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Definition

A hereditary localized amyloidosis due to amyloid-beta A4 peptide(s) deposition in the cerebral vessels. The principal clinical characteristics are recurrent cerebral and cerebellar hemorrhages, recurrent strokes, cerebral ischemia, cerebral infarction, and progressive mental deterioration. Patients develop cerebral hemorrhage because of the severe cerebral amyloid angiopathy. Parenchymal amyloid deposits are rare and largely in the form of pre-amyloid lesions or diffuse plaque-like structures. They are Congo red negative and lack the dense amyloid cores commonly present in Alzheimer disease. Some affected individuals manifest progressive aphasic dementia, leukoencephalopathy, and occipital calcifications.

Acronym

CAA-APP

Synonyms

Amyloidosis cerebroarterial APP-related
Amyloidosis hereditary with cerebral hemorrhage Dutch variant
Cerebral amyloid angiopathy APP-related Arctic variant
Cerebral amyloid angiopathy APP-related Dutch variant
Cerebral amyloid angiopathy APP-related Flemish variant
Cerebral amyloid angiopathy APP-related Iowa variant
Cerebral amyloid angiopathy APP-related Italian variant
Familial occipital calcifications with hemorrhagic strokes leukoencephalopathy arterial dysplasia dementia
FOCHS-LADD
HCHWA-D
HCHWAD
Hereditary cerebral amyloid angiopathy Dutch type
Hereditary cerebral hemorrhage with amyloidosis Dutch type
Hereditary cerebral hemorrhage with amyloidosis Italian type

Keywords

Amyloidosis