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Pathology of degenerative dementias 

Pathology of degenerative dementias
Chapter:
Pathology of degenerative dementias
Author(s):

Tamas Revesz

, Tammaryn Lashley

, and Janice L. Holton

DOI:
10.1093/med/9780199655946.003.0029
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date: 05 June 2020

Degenerative dementias represent a heterogeneous group of diseases with various aetiologies and pathological manifestations. A prominent feature of these conditions is the presence of proteinaceous intracellular inclusions and, in some, also extracellular protein deposits. The increasing knowledge about the genetic background of neurodegenerative diseases and the nature of inclusion-forming proteins has facilitated molecular classifications. The diseases, which are discussed, include those with extracellular protein aggregates and neurofibrillary degeneration. This group not only includes the most common degenerative dementia, Alzheimer’s disease, but it also comprises rare conditions as familial British dementia and familial Danish dementia. Frontotemporal lobar degenerations represent a large group of diseases, and are further subdivided on the basis of their specific proteinaceous inclusions with one of three proteins, forming cellular aggregates in the vast majority of the cases. Further diseases that are discussed include Lewy body disorders and also Huntington’s disease, which is the archetypical polyglutamine repeat disorder.

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