Manifestations of cortical vessel involvement: Other familial syndromes (as discussed above) ~40% of those with cerebral amyloid angiopathy have Alzheimer dementia type symptoms Pathological cerebral amyloid angiopathy changes are seen in ~80% of those with Alzheimer disease (Aß-42) 5-13 It is thought to clinically manifest approximately three decades after transmission has occurred 30. Iatrogenic cerebral amyloid angiopathy is an extremely rare but increasingly recognized form, considering the disease to be a prionopathy attributed to transmission of Aβ seeds through surgical procedures, such as exposure to cadaveric dura (e.g. PrPSc peptide with precursor prion protein (chromosome 20): Gerstmann-Straussler-Scheinker diseaseĪBri peptide with precursor protein ABri precursor protein (chromosome 13): familial British dementia (see case 17)ĪDan peptide with precursor protein ADan precursor protein (chromosome 13): familial Danish dementia Hereditary cerebral hemorrhage with amyloidosis (Dutch, Italian, Flemish, Iowa, Piedmont, Arctic types)ĪCys peptide with precursor protein cystatin C (chromosome 20): hereditary cerebral hemorrhage with amyloidosis Icelandic typeĪTTR peptide with precursor protein transthyretin (chromosome 18): meningovascular amyloidosis (see cerebral transthyretin-associated amyloidoses)ĪGel peptide with precursor protein gelsolin (chromosome 9): familial amyloidosis - Finnish type Furthermore, they may also be part of multi-system or other central nervous system genetic disorders 14,21.Īß peptide with precursor protein APP (chromosome 21):ĬAA related to familial Alzheimer disease Many of these disorders are only isolated to only a few families and they mainly differ from spontaneous CAA in an earlier age of onset, typically in middle to late middle age 14,21. Familial CAAįamilial cerebral amyloid angiopathy describes a group of very rare disorders that are usually encountered as autosomal dominant conditions 14,21. Importantly, it is usually not associated with systemic amyloidoses. ![]() Sporadic CAAĬerebral amyloid angiopathy is a frequent incidental finding, found on screening gradient-recalled echo imaging in up to 16% of asymptomatic elderly patients 4. Autopsy studies have found a prevalence of approximately 5-9% in patients between 60 and 69 years, and 43-58% in patients over the age of 90 4.Īutopsies of patients who have evidence of Alzheimer disease have found cerebral amyloid angiopathy in the vast majority of cases (90%). This rate is still high (20-40%) in non-demented elderly individuals 14. Cerebral amyloid angiopathy can be divided into sporadic (spontaneous), familial, and iatrogenic forms.
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