Cerebral amyloid angiopathy
Cerebral amyloid angiopathy | |
---|---|
Other names | Congophilic angiopathy[1] |
Micrograph of cerebral amyloid angiopathy using congo red stain | |
Specialty | Neurology |
Causes | Cause of CAA is unknown[2] |
Diagnostic method | PET scan, CT scan[2] |
Treatment | Management can be physical, occupational, or speech therapy.[2] |
Cerebral amyloid angiopathy (CAA) is a form of
Signs and symptoms
CAA is associated with brain hemorrhages, particularly microhemorrhages. The accumulation of amyloid beta peptide deposits in the blood vessel walls results in damage of the blood vessels and hindrance of normal blood flow, making blood vessels more prone to bleeding
Causes
CAA has been identified as occurring either sporadically (generally in elderly populations)
The reason for increased deposition of Aβ in sporadic CAA is still unclear with both increased production of the peptide and abnormal clearance having been proposed as potential causes.
In familial forms of CAA, the cause of Aβ build up is likely due to increased production rather than poor clearance.
Types
Several familial variants exist.[20] The condition is usually associated with amyloid beta.[21] However, there are types involving other amyloid peptides:
- the "Icelandic type" is associated with cystatin C amyloid (ACys).[22]
- the "British type" and "Danish type" are associated with British amyloid (ABri) and Danish amyloid (ADan) respectively. Both peptides are linked to mutations in ITM2B.[23]
- Familial amyloidosis-Finnish type is associated with gelsolin amyloid (AGel).[24]
Pathophysiology
The vascular amyloid pathology characteristic of CAA can be classified as either Type 1 or Type 2, the latter type being the more common. Type 1 CAA pathology entails detectable amyloid deposits within cortical capillaries as well as within the leptomeningeal and cortical arteries and arterioles. In type 2 CAA pathology, amyloid deposits are present in leptomeningeal and cortical arteries and arterioles, but not in capillaries. Deposits in veins or venules are possible in either type but are far less prevalent.[25]
Diagnosis
CAA can only be definitively diagnosed by a post-mortem autopsy.[27] Biopsies can play a role in diagnosing probable cases.[28] When no tissue is available for biopsy, the Boston Criteria are used to determine probable CAA cases from MRI or CT scan data. The Boston Criteria require evidence of multiple lobar or cortical hemorrhages to label a patient as probably having CAA.[27] Susceptibility weighted imaging has been proposed as a tool for identifying CAA-related microhemorrhages.[29]
Imaging
Cerebral amyloid angiopathy can be presented with
MRI sequence of
Management
The aim in cerebral amyloid angiopathy is to treat the symptoms, as there is no current cure. Physical, occupational and/or
History
Gustav Oppenheim was the first to report vascular amyloid β deposits on the vasculature of the central nervous system in 1909. The first paper focusing solely on what would come to be known as CAA was published in 1938 by WZ Scholz. In 1979, H. Okazaki published a paper implicating CAA in certain cases of lobar intracerebral hemorrhage.[24] The Boston Criteria for CAA originated in a 1995 paper from Harvard Medical School.[27]
References
- PMID 16627535.
- ^ a b c d e "Cerebral amyloid angiopathy: MedlinePlus Medical Encyclopedia". www.nlm.nih.gov. Retrieved 2015-05-27.
- ^ "UpToDate". www.uptodate.com. Retrieved 2019-08-17.
- ISBN 9780199710041.)
{{cite book}}
: CS1 maint: multiple names: authors list (link - ^ "Exploring cerebral amyloid angiopathy: Insights into pathogenesis, diagnosis, and treatment".
- ISBN 9781107310896.
- ISBN 9780080922188.
- ^ "Brain Basics: Preventing Stroke: National Institute of Neurological Disorders and Stroke (NINDS)". www.ninds.nih.gov. Retrieved 2015-05-27.
- PMID 31726962. Retrieved 2023-11-17.
- ISBN 9780781769471.
- ISBN 9781107014558.
- PMID 12163376.
- ISBN 9781461512493.
- ISBN 9780323227353.
- ISBN 9780444634948.
- ISBN 978-0702054662.
- ISBN 9781464900853.
- ISBN 9781464991721.
- ^ PMID 29604173.
- S2CID 8930792.
- ISBN 9781444341232.
- ISBN 9780521717922.
- ISBN 9780199644957.
- ^ PMID 21519520.
- PMID 11895043.
- PMID 19131406.
- ^ PMID 29335334.
- ISBN 9789401710077.
- ISBN 978-1437707014.
- PMID 28334869.
Further reading
- Chao, Christine P.; Kotsenas, Amy L.; Broderick, Daniel F. (September 1, 2006). "Cerebral Amyloid Angiopathy: CT and MR Imaging Findings". RadioGraphics. 26 (5): 1517–1531. PMID 16973779.