Abstract
Background: The clinical presentation of cerebral amyloid angiopathy (CAA) includes not only hemorrhagic stroke, but also diverse syndromes such as transient focal neurologic symptoms, progressive cognitive dysfunction, subacute confusion, and seizures. We performed a systematic analysis of the neuroimaging features of these presentations to help elucidate their underlying pathophysiologies. Methods: We performed a retrospective review of all patients seen at Massachusetts General Hospital from 2000-2011 with 1) diagnosis of probable/definite CAA by Boston criteria, 2) clinical presentation other than hemorrhagic stroke, and 3) available MR images (including T2*-weighted, diffusion-weighted, and FLAIR). Review of clinical data for 44 subjects meeting these criteria (performed blinded to neuroimaging) yielded 3 categories of presentation: transient focal motor, sensory, or language symptoms (n=15; mean±SD age 74.4±8.1), memory/cognitive impairment over months-years (n=15; age 71.2±10.7), and subacute headache, confusion, generalized seizure, or syncope (n=14; age 73.6±9.8). Images were analyzed without knowledge of clinical symptoms for hemorrhagic lesions, acute infarcts, and regional T2-hyperintensities. Results: Superficial siderosis in cortical sulci (Panel A) was present in 10 of 15 (67%) patients presenting with transient focal symptoms versus 7 of 29 (24%) in the other subgroups (p<0.01). Most locations of superficial siderosis corresponded with the localization of the patient’s transient symptoms. Conversely, a pattern of T2-hyperintensities extending to subcortical white matter and overlying cortex (Panel B) was present in 8 of 14 (57%) patients presenting with headache, confusion, or seizure/syncope versus 3 of 30 (10%) in the other subgroups (p<0.005). Conclusions: These results suggest that the underlying trigger for CAA-related transient focal neurologic symptoms may often be superficial siderosis. They also support an association between the alternative presentation of headache, confusion, or seizure/syncope and T2-hyperintensities suggestive of the inflammatory subtype of CAA.
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