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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,[1] is a form of angiopathy in which amyloid deposits form in the walls of the blood vessels of the central nervous system.[2] 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

[edit] Causes

An immune mechanism has been proposed.[3]

[edit] Presentation

Amyloid deposition predisposes these blood vessels to failure, increasing the risk of a hemorrhagic stroke. Since this can be caused by the same amyloid protein that is associated with Alzheimer's dementia[4] 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

Amyloidangiopathie Kongorot.jpg
Amyloidangiopathie Kongorot doppelbrechend.jpg

Susceptibility weighted imaging has been proposed as a tool for identifying CAA-related microhemorrhages.[5]

Biopsies also play a role in diagnosing the condition.[6]

[edit] Types

It is usually associated with amyloid beta.[7] This form has been associated with "Abeta-related angiitis".[8]

However, there are other types:

  • the "Icelandic type" is associated with Cystatin C.[9]
  • the "British type" is associated with ITM2B (also known as "BRI").[10]

Research is currently being conducted to determine if there is a link between cerebral amyloid angiopathy and ingestion of excessive quantities of aluminium, as occurred in the Camelford water pollution incident.[11]

[edit] References

  1. ^ Exley C, Esiri MM (July 2006). "Severe cerebral congophilic angiopathy coincident with increased brain aluminium in a resident of Camelford, Cornwall, UK". J. Neurol. Neurosurg. Psychiatr. 77 (7): 877–9. doi:10.1136/jnnp.2005.086553. PMID 16627535. http://jnnp.bmj.com/cgi/pmidlookup?view=long&pmid=16627535. 
  2. ^ Revesz T, Holton JL, Lashley T, et al. (July 2002). "Sporadic and familial cerebral amyloid angiopathies". Brain Pathol. 12 (3): 343–57. PMID 12146803. 
  3. ^ Yamada M, Itoh Y, Shintaku M, et al. (July 1996). "Immune reactions associated with cerebral amyloid angiopathy". Stroke 27 (7): 1155–62. PMID 8685920. http://stroke.ahajournals.org/cgi/pmidlookup?view=long&pmid=8685920. 
  4. ^ Thal DR, Griffin WS, de Vos RA, Ghebremedhin E (June 2008). "Cerebral amyloid angiopathy and its relationship to Alzheimer's disease". Acta Neuropathol. 115 (6): 599–609. doi:10.1007/s00401-008-0366-2. PMID 18369648. 
  5. ^ 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. http://www.ajnr.org/cgi/pmidlookup?view=long&pmid=17297004. 
  6. ^ Greenberg SM, Vonsattel JP (July 1997). "Diagnosis of cerebral amyloid angiopathy. Sensitivity and specificity of cortical biopsy". Stroke 28 (7): 1418–22. PMID 9227694. http://stroke.ahajournals.org/cgi/pmidlookup?view=long&pmid=9227694. 
  7. ^ 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. http://radiographics.rsnajnls.org/cgi/pmidlookup?view=long&pmid=16973779. 
  8. ^ Scolding NJ, Joseph F, Kirby PA, et al. (March 2005). "Abeta-related angiitis: primary angiitis of the central nervous system associated with cerebral amyloid angiopathy". Brain 128 (Pt 3): 500–15. doi:10.1093/brain/awh379. PMID 15659428. http://brain.oxfordjournals.org/cgi/pmidlookup?view=long&pmid=15659428. 
  9. ^ Nagai A, Terashima M, Sheikh AM, et al. (2008). "Involvement of cystatin C in pathophysiology of CNS diseases". Front. Biosci. 13: 3470–9. doi:10.2741/2941. PMID 18508448. http://www.bioscience.org/2008/v13/af/2941/fulltext.htm. 
  10. ^ 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. 
  11. ^ http://www.dailymail.co.uk/news/article-1052696/Coroner-slams-Government-refusing-fund-probe-deaths-worst-water-poisoning-cases-British-history.html

[edit] External links

Cerebral Amyloid Angiopathy research group at the Massachusetts General Hospital in Boston




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