DEGENERATIVE DISEASES AFFECTING THE CEREBRAL CORTEX-without Tau Pathology

Question 1

Some cases with clinical and pathologic findings involving the frontal and temporal lobes lack
tau deposition; instead, usually_____________are found in
superficial cortical layers in temporal and frontal lobes and in the dentate gyrus (giving rise to
the term FTD-U for ubiquitin).

Some of these cases are familial and show linkage to
chromosome 17 but are caused by mutations in the gene for progranulin (an inflammatory
modulator protein), which isclose to the MAPT locus.

Similar pathology is seen
accompanying the cognitive impairment that sometimes occurs with amyotrophic lateral
sclerosis

Answer 1

** tau-negative**, **ubiquitin-containing inclusions **

Question 2

While some individuals with cognitive decline due to vasculitis show improvement with treatment,
there is also an irreversible and progressive cognitive disorder associated with vascular injury
to the brain.

Various etiologies include______________Additionally,
dementia has been associated with so-called strategic infarcts, which are usually embolic and
involve brain regions such as the hippocampus, dorsomedial thalamus, or the cingulate gyrus
of the frontal cortex. Many individuals, in fact, demonstrate a combination of pathologic

Answer 2

• widespread areas of infarction (abundant cortical
• microinfarcts, multiple lacunar infarcts, cortical laminar necrosis associated with reduced perfusion/oxygenation),
• and diffuse white-matter injury (hypertension, cerebral autosomal dominant arteriopathy with subcortical infarcts and leukoencephalopathy).
  *

Question 3

In vasculitis additionally, dementia has been associated with so-called__________, which are** usually embolic and
involve brain regionssuch as thehippocampus, dorsomedial thalamus,or thecingulate gyrus
of the frontal cortex.** Many individuals, in fact, demonstrate a combination of pathologic changes.

Answer 3

strategic infarcts

Question 4

There is also a relationship between vascular injury and other dementing disorders,
such as AD. It has been found that individuals with vascular changes above a certain threshold
have a lower burden of plaques and tangles for their level of cognitive impairment than do
those without vascular-based cerebral pathology