Cerebral amyloid angiopathy

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Cerebral amyloid angiopathy Classification and external resources
DiseasesDB	32874
eMedicine	neuro/628
MeSH	D016657

Cerebral amyloid angiopathy, also known as congophilic angiopathy, is a form of angiopathy in which the amyloid protein associated with Alzheimer's disease, amyloid-β (Aβ), deposits in the walls of the blood vessels of the brain. The term congophilic is used because the presence of the abnormal aggregations of amyloid can be demonstrated by microscopic examination of brain tissue after application of a special stain called Congo red.

Contents 1 Presentation 2 Diagnosis 3 Types 4 References

[edit] Presentation

Amyloid deposition predisposes these blood vessel to failure, increasing the risk of a hemorrhagic stroke. Because it is the same amyloid protein that is associated with Alzheimer's dementia such brain hemorrhages are more common in people who suffer from Alzheimer's, however they can also occur in those who have no history of dementia. The hemorrhage within the brain is usually confined to a particular lobe and this is slightly different compared to brain hemorrhages which occur as a consequence of high blood pressure (hypertension) - a more common cause of a hemorrhagic stroke (or cerebral hemorrhage).

[edit] Diagnosis

Susceptibility weighted imaging has been proposed as a tool for identifying microhemorrhages.^[1]

[edit] Types

It is usually associated with amyloid beta.^[2] However, there are other types:

• the "Icelandic type" is associated with Cystatin C.^[3]
• the "British type" is associated with ITM2B (also known as "BRI").^[4]

[edit] References

1. ^ Haacke EM, DelProposto ZS, Chaturvedi S, et al (February 2007). "Imaging cerebral amyloid angiopathy with susceptibility-weighted imaging". AJNR Am J Neuroradiol 28 (2): 316–7. PMID 17297004. 
2. ^ Chao CP, Kotsenas AL, Broderick DF (2006). "Cerebral amyloid angiopathy: CT and MR imaging findings". Radiographics 26 (5): 1517–31. doi:10.1148/rg.265055090. PMID 16973779. 
3. ^ Nagai A, Terashima M, Sheikh AM, et al (2008). "Involvement of cystatin C in pathophysiology of CNS diseases". Front. Biosci. 13: 3470–9. PMID 18508448. 
4. ^ Vidal R, Frangione B, Rostagno A, et al (June 1999). "A stop-codon mutation in the BRI gene associated with familial British dementia". Nature 399 (6738): 776–81. doi:10.1038/21637. PMID 10391242. 

• Brain, March 1, 2005; 128(3): 500-515. A-beta-related Angiitis: Primary Angiitis of the CNS associated with CAA. Scolding, N.J. etal.
• Brain Pathology, July 2002, 12(3): 343. Sporadic and Familial CAA.
• J. Clin. Invest., October 1, 2005; 115 (10): 2793-2800. The Potential Role of A-beta in the Pathogenesis of Age-related Macular Degeneration.
• Stroke, 1996; 27: 1155-1162. Immune Reactions Associated with CAA. Yamada, etal.
• Stroke, July 1, 1997; 28(7): 1418-1422. Diagnosis of CAA: Sensitivity and Specificity of Cortical Biopsy. Greenberg, S.M. etal.

v • d • e Metabolic disease: amyloidosis (E85, 277.3) Forms of amyloid Serum amyloid P component - Serum amyloid A - Transthyretin - Beta-2 microglobulin - Bence Jones protein/Multiple myeloma - Amyloid precursor protein/Amyloid beta Systemic amyloidosis AL amyloidosis - AA amyloidosis - Haemodialysis-associated amyloidosis - Senile systemic amyloidosis - Finnish type amyloidosis - Familial Mediterranean fever Organ-limited amyloidosis heart (Cardiac amyloidosis) - brain (Familial amyloid neuropathy, Cerebral amyloid angiopathy, Alzheimer's disease)