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Cerebral amyloid angiopathy (CAA) is a form of angiopathy in which amyloid beta peptide deposits in the walls of small to medium blood vessels of the central nervous system and meninges. [ 2 ] [ 3 ] The term congophilic is sometimes used because the presence of the abnormal aggregations of amyloid can be demonstrated by microscopic examination ...
Hereditary cystatin C amyloid angiopathy (HCCAA) is a rare, fatal type of hereditary cerebral amyloid angiopathy found almost exclusively in Iceland.A mutation in the protein cystatin C leads to amyloid (protein aggregate) dispositions in arteries in the brain, resulting in repeated brain hemorrhages.
The cause of Cerebral Amyloid Angiopathy are unknown. Sometimes it can be hereditary, however, there have been cases where it is developed in the elderly, especially in people over 55 years of age. [3] High blood sugar is the primary cause of diabetic angiopathy.
FDD patients also have extensive brain atrophy, particularly in the cerebellum, cerebral cortex, and white matter, as well as very thin and virtually demyelinated cranial nerves. Cerebral amyloid angiopathy in this disorder is severe. [8] CAA is present in all regions of the central nervous system. [9]
Familial amyloid polyneuropathy, also called transthyretin-related hereditary amyloidosis, transthyretin amyloidosis abbreviated also as ATTR (hereditary form), or Corino de Andrade's disease, [1] is an autosomal dominant [2] neurodegenerative disease.
Mutations in the cystatin 3 gene are responsible for the Icelandic type of hereditary cerebral amyloid angiopathy, a condition predisposing to intracerebral haemorrhage, stroke and dementia. [ 57 ] [ 58 ] The condition is inherited in a dominant fashion .
However, Aβ plaques (along with cerebral Aβ-amyloid angiopathy) can be detected in the brains of living subjects by preparing radiolabeled agents that bind selectively to Aβ deposits in the brain after being infused into the blood. [41]
To date, 37 human proteins have been found to form amyloid in pathology and be associated with well-defined diseases. [2] The International Society of Amyloidosis classifies amyloid fibrils and their associated diseases based upon associated proteins (for example ATTR is the group of diseases and associated fibrils formed by TTR). [3]
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