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Pathologically Confirmed Cerebral Amyloid Angiopathy with No Radiological Sign in a Patient with Early Onset Alzheimer's Disease.

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) and susceptibility-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 atrophy and no MBs. At autopsy, the patient scored "A3, B3, C3" according to the National Institute on Aging-Alzheimer's Association guidelines; the patient thus exhibited a high level of AD neuropathological changes. Furthermore, immunohistochemical staining for Aβ showed antibody accumulation and severe cerebral amyloid angiopathic changes in numerous vessels with amyloid deposits. 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 in APOE ε4 homozygotes with AD, even if such patients do not exhibit CMB on MRI.
AuthorsSeung Joo Kim, Youjeong Seo, Hee Jin Kim, Duk L Na, Sang Won Seo, Yeshin Kim, Yeon Lim Suh
JournalYonsei medical journal (Yonsei Med J) Vol. 59 Issue 6 Pg. 801-805 (Aug 2018) ISSN: 1976-2437 [Electronic] Korea (South)
PMID29978619 (Publication Type: Case Reports)
Copyright© Copyright: Yonsei University College of Medicine 2018.
Chemical References
  • Apolipoprotein E4
  • Apolipoproteins E
Topics
  • Age of Onset
  • Alzheimer Disease (pathology)
  • Apolipoprotein E4
  • Apolipoproteins E (genetics)
  • Brain (diagnostic imaging, pathology)
  • Cerebral Amyloid Angiopathy (diagnostic imaging, pathology)
  • Female
  • Genotype
  • Humans
  • Magnetic Resonance Imaging
  • Male
  • Middle Aged

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