Neuro19

Question 1

What is the most common cause of dementia in elderly?

Answer 1

Alzheimer’s disease

Question 2

What patients have an increased risk of developing Alzheimer’s?

Answer 2

Down Syndrome pts

Question 3

In the familial form of Alzheimer’s (10%), which proteins are altered?

• Early onset
• Late onset

Answer 3

Early onset:
 - APP (chr21)
 - Presenilin-1 (chr14)
 - Presenilin-2 (chr1)
Late onset:
 - ApoE4 (chr19)

Question 4

Why do Down Syndrome patients have an increased risk of developing early onset Alzheimer’s?

Answer 4

APP gene is on chromosome 21

*downs = trisomy 21

Question 5

What protein has been found to be protective against developing Alzheimer’s?

Answer 5

ApoE2 (chr19)

Question 6

What are the gross findings of a brain with Alzheimer’s?

Answer 6

Widespread Cortical Atrophy

Question 7

A decrease in what neurotransmitter is associated with Alzheimer’s?

Answer 7

ACh

Question 8

What are the histologic findings of Alzheimer’s?

Answer 8

Senile plaques

Neurofibrillary tangles

Question 9

What are Senile plaques?

Answer 9

Extracellular ß-amyloid core

*Aß (amyloid-ß) is synthesized by cleaving amyloid precursor proteins

Question 10

What are Neurofibrillary tangles?

Answer 10

Intracellular, abnormally phosphorylated tau protein = Insoluble cytoskeletal elements

Question 11

T/F - Neurofibrillary tangles correlate with degree of dementia.

Answer 11

True

Question 12

What are Pick bodies?

Answer 12

Spherical tau protein aggregates

Question 13

What is the gross characteristic change seen in Pick’s disease?

Answer 13

Frontotemporal atrophy

• Spares parietal lobe & posterior 2/3 of superior temporal gyrus
• Picks disease = Frontotemporal dementia

Question 14

What are the signs and symptoms of Pick’s disease?

Answer 14

Dementia
Aphasia
Parkinsonian aspects
Change in Personality

Question 15

What is Lewy Body dementia?

Answer 15

Parkinsonism w/ Dementia & Hallucinations

*EARLY ON-SET

Question 16

What is the defect in Lewy Body dementia?

Answer 16

alpha-synuclein defect

Question 17

What is Creutzfeldt-Jakob disease (CJD)?

Answer 17

Rapidly progressive dementia (weeks to months) w/ Myoclonus (“startle myoclonus”)

Question 18

What are the pathologic findings of Creutzfeldt-Jakob disease?

Answer 18

Spongiform cortex
Prions
- (PrPc -> PrPsc sheet [ß-pleated sheet resistant to proteases])

Question 19

What is the 2nd most common cause of dementia in elderly?

Answer 19

Multi-infarct

Question 20

What vitamin deficiencies can cause dementia?

Answer 20

Vitamins B1, B3, B12