Introduction Acute non-traumatic convexity subarachnoid haemorrhage (cSAH) is increasingly recognised in cerebral amyloid angiopathy (CAA). We investigated: (a) the overlap between acute cSAH and cortical superficial siderosis—a new CAA haemorrhagic imaging signature and (b) whether acute cSAH presents with particular clinical symptoms in patients with probable CAA without lobar intracerebral haemorrhage.
Methods MRI scans of 130 consecutive patients meeting modified Boston criteria for probable CAA were analysed for cortical superficial siderosis (focal, ≤3 sulci; disseminated, ≥4 sulci), and key small vessel disease markers. We compared clinical, imaging and cortical superficial siderosis topographical mapping data between subjects with versus without acute cSAH, using multivariable logistic regression.
Results We included 33 patients with probable CAA presenting with acute cSAH and 97 without cSAH at presentation. Patients with acute cSAH were more commonly presenting with transient focal neurological episodes (76% vs 34%; p<0.0001) compared with patients with CAA without cSAH. Patients with acute cSAH were also more often clinically presenting with transient focal neurological episodes compared with cortical superficial siderosis-positive, but cSAH-negative subjects with CAA (76% vs 30%; p<0.0001). Cortical superficial siderosis prevalence (but no other CAA severity markers) was higher among patients with cSAH versus those without, especially disseminated cortical superficial siderosis (49% vs 19%; p<0.0001). In multivariable logistic regression, cortical superficial siderosis burden (OR 5.53; 95% CI 2.82 to 10.8, p<0.0001) and transient focal neurological episodes (OR 11.7; 95% CI 2.70 to 50.6, p=0.001) were independently associated with acute cSAH.
Conclusions This probable CAA cohort provides additional evidence for distinct disease phenotypes, determined by the presence of cSAH and cortical superficial siderosis.
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Contributors Statistical analysis was conducted by AC: study concept and design, data collection, imaging analysis, statistical analysis, write-up. GB: study concept and design, data collection, imaging analysis, critical revisions. PF: imaging analysis, write-up, critical revisions. LX: data collection, critical revisions. AMA: data collection and management. MJ: data collection and management. KMS: data collection and management. EMG: critical revisions. JR: data collection, critical revisions. SMG: funding, project design, critical revisions. AV: funding, project design, write-up, critical revisions.
Funding This work was supported by NIH grants R01!AG026484 (S.M. Greenberg). Gregoire Boulouis was supported by a J. William Fulbright Scholarship and a Monahan Foundation Biomedical Research Grant. Andreas Charidimou receives postdoctoral support from the Bodossaki Foundation. This study is not industry sponsored.
Competing interests None declared.
Provenance and peer review Not commissioned; externally peer reviewed.
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