[Clinical features of cerebral amyloid angiopathy characterized by cortical superficial siderosis]

Objective: To study the clinical and imaging characteristics of cerebral amyloid angiopathy characterized by cortical superficial siderosis and improve clinicians' understanding of the disease. Methods: Retrospective analysis was performed on 16 patients with cerebral amyloid angiopathy characterized by cortical superficial siderosis from June 2013 to August 2016 in Beijing Hospital, and the information including epidemiological data, clinical features, cranial MRI and electroencephalogram (EEG) results were analyzed. Results: The ratio of male to female in 16 patients was 1.67∶1, and the average age of onset was 73 (69-79) years. The most common clinical symptoms were transient focal neurological episodes (TFNEs)(12/16). Cranial MRI showed localized (9/16) and diffuse type cortical superficial siderosis (7/16); few of them were associated with different degrees of cerebral microbleeds. Most of the EEG findings were normal (6/9) and a few showed focal slow waves (3/9). During a mean follow-up of 17 (17±11) months, 5 patients developed repeated TFNE, of which 1 had cerebral hemorrhage. Conclusions: Cerebral amyloid angiopathy characterized by cortical superficial siderosis occurs predominantly in the elderly. TFNE is the most common clinical manifestation. Cranial MRI is the most important diagnostic method, and antithrombotic therapy should be avoided as much as possible.