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Updated: Dec 24, 2025
Longitudinal In Vivo Imaging of the Cerebrovasculature: Relevance to CNS Diseases
Published on: December 6, 2016
Cortical superficial siderosis progression in cerebral amyloid angiopathy: Prospective MRI study
Thanakit Pongpitakmetha1, Panagiotis Fotiadis1, Marco Pasi1
1From the Hemorrhagic Stroke Research Program, J. Philip Kistler Stroke Research Center, Department of Neurology (T.P., P.F., M.P., G.B., L.X., A.D.W., K.M.S., J.R., M.E.G., S.M.G., A.V., A.C.), and Division of Neurocritical Care and Emergency Neurology (J.R.), Massachusetts General Hospital, and MIND Informatics, Massachusetts General Hospital Biomedical Informatics Core (J.R.), Harvard Medical School, Boston; and Department of Pharmacology, Faculty of Medicine (T.P.), Chulalongkorn University, Bangkok, Thailand.
Objective:
To investigate the prevalence, predictors, and clinical relevance of cortical superficial siderosis (cSS) progression in cerebral amyloid angiopathy (CAA).
Methods:
Consecutive patients with symptomatic CAA meeting Boston criteria in a prospective cohort underwent baseline and follow-up MRI within 1 year. cSS progression was evaluated on an ordinal scale and categorized into mild (score 1-2 = cSS extension within an already present cSS focus or appearance of 1 new cSS focus) and severe progression (score 3-4 = appearance of ≥2 new cSS foci). Binominal and ordinal multivariable logistic regression were used to determine cSS progression predictors. We investigated future lobar intracerebral hemorrhage (ICH) risk in survival analysis models.
Results:
We included 79 patients with CAA (mean age, 69.2 years), 56 (71%) with lobar ICH at baseline. cSS progression was detected in 23 (29%) patients: 15 (19%) patients had mild and 8 (10%) severe progression. In binominal multivariable logistic regression, ICH presence (odds ratio [OR], 7.54; 95% confidence interval [CI], 1.75-53.52; p = 0.016) and baseline cSS (OR, 10.41; 95% CI, 2.84-52.83; p = 0.001) were independent predictors of cSS progression. In similar models, presence of disseminated (but not focal) cSS at baseline (OR, 5.58; 95% CI, 1.81-19.41; p = 0.004) was an independent predictor of cSS progression. Results were similar in ordinal multivariable logistic regression models. In multivariable Cox regression analysis, severe cSS progression was independently associated with increased future ICH risk (HR, 5.90; 95% CI, 1.30-26.68; p = 0.021).
Conclusions:
cSS evolution on MRI is common in patients with symptomatic CAA and might be a potential biomarker for assessing disease severity and future ICH risk. External validation of these findings is warranted.
