Lecture 87 - Neuropathology of Dementia

Question 1

At autopsy, AD patients’ brains will be < ______gm due to severe _______ and Global atrophy.

Answer 1

< 1000gm

Severe hippocampal and Global atrophy

Question 2

One of the earliest regions to be affected in AD is the ______ region of the Hippocampus.

Answer 2

CA1

Question 3

Keep in mind betaAmyloid plaques are ________ (intra or extracellular?), while Neurofibrillary tangles comprised of tau proteins are ________ (intra or extracellular?).

BetaAmyloid is Congophilic and shows ______-_____ (color) birefringence under polarized light.

Answer 3

Intracellular

Extracellular

Apple-green

Question 4

Tau proteins normally act to stabilize ________, but begin to accumulate and become insoluble when they are ________.

Answer 4

Microtubles

Hyperphosphorylated

Question 5

One of the classical presentations of brain atrophy in Pick’s disease is the sparing of the _______ 2/3 of the Temporal lobe. This helps distinguish it on gross pathology from which other dementia-type disease?

Remember that Pick bodies are Intraneuronal tau accumulations. This differs from AD in that AD tau accumulations (fibrillary tangles) are Extraneuronal (outside the neurons).

Answer 5

Posterior 2/3

This helps distinguish it from Frontal Temporal

Question 6

Frontotemporal Lobar degeneration TDP-43 is similar on gross pathology to Pick’s disease, but it shows even distribution of atrophy in the _______ lobe. On histology, look for TDP-43 in the ________ (normally it should be in the _______) of cells.

Answer 6

Temporal lobe

Cytoplasm

Nucleus

Question 7

In Corticobasal Degeneration, look for ______ neurons (expanded cytoplasm) on histology and Tau inclusions in both neurons AND ______ cells.

Answer 7

“Baloon” neurons

Question 8

Progressive Supranuclear Palsy (PSP) is similar to Corticobasal Degeneration in that both diseases show Neuronal and Glial cell accumulation of Tau proteins.

What’s significant about the Tau accumulations in PSP?

Look for PSP patients to present with ______ vision due to their inability to aim and move their eyes properly.

Answer 8

The tau accumulations in PSP are 4-repeats.

Blurred vision

Question 9

Transmissable Spongiform Encephalopathy (TSE) is caused by the misfolding of an endogenous coded for by the PrP gene on Chromosome _____. The misfolded form is termed _____ or _____.

Answer 9

Chromosome 20

PrPsc (scrapie)

PrPres (protease resistant)