The Cerebral β-Amyloid Angiopathies: Hereditary and Sporadic
Top Cited Papers
- 1 January 2006
- journal article
- review article
- Published by Wiley in Brain Pathology
- Vol. 16 (1) , 30-39
- https://doi.org/10.1111/j.1750-3639.2006.tb00559.x
Abstract
We review the clinical, radiologic, and neuropathologic features of the hereditary and sporadic forms of cerebral amyloid angiopathy (CAA) associated with vascular deposition of the beta-amyloid peptide. Amino acid substitutions at 4 sites in the beta-amyloid precursor protein, all situated within the beta-amyloid peptide sequence itself, have been shown to cause heritable forms of CAA. The vascular diseases caused by these mutations are associated primarily with cerebral hemorrhages, white matter lesions, and cognitive impairment, and only variable extents of the plaque and neurofibrillary pathologies characteristic of Alzheimer disease. Sporadic CAA typically presents 20 or more years later than hereditary CAA, but is otherwise characterized by a comparable constellation of recurrent cerebral hemorrhages, white matter lesions, and cognitive impairment. The clinical, radiologic and pathologic similarities between hereditary and sporadic CAA suggest that important lessons for this common age-related process can be learned from the mechanisms by which mutation makes beta-amyloid tropic or toxic to vessels.Keywords
This publication has 98 references indexed in Scilit:
- Hemorrhage is uncommon in new Alzheimer family with Flemish amyloid precursor protein mutationNeurology, 2004
- Hereditary cerebral hemorrhage with amyloidosis dutch type (AβPP 693): decreased plasma amyloid-β 42 concentrationNeurobiology of Disease, 2003
- Pathological correlates of late-onset dementia in a multicentre, community-based population in England and WalesThe Lancet, 2001
- Hereditary cerebral hemorrhage with amyloidosis-Dutch type (HCHWA-D): A review of the variety in phenotypic expressionAmyloid, 1999
- Presenile Alzheimer dementia characterized by amyloid angiopathy and large amyloid core type senile plaques in the APP 692Ala→Gly mutationActa Neuropathologica, 1998
- Progression of Cerebral Amyloid Angiopathy: Accumulation of Amyloid-ß40 in Affected VesselsJournal of Neuropathology and Experimental Neurology, 1998
- Dutch hereditary cerebral amyloid angiopathy: Structural lesions and apolipoprotein E genotypeAnnals of Neurology, 1997
- The length of Amyloid-β in Hereditary Cerebral Hemorrhage with Amyloidosis, Dutch TypePublished by Elsevier ,1996
- White Matter Lesions and Cognitive Deterioration in Presymptomatic Carriers of the Amyloid Precursor Protein Gene Codon 693 MutationArchives of Neurology, 1996
- Hereditary Cerebral Hemorrhage with Amyloidosis- DutchType: Better Correlation of Cognitive Deterioration with Advancing Age than with Number of Focal Lesions or White Matter HyperintensitiesAlzheimer Disease & Associated Disorders, 1996