An overview of the neuropathology of neurodegenerative diseases

Published on July 1, 2014   43 min

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Other Talks in the Series: The Genetic Basis of Neurological Disorders

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My name is Tammaryn Lashley. I'm a research fellow at Queen Square Brain Bank at the institute of neurology at ULC. I'll be giving an overview of the neuropathology found in neurodegenerative diseases.
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The nuerodegenerative diseases that I will be discussing are Alzheimer's disease, dementia with Lewy bodies, frontal temporal dementias, and the familial British and Danish dementias.
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For each of these neurodegenerative diseases, I will discuss the macroscopic features that are characteristic to the diseases, the major pathological proteins that are involved in those diseases, the microscopic pathological lesions present, and how we diagnose the different diseases from the pathological features, and also discuss the importance of the clinical pathological studies that will help in earlier and correct diagnosis of future cases.
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Alzheimer's disease is the most common form of dementia, with dementia being defined as a clinical decline in daily functioning due to cognitive impairment. The prevalence of AD increases with age. And from epidemiological studies in 65 to 69-year-olds, the prevalence of dementia is approximately one in a hundred, and for every subsequent five years, the prevalence doubles. There are several risk factors associated with dementia. By far, the most important risk factor is age, followed by family history of dementia, hypertension, and diabetes. Nueropathologically, brains reveal a global brain atrophy, abundant amounts of amyloid plaques composed of Aβ and neurofibrillary tangles composed of tau. Inherited forms of AD account for less than 5% of the total number of cases, and early onset AD is caused by mutations in the amyloid precursor protein. Over 30 different pathological mutations have been identified, and mutations in the Presenilin-1 and -2 proteins. The majority of mutations are found in PS-1, while only 14 mutations have been found in the PS-2 gene. The genetics of late onset AD are considerably more challenging than early onset AD, and variations in the apolipoprotein E and TREM2 gene have been shown to increase the risk of developing late onset Alzheimer's disease.
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An overview of the neuropathology of neurodegenerative diseases

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