Cerebral amyloid angiopathy (CAA) is characterized by cerebrovascular amyloid deposition and is a cause of cerebral hemorrhage in older individuals. It has a close association with Alzheimer's disease, with a prevalence of around 80-90% in AD patients. CAA becomes more common with age, affecting around half of elderly individuals. While there are no definitive treatments, hematoma evacuation appears relatively safe for younger patients with CAA-related hemorrhages.

1. Cerebral Amyloid Angiopathy
(CAA)
Ade Wijaya, MD – December 2017

2. Outline:
• Introduction
• Classiffication
• Epidemiology
• Pathology
• Pathophysiology
• Clinical features
• Diagnostic criteria
• Biomarkers
• Risk factors
• Treatment
• Summary

3. Introduction
• Cerebrovascular amyloid deposition
• A cause of normotensive cerebral hemorrhage in older individuals
• Close association with Alzheimer’s disease (AD)
• Important but underrecognized
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4. Classification
Yamada M, Naiki H. Cerebral amyloid angiopathy. Prog Mol Biol Transl Sci. 2012;51:41–78.

5. Epidemiology
• The prevalence of CAA increases with age
• Half of elderly individuals
• CAA is commonly observed in AD with a prevalence of about 80%-90%
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6. Pathology
CAA and CAA-associated vasculopathies.
Massive amyloid deposition of amyloid
fibrils with degeneration of smooth muscle
cells in the media (A). Microaneurysmal
dilatation (arrow) with fibrinoid necrosis
(*) (B). Thickening of the intima (arrow)
and double barreling of vascular walls
(arrowheads) (C). (A, electron micrograph,
bar=1 µm; B, Congo red, original
magnification 110×; C, Congo red, original
magnification 170×).
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7. Pathophysiology
Yamada M. Predicting cerebral amyloid angiopathy-related intracerebral hemorrhages and other cerebrovascular disorders in Alzheimer's disease. Front Neurol. 2012;3:64.

8. Clinical Features
• Intracerebral hemorrhages (Symptomatic lobar intracerebral hemorrhages & Cortical
microhemorrhages)
• Cortical superficial siderosis/focal convexity subarachnoid hemorrhages
• White matter disease and cortical infarction
• Dementia
• CAA-related inflammation
Yamada, M. (2015). Cerebral Amyloid Angiopathy: Emerging Concepts. Journal of Stroke, 17(1), 17–30. http://doi.org/10.5853/jos.2015.17.1.17

9. Boston criteria were
proposed for the diagnosis of
CAA-related ICH
Linn J, Halpin A, Demaerel P, Ruhland J, Giese AD, Dichgans M, et al.
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10. Yamada, M. (2015). Cerebral Amyloid Angiopathy: Emerging Concepts. Journal of Stroke, 17(1), 17–30. http://doi.org/10.5853/jos.2015.17.1.17

11. Yamada, M. (2015). Cerebral Amyloid Angiopathy: Emerging Concepts. Journal of Stroke, 17(1), 17–30. http://doi.org/10.5853/jos.2015.17.1.17

12. Treatment
• No treatment to halt or reverse β-amyloid deposition
• Hematoma evacuation appears relatively safe in patients <75 years of age
without intraventricular extension
• Tramiprosate
• Immunosuppresants
• Corticosteroids
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Greenberg SM, Rosand J, Schneider AT, Creed Pettigrew L, Gandy SE, Rovner B, et al. A phase 2 study of tramiprosate for cerebral amyloid angiopathy. Alzheimer Dis Assoc Disord 2006;20:269-74.

13. Summary
• Cerebrovascular amyloid deposition
• A cause of normotensive cerebral hemorrhage in older individuals
• Close association with Alzheimer’s disease (AD)
• Elderly individuals
• No definitive treatments