (odds ratio [OR]: 1.09; 95% CI: 1.03-1.15; p = 0.002), chronic lobar ICH (OR: 3.94; 95% CI: 1.54-10.08; p = 0.004), and a history of transient focal neurologic episodes (OR: 11.08; 95% CI: 3.49-35.19; p < 0.001) were independently associated with cSS. However, cSS occurred in 17 of 48 patients with probable CAA (35.4%; 95% CI: 22.2%-50.5%) without chronic lobar ICH. cSS (particularly if disseminated) is a common and characteristic feature of CAA. Chronic lobar ICH is an independent risk factor for cSS, but the causal direction and mechanism of association are uncertain. Hemorrhage into the subarachnoid space, independent of previous (chronic) lobar ICH, must also contribute to cSS in CAA. Transient focal neurologic episodes are the strongest clinical marker of cSS.
Highlights
GLOSSARY CAA 5 cerebral amyloid angiopathy; CI 5 confidence interval; cerebral microbleeds (CMBs) 5 cerebral microbleed; cSAH 5 convexity subarachnoid hemorrhage; cSS 5 cortical superficial siderosis; FLAIR 5 fluid-attenuated inversion recovery; ICH 5 intracerebral hemorrhage; OR 5 odds ratio; T2*-GRE 5 T2*-weighted gradient recalled echo
The final cohort consisted of 209 patients: 120 patients with probable CAA (9 with supportive pathology) based on the Boston criteria and 89 patients with other ICH not fulfilling the Boston criteria for probable CAA, forming 2 comparison groups—67 patients with a single lobar ICH or mixed
We detected cSS in 15% of patients with a single lobar ICH or mixed hemorrhages; in most of these patients with mixed hemorrhages, lobar CMBs were present, suggesting that they might harbor some degree of CAA pathology
Summary
We conducted a retrospective multicenter cohort study of 120 patients with probable CAA and 2 comparison groups: 67 patients with either single lobar ICH or mixed (deep and lobar) hemorrhages; and 22 patients with strictly deep hemorrhages. In probable CAA, age (odds ratio [OR]: 1.09; 95% CI: 1.03–1.15; p 5 0.002), chronic lobar ICH (OR: 3.94; 95% CI: 1.54–10.08; p 5 0.004), and a history of transient focal neurologic episodes (OR: 11.08; 95% CI: 3.49–35.19; p , 0.001) were independently associated with cSS. Sporadic cerebral amyloid angiopathy (CAA) is a common age-related small-vessel disease caused by progressive deposition of amyloid-b in the walls of small arteries, arterioles, and capillaries in the cerebral cortex and overlying leptomeninges.[1] CAA is most often recognized in life by symptomatic lobar intracerebral hemorrhage (ICH) in elderly patients.[1,2,3] CAA is associated with characteristic MRI findings including lobar cerebral microbleeds (CMBs) and white matter hyperintensities (leukoaraiosis).[1,4]. We sought to determine the prevalence and extent of cSS in a European multicenter cohort of patients with clinical-radiologic probable CAA, and investigate its associations with other imaging findings including CMBs, white matter changes, and ICH. We hypothesized that cSS 1) is common in subjects with probable CAA, and more prevalent compared to comparison subjects with other spontaneous ICH not attributed to probable CAA, and 2) is associated with other hemorrhagic manifestations of CAA (lobar ICH and lobar CMBs)
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