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Brain and Behavior > 6.4.2 Neurodegenerative Diseases > Flashcards

6.4.2 Neurodegenerative Diseases Flashcards

1
Q

What is the white arrow pointing at?

A

Lewy body in substania nigra

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2
Q

What is the main type of deposit in FTLDs?

A

Tau deposits

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3
Q

Prior to death, this patient demonstrated signs of dementia. What is the most likely cause of this patient’s dementia?

A

DLB (not Alz Dz because there is no cortical atrophy)

DLB often has a normal brain weight with little to no atrophy

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4
Q

What is the most common genetic cause of ALS?

A

C9orf72- intronic hexanucleotide expansion (open frame mutation)

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5
Q

What is the most common neurodegenerative dz behind Alz Dz?

A

Dementia with Lewy bodies (DLB)

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6
Q

What are the two primary protein aggregates in AD?

A

Beta-amyloid plaques (extracellular)

Phosphorylated tau tangles and threads (intracellular)

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7
Q

What are major degenerative disorders that cause adult dementia?

A

Alzheimer disease, Dementia with Lewy bodies, Frontotemporal lobar dementia (Pick dz)

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8
Q
A
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9
Q

What are the main clinical features of a patient with Parkinson dz?

A

resting tremor, bradykinesia (or akinesia), rigidity that may be initially unilateral but progressees to involve both sides

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10
Q

Which lobes of the brain are primarily affected by AD? Which lobe of the brain is typically preserved?

A

Atrophy prominent in temporal pole, medial temporal lobe (amygdala and hippocampus), frontal and partietal lobes

Preserved: Occipital lobe

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11
Q

What type of proteinopathy are these conditions?

A
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12
Q

What is the common thread between protein aggregates that lead to disease?

A

The native protein structure is lost, and fibrils form and accumulate

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13
Q

What are some genes that have been linked to Alz Dz?

A

APP, PS1, PS2, ApoE4

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14
Q

What are some of the genetic loci that have been identified in AD or AR inherited Parkinson dz?

A

LRRK2, gene for alpha-synuclein

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15
Q

Name this condition. What is the importance of fluorescence on Thioflavin S staining?

A

AD; Fluorescence confirms beta-pleated sheet conformation of protein aggregate

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16
Q

What is the black arrow pointing at? What protein comprises that aggregate?

A

Lewy body; alpha-synuclein

In DLB: Lewy bodies accumulate in brain stem, pons, limbic areas, neocortex

17
Q

Which protein aggregate found in AD correlates better with dementia?

A

Tau “tangles” (ptau)

18
Q

Diagnose him

A

Parkinson Dz

19
Q

What intracellular inclusion is being stained in these slides?

A

Tau neurofibrillary tangles

20
Q

Name this condition

A

Pick dz (FTLD-tau)

21
Q

What is the neuropathology and the affected areas of ALS?

A
22
Q

How can Parkinson Dz be distinguished from other extrapyramidal movement disorders at autopsy?

A

Presence of lewy bodies in the surviving neurons of the brainstem

23
Q

What disease? What is shown in these images?

A

Parkinson dz

1st 2 images: substantia nigra compacta cell loss, loss of striatal dopamine content

3rd image: Lewy body

24
Q

What are the main clinical features of frontal temporal lobar degeneration (FTLD)?

A

Progressive language deterioration and personality changes

25
Q

Describe these conditions [ALS, Alz dz, Parkinson dz, dementia with lewy bodies (DLB), FTLD (pick dz)] as either cerebral cortical disorders or motor pathway disorders.

A
26
Q
A

Pick bodies and Pick cells

Severe neuronal loss in outer cortical layers

Pick cells are swollen pale neurons (ballooned neurons)

27
Q

Name two alpha-synucleinopathies.

A

Dementia with lewy bodies (dementia

Parkinson Dz (movement disorder)

28
Q

What are the clinical features of DLB?

A

Progressive dementia with flucuations in coginition and arrousal

REM sleep behavior disorder

Visual halLEWYnations

Variable mild parkinsonism

29
Q
A
30
Q

What pathologic feature of AD are the red arrows pointing at? Why does this occur?

A

Hydrocephalus ex vacuo: occurs in response to atrophy of the cerebral lobes, not as a result of excessive CSF production

31
Q

Name the type of dz

A

FTLD

32
Q

What type of protein is tau>

A

Axonal microtubule-associated protein (MAP)

33
Q

Name this condition

A

Alzheimer Disease

34
Q

What type of aggregates can be found in motor neurons of ALS patients?

A

TDP-43

35
Q

What gene is associated with Alzheimer disease because of its role in processing of amyloid precursor proteins? Chromosome?

A

APP gene on chromosome 21

36
Q

What neurotransmitter is decreased in the cortex of a patient with AD?

A

Ach

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