Asymmetrical Cortical Degeneration Syndromes

This is a heterogeneous group of disorders that produce distinctive cortical syndromes including aphasia, apraxia, and agnosia but that have a more focal appearance than Alzheimer's dementia. They can be roughly localized by the more focal features, though only a small subset are truly focal. There are four primary categories, each with several subtypes, including progressive aphasia, progressive frontal lobe/frontotemporal syndromes, progressive perceptual-motor syndromes, and progressive bitemporal syndromes (i.Tab!e..,.3.3:2. ).

Pathogenesis and Pathophysiology. Despite the semiological and pathological diversity of the ACDS group, there is a common clinicopathological theme among all members: clinical presentation is dictated by the topographical distribution of degeneration. Nonspecific degenerative changes, consisting of neuronal loss, gliosis, and vacuolation of neuropil predominantly affecting superficial cortical laminae ( . Fig, 3.3.-5. ), are common to all clinical subtypes and probably account for the overall majority of pathological findings in ACDS.

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