Familial cerebral amyloid angiopathy related to stroke and dementia.
The term cerebral amyloid angiopathy (CAA) refers to the specific deposition of amyloid fibrils in the walls of leptomeningeal and cortical arteries, arterioles and, although less frequently in capillaries and veins. It is commonly associated with Alzheimers disease, Down's syndrome and normal aging, as well as with a variety of familial conditions related to stroke and/or dementia: hereditary cerebral hemorrhage with amyloidosis of Icelandic type (HCHWA-I), various inherited disorders linked to Abeta mutants (including the Dutch variant of HCHWA), and the recently described chromosome 13 familial dementia in British and Danish kindreds. This review focuses on four different types of hereditary CAA, emphasizing the notion that CAA is not only related to stroke but also to neurodegeneration and dementia of the Alzheimer's type.
Frangione
B
B
Department of Pathology, New York University School of Medicine, New York 10016, USA. frangb01@popmail.med.nyu.edu
Révész
T
T
Vidal
R
R
Holton
J
J
Lashley
T
T
Houlden
H
H
Wood
N
N
Rostagno
A
A
Plant
G
G
Ghiso
J
J
D000544:Alzheimer Disease / Q000209:etiology; D016229:Amyloid beta-Peptides / Q000235:genetics; D028243:Cerebral Amyloid Angiopathy, Familial / Q000209:etiology* / Q000235:genetics / Q000473:pathology; D055316:Cystatin C; D015891:Cystatins / Q000235:genetics; D003704:Dementia / Q000209:etiology*; D003718:Denmark; D005799:Genes, Dominant; D006801:Humans; D009154:Mutation; D020521:Stroke / Q000209:etiology*; D006113:United Kingdom
D016229:Amyloid beta-Peptides; C525030:CST3 protein, human; D055316:Cystatin C; D015891:Cystatins