95b - Alzheimer's and other Neurodegenerative Diseases Flashcards

1
Q

Which areas of the brain are usually spared in AD?

A

Sensory areas

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

At the cellular level, which protein is mutated in Alzheimer’s Disease?

What is the result?

A
  • Beta and/or gamma secretase don’t function properly
  • Instead of making normal cleavages, they cut at the wrong spot and produce amyloid beta40 and amyloid beta 42
  • ​These toxic structures result in microglial activation
  • -> Inflammation/glutamatergic toxicity
  • -> Phosphorylation of Tau proteins
  • -> Neurofibrillary tangles
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Which two classes of medications can be used to treat Alzheimer’s disease?

A
  • NMDA Receptor Inhibitors (memantine)
    • Blocks cytotoxicity caused by amyloid
  • Cholinesterase inhibitors
    • Increase ACh inthe brain
    • Slow progression; best if started early
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Which area/network of the brain is damaged in Alzheimer’s disease?

A

Limbic system

Involved in retentive memory

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

How do cholinesterase inhibitors affect patients with Dementia with Lewy Bodies vs. Alzheimer’s disease differently?

A

In Demetia with Lewy Bodies, cholinesterase inhibitors will make patients better

Vs. Alzheimer’s disease, they will slow progression

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

What is Benson’s syndrome?

How does it differ from typical Alzheimer’s disease?

A

Visual variant of Alzheimer’s Disease

  • Delcine in visual processing
  • Earlier onset than typical AD
  • Memory and language remain intact
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

What are the non-pharmocological interventions for frontotemporla dementia?

A

PT, OT, speech therapy, reward-based therapy

(Main pharmacoogical is SSRI)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Before cognitive impairment, what symptoms of Lewy Body Dementia might be present?

A
  • Constipation
  • Violent dream eneactment
  • Occasional dizziness
    • Followed by slowing of movements, impaired balance
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Describe the “typical” presenting symptoms/complaints associated with Alzheimer’s Disease

A
  • Older patient
  • Frequent forgetfulness
    • Conversations, events sometimes
    • Repetitive in conversations
  • Trouble with word finding
  • Results in difficulty with some functions
    • Finances
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What preventative measures can be taken if a person is at risk of Alzheimer’s Disease?

A
  • Weight control
  • Exercise
  • Mental and social engagement
  • Control:
    • Blood pressure
    • Blood sugar
    • Cholestoral
    • Vitamin B12
  • Normalize sleep
  • Mediterranean diet
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Which genes are associated with Alzheimer’s Disease?

A
  • Deterministic genes
    • APP
    • Presenilin 1
    • Presenilin 2
  • Risk genes
    • APO E4

But <1% of AD is thought to be genetic

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

What is the main pharmacological intervention for frontotemporal dementia?

A

SSRIs

(Non-pharmacological include PT, OT, speech therapy, reward-based therapy)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Frontotemporal lobar degeneration is an umbrella term that encompasses 3 types of dementias

What are they?

A
  • Language type: primary progressive aphasia
  • Behavioral type: Behavioral type frontotemporal dementia(akaPick’s Disease)
  • Motor type: Corticobasal syndrome, Progressive supranuclear palsy, or FTD-MND (Associated with ALS)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

What CSF findings will be present in a patient with Alzheimer’s?

A

Decreased amyloid in the CSF

  • It’s busy messing with the brain :(
  • Amyloid:Tau index <1
  • P-tau >60
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

What are the three motor types of frontotemporal dementia?

How do you differentiate them?

A

PSP and CBD will both have problems with balance, vision, speech, swallowing, and memory

  • Progressive supranuclear palsy (PSP)
    • Cannot look down voluntarily
  • Corticobasal degeneration (CBD)
    • Asymmetric (compared with PSP)
    • Behavior deficits
  • FTD-Motor Neuron Disease (FTD-MND)
    • Frontotemporal dementia + ALS
    • Muscle weakness, shrinkage, jerking
    • Fewer memory deficits, more language deficits compared with PSP, CBD
    • Behavior deficits (like CBD)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Stepwise impairments in cognition usually have what etiology?

A

Lacunar strokes

-> vascular cognitive impairment

17
Q

Describe the symptoms of Pick’s Disease

A

Pick’s disease = behavioral variant of frontotemporal dementia

Characterized by progressive deterioration of behavior and cognition

  • Behavioral disinhibition
  • Apathy or inertia
  • Loss of sympathy or empathy
  • Preservative, stereotyped, or compulsive/ritualistic behavior
  • Hyperorality, dietary changes
  • Memory and visuospatial reasoning are spared
    • vs. Alzheimers has loss of memory
    • vs. Dementia with Lewy Bodeis has visuospatial impairment
18
Q

Which neurodegeneratie disease is associated with amyloid plaques and neurofibrillary tangles?

A

Alzheimer’s Disease

19
Q

Which genes contribute to frontotemporal dementia?

A
  • MAPT
    • -> FTLD-Tau
  • PGRN
    • -> FTLD-TDP (Type A = language type)
  • C9ORF72
    • -> FTLD-TDP (Type B = behavioral type)

Not 100% sure about the types - pls DM if you can shed some light!

20
Q

In Alzheimer’s Disease, which major brain areas are affected by the abnormal tau and amyloid proteins?

A

Nucleus basalis of Meynert

  • ACh is produced here
  • Tau and amyloid deposits -> damage
  • -> Whole brain is hypocholinergic, especially the limbic system
    • Typical Alzheimer’s Disease
21
Q

How will Dementia with Lewy Bodies appear on imaging?

A

Hypometabolism in the occipital lobe

(Vs decreased metabolism in the cingulate cortex in Alzheimer’s)

22
Q

How is working memory affected by Alzheimer’s Disease?

How will you know on exam?

A

Working memory is usually intact

If you tell the patient 4 words, they will be able to repeat them back to you

(But will forget them when distracted due to limbic destruction)

23
Q

What pathologenic changes are associated with Alzheimer’s Disease?

A

Amyloid plaques

Neurofibrillary Tangles (NFTs)