Alzheimer's disease and CADASIL are heritable, adult-onset dementias that both involve damaged small blood vessels.

[cadasil]

This essay explores an alternative pathway to Alzheimer 's dementia that focuses on damage to small blood vessels rather than late -stage toxic amyloid deposits as the primary pathogenic mechanism that leads to irreversible dementia . While the end-stage pathology of AD is well known , the pathogenic processes that lead to disease are often assumed to be due to toxic amyloid peptides that act on neurons , leading to neuronal dysfunction and eventually neuronal cell death . Speculations as to what initiates the pathogenic cascade have included toxic abeta peptide aggregates , oxidative damage , and inflammation , but none explain why neurons die . Recent high -resolution NMR studies of living patients show that lesions in white matter regions of the brain precede the appearance of amyloid deposits and are correlated with damaged small blood vessels . To appreciate the pathogenic potential of damaged small blood vessels in the brain , it is useful to consider the clinical course and the pathogenesis of CADASIL , a heritable arteriopathy that leads to damaged small blood vessels and irreversible dementia . CADASIL is strikingly similar to early onset AD in that it is caused by germ line mutations in NOTCH 3 that generate toxic protein aggregates similar to those attributed to mutant forms of the amyloid precursor protein and presenilin genes . Since NOTCH 3 mutants clearly damage small blood vessels of white matter regions of the brain that lead to dementia , we speculate that both forms of dementia may have a similar pathogenesis , which is to cause ischemic damage by blocking blood flow or by impeding the removal of toxic protein aggregates by retrograde vascular clearance mechanisms .

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