Congophilic angiopathies
Paweł P. Liberski
Affiliation and address for correspondenceAktualn Neurol 2005, 3 (5), p. 183-188
Abstract
Congophilic angiopathies (CAA) are a group of neurodegenerations caused by amyloid deposition in the vessel walls. Amyloids are molecularly different and, as in all amyloidoses, CAA may be hereditary caused by mutations in a precursor for a given amyloid or sporadic of unknown cause. “Amyloid” is a generic terms to define diverse proteins of common physicochemical properties. In particular, they are congophilic (stained with Congo red), they fluorescent following staining with thioflavin S and they are composed of fibrils which are visible under transmission electron microscope.
In this review we will cover:
- dementia of BRI family:
- familial British dementia, FBD (peptide ABri),
- familial Danish dementia, FDD (peptide ADan);
- hereditary cerebral haemorrhage with amyloidosis, Dutch type, HCHWA-D (peptide Ab);
- hereditary cerebral haemorrhage with amyloidosis, Icelandic type, HCHWA-I (cystatin C);
- CAA associated with Alzheimer disease (peptide Ab);
- familial Finnish amyloidosis (gelsolin).
Keywords
congophilic angiopathies, familial British dementia, familial Danish dementia, familial Finnish amyloidosis, amyloid
