Hereditary cerebral hemorrhage with amyloidosis--Dutch type: a congophilic angiopathy. An overview.
Hereditary cerebral hemorrhage with amyloidosis--Dutch type (HCHWA-D) is characterized by recurrent cerebral hemorrhages and dementia at a relatively young age. The symptoms are caused by extensive deposition of amyloid in cerebral arterioles and leptomeningeal arteries. A point-mutation in the beta-protein precursor gene on chromosome 21 is the underlying cause of the disease. This paper summarizes the clinical, radiologic, pathologic, and genetic features of this disease, with special attention to the relation between HCHWA-D and Alzheimer's disease, which is also characterized by beta-protein deposition.
Roos
R A
RA
Department of Neurology, University Hospital, Leiden, The Netherlands.
Haan
J
J
Van Broeckhoven
C
C
D016229:Amyloid beta-Peptides / Q000378:metabolism; D000686:Amyloidosis / Q000150:complications / Q000000981:diagnostic imaging / Q000235:genetics / Q000473:pathology*; D016657:Cerebral Amyloid Angiopathy / Q000150:complications / Q000000981:diagnostic imaging / Q000235:genetics / Q000473:pathology*; D002543:Cerebral Hemorrhage / Q000150:complications / Q000000981:diagnostic imaging / Q000235:genetics / Q000473:pathology*; D005260:Female; D006801:Humans; D008297:Male; D011859:Radiography
D016229:Amyloid beta-Peptides