Charidimou A, Boulouis G, Xiong L, et al. Cortical superficial siderosis and first-ever cerebral hemorrhage in cerebral amyloid angiopathy. Neurology. 2017;88(17):1607-1614. doi:10.1212/WNL.0000000000003866.
Cortical superficial siderosis (cSS) on T2*-GRE or SWI is a strong hemorrhagic signature of cerebral amyloid angiopathy (CAA)—a common small vessel disease characterized by cerebrovascular amyloid deposition affecting superficial cortical microvascular networks, leading to spontaneous lobar intracerebral hemorrhage (ICH). Cortical superficial siderosis results from bleeding episodes within or adjacent to cortical sulci, presumably from amyloid-laden superficial cortical and leptomeningeal arterioles. Cortical superficial siderosis is a common manifestation of cerebral amyloid angiopathy, being found in 40%–60% of patients.
In this study, consecutive patients meeting modified Boston criteria for probable CAA in the absence of ICH from a single-center cohort were analyzed. Cortical superficial siderosis and other small vessel disease MRI markers were assessed according to recent consensus recommendations. Patients were followed prospectively for future incident symptomatic lobar ICH.
The cohort included 236 patients with probable CAA without lobar ICH at baseline. Cortical superficial siderosis prevalence was 34%. During a median follow-up of 3.26 years, 27 of 236 patients (11.4%) experienced a first-ever symptomatic lobar ICH. Cortical superficial siderosis was a predictor of time until first ICH. The risk of symptomatic ICH at 5 years of follow-up was 19% for patients with cortical superficial siderosis at baseline vs 6% for patients without cortical superficial siderosis. In multivariable Cox regression models, cortical superficial siderosis presence was the only independent predictor of increased symptomatic ICH risk during follow-up.
The authors found that cortical superficial siderosis on T2*-GRE/SWI MRI is associated with an increased risk of future first-ever symptomatic lobar ICH. The prognostic value of cSS in this setting was strong and independent of age and other neuroimaging markers of CAA severity, including lobar cerebral microbleed burden and WMH. Hence, cortical …