11.28 Neurodegenerative Disorders/Lab

Question 1

what protein is spliced differently in Alzheimer’s to make the defective amyloid protein?

Answer 1

amyloid precursor protein (APP)

Question 2

what is Parkinson’s caused by?

Answer 2

degeneration of dopaminergic neurons in the substantial nigra

Question 3

Infectious prion protein in CJD differs from normal by a _____________ change (_______ ____ to a ______ _______).

Answer 3

conformational

alpha helix, beta sheet

Question 4

what is this histologically? disease?

Answer 4

neurofibrillary tangle

Alzheimer’s

Question 5

what is this depicting? what disease?

Answer 5

spongiform degeneration with amyloid plaques

the new strian of CJD

Question 6

what are the two proteins (not structures they make) that are accumulated in Alzheimer’s?

Answer 6

tau amyloid-beta peptide

Question 7

what are the three forms (ways) of Creutzfeldt-Jacob Disease?

Answer 7

Inherited Spontaneous Transmissible

Question 8

what is the most major feature in the pathogenesis of neurodegenerative disease?

Answer 8

accumulation of misfolded or aggregation-prone proteins

Question 9

what do Lewy bodies contain?

Answer 9

alpha-synuclein

Question 10

what are the two structures that can be seen in histology to make the diagnosis of Alzheimer’s?

Answer 10

neurofibrillary tangles neurotic plaques

Question 11

what does Creutzfeldt-Jacob Disease present as?

Answer 11

rapidly progressing dementia with myoclonus and death an approx 7 months

Question 12

what aggregated protein is seen in Parkinson’s

Answer 12

alpha-synuclein

Question 13

spongiform encephalopathy is seen in _____ diseases

Answer 13

prion

Question 14

what can you see in a spinal cord cut in ALS histology?

Answer 14

degeneration of corticospinal tract loss of LMN in ventral horn

Question 15

subacute fatal disease of cerebellar degeneration with ataxia and dementia following ritualistic cannibalism

Answer 15

kuru

Question 16

what is this histologically? what is it an aggregate of? disease?

Answer 16

Lewy body

alpha-synuclein

Parkinson’s

Question 17

when Lewy bodies are seen in the cerebral cortex this is called:

Answer 17

Dementia with Lewy bodies

Question 18

what is this histologically? disease?

Answer 18

neurotic plaques (amyloid beta core and taue containing processes on outside)

Alzheimer’s

Question 19

what is the most common form of spongiform encephalopathy in humans?

Answer 19

Creutzfeldt-Jacob Disease

Question 20

progressive neurodegenerative disease characterized by loss of UMNs and LMNs.

Answer 20

ALS (amyotrophic lateral sclerosis)

Question 21

what is wrong with the prion in Creutzfeldt-Jacob Disease? what can it do to the host?

Answer 21

it has an abnormal conformational change it can cause a conformational change in endogenous prion molecules of the host

Question 22

what are the inclusions in cells histologically of ALS?

Answer 22

TDP-43 inclusions in remaining motor neurons

Question 23

what do neurotic plaques consist of? in what neurodegenerative disease are they found?

Answer 23

abnormal neurites (processes with tau) that surround a core of amyloid

Question 24

neurofibrillary tangles are seen in _________. What abnormal aggregate of protein is inside them? Where?

Answer 24

Alzheimer’s tau cytoplasm

Question 25

what is this histologically? what is it an aggregate of? disease?

Answer 25

Lewy body

alpha-synuclein

Parkinson’s

Question 26

what is this depicting? what is the disease?

Answer 26

ALS
it is showing demyelination of the corticospinal tract. with loss of axons, there is loss of myelination

Question 27

what is the hallmark of Parkinson’s on histology?

Answer 27

the Lewy body (eosinophilic inclusion found in cytoplasm of remaining pigmented cells of the substantial nigra)

Question 28

what is this depicting? what is it caused by? what disease?

Answer 28

spongiform degeneration

an abnormal conformation of prion protein

Creutzfeldt-Jakob disease

Question 29

prion diseases are characterized by what morphologically in tissue?

Answer 29

spongiform degeneration and gliosis