Acta Neuropathologica

, Volume 115, Issue 6, pp 599–609

Cerebral amyloid angiopathy and its relationship to Alzheimer’s disease

  • Dietmar Rudolf Thal
  • W. Sue T. Griffin
  • Rob A. I. de Vos
  • Estifanos Ghebremedhin
Review

DOI: 10.1007/s00401-008-0366-2

Cite this article as:
Thal, D.R., Griffin, W.S.T., de Vos, R.A.I. et al. Acta Neuropathol (2008) 115: 599. doi:10.1007/s00401-008-0366-2

Abstract

Cerebral amyloid angiopathy (CAA) is characterized by the deposition of the amyloid β-protein (Aβ) within cerebral vessels. The involvement of different brain areas in CAA follows a hierarchical sequence similar to that of Alzheimer-related senile plaques. Alzheimer’s disease patients frequently exhibit CAA. The expansion of CAA in AD often shows the pattern of full-blown CAA. The deposition of Aβ within capillaries distinguishes two types of CAA. One with capillary Aβ-deposition is characterized by a strong association with the apolipoprotein E (APOE) ε4 allele and by its frequent occurrence in Alzheimer’s disease cases whereas the other one lacking capillary Aβ-deposits is not associated with APOE ε4. Capillary CAA can be seen in every stage of CAA or AD-related Aβ-deposition. AD cases with capillary CAA show more widespread capillary Aβ-deposition than non-demented cases as well as capillary occlusion. In a mouse model of CAA, capillary CAA was associated with capillary occlusion and cerebral blood flow disturbances. Thus, blood flow alterations with subsequent hypoperfusion induced by CAA-related capillary occlusion presumably point to a second mechanism in which Aβ adversely affects the brain in AD in addition to its direct neurotoxic effects.

Keywords

Alzheimer’s disease Cerebral amyloid angiopathy Amyloid β-protein Cerebral blood flow Drainage 

Copyright information

© Springer-Verlag 2008

Authors and Affiliations

  • Dietmar Rudolf Thal
    • 1
  • W. Sue T. Griffin
    • 2
  • Rob A. I. de Vos
    • 3
  • Estifanos Ghebremedhin
    • 4
  1. 1.Laboratory of Neuropathology, Institute of PathologyUniversity of UlmUlmGermany
  2. 2.Donald W. Reynolds Center on AgingUAMS, Geriatric Research Education and Clinical Center, Veterans’ Affairs Medical CenterLittle RockUSA
  3. 3.Laboratorium Pathologie Oost NederlandEnschedeThe Netherlands
  4. 4.Institute for Clinical NeuroanatomyJ. W. Goethe UniversityFrankfurt am MainGermany