Pathologically Confirmed Cerebral Amyloid Angiopathy with No Radiological Sign in a Patient with Early Onset Alzheimer’s Disease
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Cerebral amyloid angiopathy (CAA) is associated with perivascular disruption, which is caused by progressive amyloid-beta (Aβ)deposition in vessels. Previous autopsy studies have shown that the prevalence of CAA in Alzheimer’s disease (AD) is 70% to 90%. CAA is principally characterized by restricted lobar microbleeds (MBs), which can be detected by gradient-echo T2* (GRE) andsusceptibility-weighted imaging (SWI). We herein report on a 62-year-old man who presented with 8 years of memory impairment. The apolipoprotein E (APOE) genotype was ε4/ε4, and a brain GRE performed 28 months before death revealed mild atrophyand no MBs. At autopsy, the patient scored “A3, B3, C3” according to the National Institute on Aging-Alzheimer’s Associationguidelines; the patient thus exhibited a high level of AD neuropathological changes. Furthermore, immunohistochemical stainingfor Aβ showed antibody accumulation and severe cerebral amyloid angiopathic changes in numerous vessels with amyloiddeposits. Our case suggests that radiological CAA markers, such as cerebral microbleed (CMB) or cerebral superficial siderosis,may not suffice to detect amyloid angiopathy in cerebral vessels. CAA should therefore be considered as a combined pathology inAPOE ε4 homozygotes with AD, even if such patients do not exhibit CMB on MRI.
Abstract
Cerebral amyloid angiopathy (CAA) is associated with perivascular disruption, which is caused by progressive amyloid-beta (Aβ)deposition in vessels. Previous autopsy studies have shown that the prevalence of CAA in Alzheimer’s disease (AD) is 70% to 90%. CAA is principally characterized by restricted lobar microbleeds (MBs), which can be detected by gradient-echo T2* (GRE) andsusceptibility-weighted imaging (SWI). We herein report on a 62-year-old man who presented with 8 years of memory impairment. The apolipoprotein E (APOE) genotype was ε4/ε4, and a brain GRE performed 28 months before death revealed mild atrophyand no MBs. At autopsy, the patient scored “A3, B3, C3” according to the National Institute on Aging-Alzheimer’s Associationguidelines; the patient thus exhibited a high level of AD neuropathological changes. Furthermore, immunohistochemical stainingfor Aβ showed antibody accumulation and severe cerebral amyloid angiopathic changes in numerous vessels with amyloiddeposits. Our case suggests that radiological CAA markers, such as cerebral microbleed (CMB) or cerebral superficial siderosis,may not suffice to detect amyloid angiopathy in cerebral vessels. CAA should therefore be considered as a combined pathology inAPOE ε4 homozygotes with AD, even if such patients do not exhibit CMB on MRI.
참고문헌 (12)
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