Neurodegenerative Pathology

Question 1

Mutations in three key genes, ____________ predispose to early-onset Alzheimer’s disease.

Answer 1

presenilin-1, presenilin-2, and APP,

Question 2

The possession of an _______allele is a risk factor for late-onset Alzheimer’s disease

Answer 2

apolipoprotein ε4

Question 3

what are Neurofibrillary Tangles?

Answer 3

Bundles of paired helical filaments in the cytoplasm of neurons that displace or encircle the nucleus.
as seen in Alzheimers disease

Question 4

Filaments of Neurofibrillary Tangles are primarily composed of hyperphosphorylated forms of the protein _______.

Answer 4

tau

Question 5

what are the NIA-AA criteria for the neuropathologic assessment of AD

Answer 5

“ABC” score to describe AD neuropathologic changes:
A - Thal phase of the extent ofAmyloid deposition
B - Braak and Braak neurofibrillary stage
C - Neuritic plaque density score of CERAD

Question 6

what are the clinical classifications of Frontotemporal Lobar Degeneration

Answer 6

Behavioral variant (60%)
Primary Progressive Aphasia (40%)
-Progressive non-fluent aphasia
-Semantic dementia

Question 7

_______ is a major constituent of ubiquitin positive inclusions in both FTLD and ALS

Answer 7

TDP-43

Question 8

TDP-43 inclusions are usually associated with mutations in ______, a growth factor secreted in response to injury and/or inflammation.

Answer 8

progranulin

Question 9

what is The second most common dementing disorder in late life

Answer 9

Dementia with Lewy bodies (DLB)

Question 10

what gross brain findings are seen in dementia with lewy bodies.

Answer 10

There is typically significant atrophy involving the limbic system

Question 11

Bunina bodies are small eosinophilic inclusions, 2–5µm in diameter. They are often arranged in small beaded chains and are sparse in most ______

Answer 11

ALS cases.

Question 12

what is the pathology of huntington disease?

Answer 12

Progressive degeneration of the striatum (caudate and putamen) and frontal cortex with neuronal loss and gliosis

Question 13

what is the Pathology of Parkinson disease

Answer 13

Depigmentation of the substantia nigra and locus ceruleus

Question 14

Describe plaques in AD.

Answer 14

extracellular, roughly spherical, focal aggregates of amyloid material, 60–120μm in diameter, that are often separated into dense core and peripheral halo regions

Question 15

Neurofibrillary tangles contain ________

Neuritic plaques contain _____

Answer 15

tau protein,

ß-amyloid