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Abstract
Objective To investigate differential atrophy patterns based on the presence of cortical superficial siderosis (cSS) and the role of cSS in predicting amyloid positivity in memory clinic patients fulfilling the diagnostic criteria for probable cerebral amyloid angiopathy (CAA).
Methods We retrospectively collected data from 44 cognitively impaired patients with probable CAA who underwent 3-dimensional, T1-weighted MRIs (cSS+, n = 27; cSS−, n = 17). Amyloid-positive patients with Alzheimer disease (AD) (n = 56) and amyloid-negative cognitively normal participants (n = 34) were recruited as controls. Among the patients with CAA who underwent amyloid-PET scans (75.0%), we investigated whether amyloid-negative cases were unevenly distributed based on cSS presentation. APOE genotypes, Mini-Mental State Examination scores, and cortical atrophy pattern along with hippocampal volume were compared across groups.
Results Ten patients with probable CAA presented amyloid negativity and all of them belonged to the cSS− group (58.8%). Compared to the cSS− group, the cSS+ group presented higher APOE ε4 frequency, worse memory dysfunction, and lower hippocampal volume. Compared with cognitively normal participants, the cSS+ group exhibited atrophy in the precuneus, posterior cingulate, parietotemporal, superior frontal, and medial temporal areas, a pattern similar to AD-specific atrophy. The cSS− group exhibited atrophy in the parietotemporal, superior frontal, and precentral regions.
Conclusion Our findings imply that the current version of the Boston criteria may not be sufficient enough to remove non-CAA cases from a cognitively impaired population, especially in the absence of cSS. Patients with probable CAA presenting cSS appear to reflect a CAA phenotype that shares pathologic hallmarks with AD, providing insight into the CAA-to-AD continuum.
Glossary
- AD=
- Alzheimer disease;
- ANCOVA=
- analysis of covariance;
- CAA=
- cerebral amyloid angiopathy;
- CAA-CI=
- cerebral amyloid angiopathy–associated cognitive impairment;
- CDR=
- Clinical Dementia Rating;
- CMB=
- cerebral microbleed;
- CN=
- cognitively normal;
- cSS=
- cortical superficial siderosis;
- FBB=
- florbetaben;
- GRE T2*=
- gradient-recalled echo, T2*-weighted;
- ICH=
- intracerebral hemorrhage;
- MCI=
- mild cognitive impairment;
- PCC=
- posterior cingulate cortex;
- PiB=
- Pittsburgh compound B;
- SVCI=
- subcortical vascular cognitive impairment;
- WMH=
- white matter hyperintensity
Footnotes
Go to Neurology.org/N for full disclosures. Funding information and disclosures deemed relevant by the authors, if any, are provided at the end of the article.
↵* These authors contributed equally to this work as first authors.
- Received October 4, 2017.
- Accepted in final form February 26, 2018.
- © 2018 American Academy of Neurology
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