DEGENERATIVE DISEASES

Question 1

name the disease

most common cause of dementia in the elderly

Answer 1

Alzheimer’s Disease

Question 2

name the disease

A group of disorders classified together because they shared clinical features

• Progressive deterioration of language
• Personality changes
• -Degeneration and atrophy of the temporal and frontal lobes

Several of these disorders share the accumulation of tau-containing deposits

Frontotemporal Dementias with Tau

Mutations
-Pick Disease

Answer 2

Frontotemporal Dementias

Question 3

name the disease

idiopathic Parkinson
progressive supranuclear palsy
corticobasilar degeneration
post encephalic parkinsonism
multiple system atrophy

Answer 3

parkinsonism

Question 4

name the disease

autosomal dominant disease causing progressive movement problems/dementia

Answer 4

Huntington’s Disease

Question 5

this correlates with clinical impairment of the AD?

Answer 5

number of neurofibrillary tangles

Question 6

biochemical markers that correlate with degree of dementia in AD?

Answer 6

• Loss of choline acetyl-transferase
• Synaptophysin immunoreactivity
• Amyloid burden***

Question 7

for pathogenesis of AD, this is created when the transmembrane protein amyloid precursor protein is sequentially cleaved by enzymes, what are these too?

Answer 7

beta amyloid (AB)

B-amyloid converting enzyme (BACE)

y-secretase

Question 8

APP, which generates AB, is cleaved by these enzymes that produce a different nonpathogenic AB?

Answer 8

alpha secretase

gamma secretase

Question 9

Amyloid Precursor Protein (APP) found on what chromosome?

Answer 9

21

Question 10

Presenilin-1 (a component of γ-secretase) found on what chromosome?

Answer 10

14

Question 11

Presenilin-2 (a component of γ-secretase) found on what chromosome?

Answer 11

1

Question 12

Apolipoprotein E found on what chromosome?

Answer 12

19

Question 13

The presence of Aβ (beta amyloid) causes

Answer 13

hyperphosphorylation of Tau

Question 14

intracellular neuronal-binding protein involved in the assembly of intraaxonal
microtubules

Answer 14

Tau

Question 15

Clinical impairment is based on the number of

Answer 15

neurofibrillary tangles

Question 16

major morphology of AD?

Answer 16

senile plaque
amyloid angiopathy
granulovascular degeneration

Question 17

pick disease is characteristic of?

noted features, microscopically?

Answer 17

Frontotemporal Dementias with Tau

pick cells and bodies

Question 18

major clinical findings of parkinsonism?

Answer 18

TRAP

resting tremor-pill rolling
cogwheel rigidity-diminished facial expression
akinesia-slow voluntary movements
posture-problems w/ equilibrium

Question 19

protein involved in synaptic transmission; mutation causes the autosomal dominant form
of PD?

Answer 19

alpha-synuclein

Question 20

name the lewy structure?

intracytoplasmic eosinophilic inclusions, composed of
presynaptic α-synuclein protein

Answer 20

Lewy Bodies

Question 21

name the lewy structure?

dystrophic neutrites that also have tons of abnormally
aggregated α-synuclein

Answer 21

Lewy Neurites

Question 22

atrophy of the frontal lobe,
parietal lobe, caudate nucleus & putamen is indicative of what disease?

pathogenesis indicates this triculeotide repeat?

Answer 22

huntingtons disease

CAG