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Hereditary Cerebral Hemorrhage with Amyloidosis—Dutch Type: A Congophilic Angiopathy
Author(s) -
ROOS RAYMUND A. C.,
HAAN JOOST,
BROECKHOVEN CHRISTINE VAN
Publication year - 1991
Publication title -
annals of the new york academy of sciences
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.712
H-Index - 248
eISSN - 1749-6632
pISSN - 0077-8923
DOI - 10.1111/j.1749-6632.1991.tb00208.x
Subject(s) - cerebral amyloid angiopathy , amyloidosis , angiopathy , pathology , medicine , dementia , disease , amyloid (mycology) , point mutation , mutation , gene , endocrinology , biology , genetics , diabetes mellitus
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.