Pbd 2 Exam 3 - Week 8

Front 1

True/False
The hallmark of Alzheimer's disease is the presence of neurofibrillary plaques and amyloid tangles.

Back 1

amyloid plaques and neurofibrillary tangles.

Front 2

True/False
The motor cortex is the first area of the brain to be affected by Alzheimer's Disease.

Back 2

False. The hippocampus

Front 3

True/False
Amyloid plaques are only seen in Alzheimer's Disease, not normal aging.

Back 3

False. are also seen in normal aging

Front 4

True/False
Allele apoE4 increases Abeta 1-42 deposition and is associated with increased incidence of Alzheimer's Disease.

Back 4

True

Front 5

Which of the following is NOT implicated in reducing the incidence of or the delay of onset of Alzheimer's Disease.
a. nicotine
b. estrogen
c. high cholesterol levels
d. reading / higher education
e. NSAID

Back 5

c. high cholesterol levels

Front 6

True/False
Analogous to the degeneration of movement regulation-related neurological pathways with age, loss of memory and cognition-associated neural pathways is part of the normal aging process.

Back 6

False. is not part

Front 7

True/False
A person who carries both alleles of the ApoE4 gene is definitely going to develop Alzheimer’s disease because APE4 is a causative gene for the disease.

Back 7

False. is more likely to develop

Front 8

True/False
Despite decades of intense research, the etiology of Parkinson’s disease, Alzheimer’s disease or Huntington’s disease remains unclear.

Back 8

False. Parkinson's disease and Alzheimer's disease only

Front 9

True/False
Most cases of Parkinson’s and Alzheimer’s diseases are results of known gene mutations.

Back 9

False. are results of an unknown etiology.

Front 10

True/False
Only gamma-secretase is needed to release Abeta (1-40) or Abeta(1-42) peptides from APP.

Back 10

False. Beta-secretase and gamma-secretase are needed

Front 11

The predominant form of beta-amyloid peptide in a diffuse plaque is:
A). AB 1-40
B). APP
C). AB 1-42
D). sAPP

Back 11

C). AB 1-42

Front 12

Generation of the Abeta(1-42) peptide requires the enzymatic activity of:
a)Alpha-secretase.
b)Alpha-secretase and beta-secretase.
c)Beta-secretase and gamma-secretase.
d)Beta-secretase and APP gene mutations.

Back 12

c)Beta-secretase and gamma-secretase.

Front 13

In relation to the development of Alzheimer’s disease, the following can be said for the ApoE4 protein:
a)ApoE4 gene is one of the causative genes of some cases of Alzheimer’s disease.
b)Comparing to people carrying various forms of the ApoE gene, a person having both ApoE4 alleles is at the highest risk of developing Alzheimer’s disease.
c)Comparing to people carrying various forms of the ApoE genes, a person having both ApoE4 alleles is at the lowest risk of developing Alzheimer’s disease.
d)Comparing to people carrying various forms of the ApoE genes, a person having both ApoE4 alleles is at the same risk as others of developing Alzheimer’s disease

Back 13

b)Comparing to people carrying various forms of the ApoE gene, a person having both ApoE4 alleles is at the highest risk of developing Alzheimer’s disease.

Front 14

_________________ are formed outside of neurons and _________________ are formed inside of neurons.

Back 14

Plaques
Tangles

Front 15

True/False
Mutations in the beta-secretase gene increase its efficiency to produce beta-amyloid peptide (1-42).

Back 15

False. APP gene increase beta-secretases efficiency

Front 16

True/False
Hypophosphorylation of tau reduces its affinity to microtube and promotes the formation of PHF.

Back 16

False. Hyperphosphorylation

Front 17

True/False
In patients with Alzheimer Disease (AD), neurofibrilliary tangles and neuritic plaques are only found in the limbic system.

Back 17

False. not only found

Front 18

True/False
The leading psychiatric symptom associated with AD is personality change.

Back 18

True

Front 19

True/False
A neurologic finding associated with Parkinson's Disease would be decreased muscle tone and cerebellar ataxia.

Back 19

False. increased muscle tone and rigidity.

Front 20

True/False
The most common cause of Parkinson's Disease is a genetic mutation on chromosome 4.

Back 20

False. of Huntington's Disease

Front 21

True/False
Akinesia is a symptom of Parkinson's Disease.

Back 21

True

Front 22

Which of the following is NOT characteristic of Parkinson's Disease:
a. loss of dopaminergic neurons of the substantia nigra
b. loss of dopamine input to the striatum
c. hypocholinergic striatal tone
d. deposition of Lewy bodies
e. all of the above are characteristic

Back 22

c. hypocholinergic striatal tone

Front 23

True/False
In Parkinson's disease the loss of dopaminergic input into the striatum results in an exaggerated increase in GABAergic inhibition of the thalamus via pathways from the medial globus pallidus and the pars reticulata of the substantia nigra.

Back 23

True

Front 24

True/False
Mutations in the gene coding for alpha-synuclein account for the majority of all cases of Parkinson's disease.

Back 24

False. do not account for

Front 25

True/False
The metabolism of dopamine by monoamine oxidase B results in the formation of dopamine quinone and superoxide radical.

Back 25

False. ?

Front 26

True/False
The etiology of sporadic cases of Parkinson’s disease that represent >95% of all cases of the diseases is known.

Back 26

False. is unknown.

Front 27

The neurotransmitter that is affected the first and foremost in Parkinson’s disease is
a)GABA
b)Levodopa
c)Dopamine
d)Acetylcholine

Back 27

c)Dopamine

Front 28

True/False
Most cases of Parkinson’s disease are a result of mutations in several very important genes that include alpha-synuclein, parkin and UCH-L1.

Back 28

False. are a result of an unknown etiology.

Front 29

True/False
Mutations in several very important genes that include alpha-synuclein, parkin and UCH-L1 are not responsible for the majority of the cases of Parkinson’s disease.

Back 29

True

Front 30

Loss of a subset of neurons that produce the following neurotransmitter underlies the neuro-pathology of Parkinson’s disease .
a. GABA
b. Acetylcholine
c. Levodopa
d. Dopamine

Back 30

d. Dopamine

Front 31

True/False
Gene mutations are responsible for the majority of cases of Parkinson’s disease.

Back 31

False. are not responsible

Front 32

True/False
Analogous to the degeneration of movement regulation-related neurological pathways with age, loss of memory and cognition-associated neural pathways is part of the normal aging process.

Back 32

False. is not part of

Front 33

True/False
In Parkinson’s disease, there is a massive loss of dopamine neurons in the pathway from ventral tagmental area (VTA) and nuclear accumbens that plays a key role in the regulation of movements.

Back 33

False. substantia nigra and striatum

Front 34

True/False
Depending on the type of post-synaptic dopamine receptors it interacts with, dopamine released in the striatum by dopamine neurons can either stimulate (facilitate) or inhibit (reduce) the activity of the post-synaptic neurons.

Back 34

True

Front 35

True/False
Most cases of Parkinson’s and Alzheimer’s diseases are results of known gene mutations.

Back 35

False. are results of unknown etiology.

Front 36

In the ganglia-thalamocortical circuitry, destruction of 90% the GABA-releasing neurons that originate in Gpe and innervate into STN will significantly:
a)Increase the release of GABA of neurons that go from striatum to Gpe.
b)Decrease the release of GABA of neurons that go from striatum to Gpe.
c)Increase the release of GABA of neurons that go from STN to SNpr and/or Gpi.
d)Increase the release of glutamate of neurons that go from STN to SNpr and/or Gpi.

Back 36

d)Increase the release of glutamate of neurons that go from STN to SNpr and/or Gpi.

Front 37

______________ is a term used to describe the continuous occurrence of rapid jerky movements seen in Huntington's disease.

Back 37

Chorea

Front 38

In the course of the progression of Huntington’s disease, neuronal loss is firstly and mostly observed in:
A). Hippocampus
B). Substantia nigra
C). Striatum
D). Cortex

Back 38

C). Striatum

Front 39

Of the neurodegenerative diseases that we’ve covered in the PBD lectures, which one(s) has a clear genetic link (regarding etiology) for all cases of the disease(s):
a)Parkinson’s disease, Alzheimer’s disease and Huntington’s disease.
b)Alzheimer’s disease and Huntington’s disease.
c)Huntington’s disease.
d)Parkinson’s disease and Huntington’s disease.

Back 39

c)Huntington’s disease.

Front 40

In relation to the development of Huntington’s disease, the following can be said for the CAG (encoding for glutamine) repeats in the Huntingtin gene:
a). A person inherits a fixed number of repeats from parents and the same number of repeats gets passed down the generations.
b). The length of repeats for a given person has no association with the probability of developing the disease.
c). During vertical transmission (parents to offspring), the length of the repeats in a parent’s gene affects the probability of change regarding the number of repeats in the offspring’s gene.
d). The longer the repeats, the lower the probability of developing the disease.

Back 40

c). During vertical transmission (parents to offspring), the length of the repeats in a parent’s gene affects the probability of change regarding the number of repeats in the offspring’s gene.

Front 41

True/False
In Huntington’s disease, pathological changes at the cellular level start with the loss of _____________-releasing neurons in the _____________.

Back 41

GABA
Striatum

Front 42

True/False
Parkinson’s disease is a result of significant degeneration of the dopaminergic pathway between ventral tagmental area and nuclear accumbens that plays a key role in the regulation of movements.

Back 42

False. between substantia nigra and striatum that

Front 43

True/False
Dopamine released in the striatum by dopamine nerve terminals interacts with post-synaptic neurons only to reduce their activity (i.e., their release of relevant neurotransmitters).

Back 43

False. increase or decrease

Front 44

True/False
A small fraction (<5-10%) of Parkinson’s or Alzheimer’s diseases are caused by mutations in several genes.

Back 44

True

Front 45

True/False
Alpha-secretase is needed to produce the Abeta (1-40) or Abeta(1-42) peptides from APP.

Back 45

False. Beta-secretase and gamma-secretase are

Front 46

One of the primary neuropathological hallmarks of Huntington's disease is:
a. depigmentation of the substancia nigra
b. decreased quinolinic acid formation
c. a loss of dopamine neurons
d. a loss of striatal GABAergic neurons
e. Only B and D are true

Back 46

d. a loss of striatal GABAergic neurons

Front 47

Which of the following statements are true about Huntington's Disease?
a. There is a progressive increase in chorieform movements and hyperreflexia
b. Huntington's disease has a recessive mode of inheritance
c. Onset generally occurs in the 6th or 7th decade
d. It is occurs predominantly in people of color
e. A and C are true

Back 47

a. There is a progressive increase in chorieform movements and hyperreflexia

Front 48

Huntingtin
a. is an abnormal gene product
b. is made when there are too many (CAG)n repeats in the HD gene
c. forms insoluble aggregates that can impair protein interactions
d. A and B are true
e. All of the above are true

Back 48

e. All of the above are true

Front 49

Parkinson's disease is most likely characterized by:
a. hyperactivity
b. memory loss
c. reduced sweating
d. resting tremor
e. hyperflexibility

Back 49

d. resting tremor

Front 50

During which of the following periods do about 2/3 of Parkinson's disease patients show symptoms:
a. 10-30 years
b. 30-50 years
c. 50-70 years
d. 70-100 years

Back 50

c. 50-70 years

Front 51

The loss of neuromelanin in the substantia nigra of Parkinson's disease patients reflects:
a. the loss of dopamine neurons in this region
b. the loss of dopamine neurons in the neocortex projecting to this region
c. the increased activation of the thalamus
d. a compensatory mechanism in the brain to protect serotonin neurons
e. the loss of serotonin neurons in this region

Back 51

a. the loss of dopamine neurons in this region

Front 52

Lewy bodies are:
a. amyloid containing plaques found outside of dying cells
b. extracellular processes seen in Parkinson's disease
c. bilateral brain regions affected by Parkinson's disease
d. bodies that contain neuromelanin
e. intracellular inclusions observed in Parkinson's disease

Back 52

e. intracellular inclusions observed in Parkinson's disease

Front 53

Which of the following would you LEAST likely see in a Parkinson's disease patient?
a. dopamine loss in the striatum
b. dopamine loss in the substantia nigra
c. dopamine los in the area postrema
d. abnormalities in gait and posture
e. Lewy bodies

Back 53

c. dopamine los in the area postrema

Front 54

Which of the following genes has been associated with Alzheimer's disease?
a. beta-amyloid gene on chromosome 21
b. presenilin 1 gene on chromosome 14
c. presenilin 2 gene on chromosome 1
d. all of the above
e. none of the above

Back 54

d. all of the above

Front 55

Which of the following factors has NOT been associated with Alzheimer's disease (either increased or decreased risk)?
a. aging
b. smoking
c. reading
d. body weight
e. cholesterol levels

Back 55

d. body weight

Front 56

Familial Alzheimer's disease
a. is more common than sporadic cases of the disease
b. is typically autosomal dominant and occurs earlier than sporadic cases
c. is associated with reduced levels of Abeta 1-42 in the brain compared to normal people
d. usually progresses more slowly than sporadic Alzheimer's disease
e. all of the above are true statements

Back 56

b. is typically autosomal dominant and occurs earlier than sporadic cases

Front 57

Alzheimer's disease:
a. causes a gradual loss of short term memory followed by long term memory loss
b. improves cognition but impairs memory
c. increases personality affect
d. reduces the likelihood of clinical depression
e. causes a loss of long term memory prior to loss of short term memory

Back 57

a. causes a gradual loss of short term memory followed by long term memory loss

Front 58

Diagnosis of Alzheimer's disease must rule out:
a. hypercolesterolemia
b. diabetes
c. acute vascular causes
d. Lewy body formation
e. psychosis

Back 58

e. psychosis

Front 59

One of the arguments AGAINST amyloid plaques being the cause of Alzheimer's disease is that:
a. amyloid plaques are found in Parkinson's patients, not Alzheimer's patients
b. drugs that completely cure Alzheimer's disease have no effect on amyloid plaques
c. transgenic mice that overexpress amyloid plaques have few signs of Alzheimer's disease
d. amyloid plaques are only found in the periphery, not the brain
e. amyloid plaques are only found in familial cases of the disease

Back 59

c. transgenic mice that overexpress amyloid plaques have few signs of Alzheimer's disease

Front 60

Neurofibrillary tangles seen in Alzheimer's disease:
a. are deficient in the protein amyloid
b. have normal levels of the protein amyloid, but have lower than normal levels of phosphorylated amyloid
c. are very soluble
d. consist mostly of amyloid protein
e. are rich in protein tau

Back 60

e. are rich in protein tau

Front 61

Alzheimer's disease affects many neurotransmitters systems, but the most studied include:
a. nigrostriatal dopamine neurons
b. nigrostriatal GABA neurons
c. septal hippocampal cholinergic neurons
d. thalamic glutamate interneurons
e. hypothalamic substance P neurons

Back 61

c. septal hippocampal cholinergic neurons

Front 62

A mutation in alpha-synuclein has been found that:
a. leads to increased amyloid protein expression
b. is associated with Parkinson's disease
c. is curative for Huntington's disease
d. reduces GABA levels levels in the neocortex
e. improves motor activity in humans

Back 62

b. is associated with Parkinson's disease

Front 63

For Parkinson's disease, pallidotomy refers to:
a. stimulation of the thalamic body
b. stem cell transplantation
c. NGF treatment of the globus pallidus
d. lesioning of the internal segment of the globus pallidus
e. GABA-activation with benzodiazepines

Back 63

d. lesioning of the internal segment of the globus pallidus

Front 64

Which of the following is NOT used to treat Parkinson's disease?
a. L-DOPA
b. dopamine agonists
c. anti-cholinergic
d. COMT-inhibitors
e. NGF

Back 64

e. NGF

Front 65

The most common neurodegenerative disease is:
a. Parkinson's disease
b. Alzheimer's disease
c. Huntington's disease
d. Lou Gehrig's disease

Back 65

b. Alzheimer's disease

Front 66

Parkinson's disease is a result of massive loss of dopaminergic neurons in:
a. Subthalamic nucleus
b. Substantia nigra
c. Cortex
d. Hippocampus

Back 66

b. Substantia nigra

Front 67

Simply stated, decreased movements observed in patients with Parkinson's disease is result of:
a. Decreased stimulatory output from dopamine neurons in the affected region
b. Increased inhibitory output from dopamine neurons in the affected region
c. Decreased inhibitory output from dopamine neurons in the affected region
d. Increased stimulatory output from dopamine neurons in the affected region

Back 67

c. Decreased inhibitory output from dopamine neurons in the affected region

Front 68

The cardinal clinical signs of Parkinson's disease are:
a. Bradykinesia, tremor, chorea and shaking
b. Memory loss, depression, restlessness and anxiety
c. Slow movement, resting tremor, rigidity and posture instability
d. Chorea, memory loss and muscle weakness

Back 68

c. Slow movement, resting tremor, rigidity and posture instability

Front 69

The following pharmacological agents are used to treat the motor dysfunctions in patients with Parkinson's disease:
1. L-tyrosine as a dopamine biosynthesis precursor
2. L-DOPA as a dopamine biosynthesis precursor
3. Inhibitors of DOPA decarboxylase to increase dopamine synthesis
4. Inhibitors of COMT to increase availability of dopamine
5. Dopamine agonist to mimic dopamine
a. All of the above
b. 1, 3 and 5
c. 2, 4 and 5
d. 2 and 4

Back 69

c. 2, 4 and 5

Front 70

The predominant cases of Parkinson's disease:
a. Occur in early adulthood
b. Are linked to mutations in related genes such as parkin and alpha-synuclein
c. Occur in families with a history of the disease
d. Occur late in life

Back 70

d. Occur late in life

Front 71

The double-hit hypothesis for the development of Parkinson's disease suggests that:
a. Dopamine neurons receive insults from two toxins simultaneously
b. Dopamine neurons receive two toxic insults at different times
c. Dopamine neurons receive an acute toxic insult over the otherwise normal aging process
d. Dopamine neurons receive one insult from a toxin that enters the body and mutations in certain genes deliver another blow to the dopamine neurons

Back 71

c. Dopamine neurons receive an acute toxic insult over the otherwise normal aging process

Front 72

The development of most cases of Alzheimer's disease is the result of:
a. Mutations of APP genes
b. Mutations in PS1 and PS2 genes
c. Mutations in ApoE genes
d. None of the above

Back 72

d. None of the above
(Etiology is unknown in most cases)

Front 73

By estimate, the percentage of people over 85 who will have Alzheimer's disease is:
a. 29%
b. 50%
c. 80%
d. 15%

Back 73

b. 50%

Front 74

The pathological hallmarks of Alzheimer's disease are:
a. Lewy bodies and cytoplasmic inclusions
b. Diffuse plaques and neurofibrillary tangles
c. Neuritic plaques and neurofibrillary tangles
d. Diffuse plaques and senile plaques

Back 74

c. Neuritic plaques and neurofibrillary tangles

Front 75

The generation of A-beta (1-42) requires the enzymatic activity of:
a. Alpha-secretase and beta-secretase
b. Beta-secretase
c. Gamma-secretase
d. Gamma-secretase and beta-secretase

Back 75

d. Gamma-secretase and beta-secretase

Front 76

The generation of A-beta (1-42) or A-beta (1-40) requires the enzymatic activity of:
a. Alpha-secretase and beta-secretase
b. Beta-secretase
c. Gamma-secretase
d. Gamma-secretase and beta-secretase

Back 76

d. Gamma-secretase and beta-secretase

Front 77

Over-production of A-beta (1-42) can be result of mutations in:
a. APP genes
b. PS1 gene
c. PS2 gene
d. All of the above

Back 77

d. All of the above

Front 78

A key component of neurofibrillary tangles is:
a. A-beta (1-42)
b. Neurofilament
c. The protein tau which forms paired helical filamental structures
d. The cytoskeletal protein microtubule

Back 78

c. The protein tau which forms paired helical filamental structures

Front 79

In studying the mechanism of neuron death in Alzheimer's disease, oxidative injuries of neurons may activate kinases which:
a. Assemble tau protein into bundles
b. Remove phosphate groups from tau
c. Add phosphate groups to tau
d. Bundle up microtubule proteins

Back 79

c. Add phosphate groups to tau

Front 80

A person at the greatest risk to develop Alzheimer's disease is the one who carries the following ApoE alleles:
a. E2/E3
b. E2/E4
c. E3/E4
d. E4/E4

Back 80

d. E4/E4

Front 81

Development of Huntington's disease is result of:
a. Exposure to specific neurotoxins in early life
b. Posessing an extra allele of a specific chromosome
c. Mutations that change the nature of specific amino acids in one particular protein
d. Mutations in a specific gene resulting in the appearance of numerous repeats of a particular amino acid

Back 81

d. Mutations in a specific gene resulting in the appearance of numerous repeats of a particular amino acid

Front 82

In patients with Huntington's disease, chorea is a result of:
a. Loss of dopamine neurons leading to loss of dopamine
b. Loss of striatal dopamine fibers leading to loss of dopamine
c. Loss of striatal cholinergic neurons leading to loss of acetylcholine
d. Loss of striatal GABAergic neurons leading to loss of inhibition on dopamine neurons

Back 82

d. Loss of striatal GABAergic neurons leading to loss of inhibition on dopamine neurons

Front 83

Since Huntington's disease is autosomal dominant in heredity, children of a HD patient
a. will have a 33% chance of developing the disease
b. Females will have a greater chance of developing the disease
c. will have an equal probability to develop the disease
d. will never develop the disease

Back 83

c. will have an equal probability to develop the disease

Front 84

Which of the following statements is correct:
a. Memory loss observed in Alzheimer's patients reflects an accelerated degenerative process on top of the normal decay of memory-related functions occurring as a normal part of aging
b. Degeneration of dopamine neurons in the brain occurs with aging in a normal person
c. Drugs are available now to stop the neurodegenerative process observed in Parkinson's disease and the same is true for Alzheimer's disease
d. Mutations in Parkin and alpha-synuclein, as well as several other recently identified genes are responsible for 50% of the cases of Parkinson's disease

Back 84

b. Degeneration of dopamine neurons in the brain occurs with aging in a normal person

Front 85

A degenerative neurological disorder is a disease of the neural system that:
A. Affects the cells in the brain.
B. Involves a progressive destruction of particular brain cells and related functions.
C. Cause a sudden loss of critical brain functions.
D. Reduces the regeneration of brain cells.

Back 85

B. Involves a progressive destruction of particular brain cells and related functions.

Front 86

Regardless of etiology, the most prominent and specific pathological changes observed in brains of Parkinson’s disease are:
A. Dilation of ventricles due to a massive loss of neurons in the brain.
B. Formation of tangles and plaques in the affected neurons/regions.
C. Degeneration of the dopaminergic pathway between ventral tagmental area and nucleus accumbens area.
D. Degeneration of the dopaminergic pathway between substantia nigra and striatum.

Back 86

D. Degeneration of the dopaminergic pathway between substantia nigra and striatum.

Front 87

Dopamine is a key neurotransmitter in relation to the pathogenesis of Parkinson’s disease because:
A. It binds dopamine receptors on neurons in the substantia nigra to regulate movements.
B. It binds dopamine receptors on neurons in the striatum to send out a stimulative signal to neurons in other regions to regulate movements.
C. It interacts with dopamine receptors on neurons in the striatum to maintain a fine balance between two opposing pathways to precisely regulate movements.
D. It interacts with other neurons in the brain to suppress the inhibitory signal sent out of basal ganglia to fine tune the movements.

Back 87

C. It interacts with dopamine receptors on neurons in the striatum to maintain a fine balance between two opposing pathways to precisely regulate movements.

Front 88

The typical clinical symptoms of Parkinson’s diseases include:
A. Bradykinesia, slow movement, stiffness, and rigidity.
B. Bradykinesia, rigidity, tremor, and posture instability.
C. Hyperkinesia, chorea, shaking, and slow movement.
D. Hypokinesia, akinesia, pill-rolling and speech impairment.

Back 88

B. Bradykinesia, rigidity, tremor, and posture instability.

Front 89

James Parkinson has a disease named after him because:
A. He died of the disease and while battling the disease, he wrote a long memoir detailing the symptoms of the disease.
B. He was the first to document and publish his diagnosis of the disease based on his careful and detailed description of his patients whom he received during the Spanish flu era in the early 1900’s.
C. His enormous contribution through careful and detailed description of his patients whom he received during the Spanish flu era in the early 1900’s was recognized by his peers such that he received the Nobel Prize in Medicine in 1921.
D. He was the first to document and publish his diagnosis of the disease based on his careful and detailed description of patients.

Back 89

D. He was the first to document and publish his diagnosis of the disease based on his careful and detailed description of patients.

Front 90

Some of the risk factors proposed for the development of idiopathic Parkinson’s disease include:
A. Exposure to certain infectious agents, environmental toxins and oxidative damage.
B. Exposure to certain infectious agents, environmental toxins and too much exposure to sunlight.
C. Exposure to environmental toxins and carrying certain mutations in genes such as Parkin and alpha-synuclein.
D. All of the above

Back 90

A. Exposure to certain infectious agents, environmental toxins and oxidative damage.

Front 91

The search for mutations related to the development of Parkinson’s disease:
A. Has associated mutations in nearly a dozen genes/loci with genetic PD cases.
B. Has uncovered mutations/genes that are responsible for the development of more than 50% of the PD cases.
C. Has identified mutations in Parkin, a gene so named because certain mutations in this gene are responsible for more than 50% of the PD cases.
D. Has led to discovery that mutations in genes that are involved in protein folding in the neurons are the causative factor for the development of Parkinson’s disease.

Back 91

A. Has associated mutations in nearly a dozen genes/loci with genetic PD cases.

Front 92

Years of intensive research on Parkinson’s Disease has provided us with:
A. A rapid lab test to make a firm diagnosis of the disease.
B. Multiple intervention strategies to change the course of the neurodegeneration.
C. Significantly enriched our knowledge on the disease process.
D. Effective means to prevent the development of the disease.

Back 92

C. Significantly enriched our knowledge on the disease process.

Front 93

The development of a minority of the cases of Alzheimer’s disease is the result of:
A. Mutations in PINK-1, DJ-1 or UCH-1 genes
B. Mutations in PS1, PS2, or APP genes
C. Mutations in PS1, PS2, or ApoE genes
D. All of the above

Back 93

B. Mutations in PS1, PS2, or APP genes

Front 94

The prevalence of Alzheimer’s disease (~4 million) is higher than that of Parkinson’s disease (~1 million) due to the fact that:
A. Memory loss is part of the aging process.
B. There are more genes that carry disease-causing mutations for AD.
C. Both A and B.
D. Neither A nor B.

Back 94

D. Neither A nor B.

Front 95

In postmortem brains of Alzheimer’s disease, the typical pathological findings are:
A. Lewy bodies, cytoplasmic inclusions and disappearance of pigmented cells in the midbrain.
B. Plaques that involve multiple cell types in the brain and neurofibrillary tangles in affected neurons.
C. Senile plaques and neurofibrillary tangles at the microscopic level and cortical atrophy and ventricular dilation at the gross level.
D. Extra-cellular senile plaques and intra-cellular neurofibrillary tangles

Back 95

C. Senile plaques and neurofibrillary tangles at the microscopic level and cortical atrophy and ventricular dilation at the gross level.

Front 96

The approximate percentage of people over 75 currently estimated to have Alzheimer;s disease is:
A. 85%
B. 68%
C. 18%
D. 1.8%

Back 96

B. 68%

Front 97

The core of senile plaque is made of:
A. Amyloid proteins
B. Alpha-synuclein
C. The protein tau which forms paired-helical filamental structures
D. The cytoskeletal protein microtubule

Back 97

A. Amyloid proteins

Front 98

The generation of A-beta (1-40) requires the enzymatic activity of:
A). Alpha-secretase
B). Beta-secretase
C). Gamma-secretase and alpha-secretase
D). Gamma-secretase and beta-secretase

Back 98

D). Gamma-secretase and beta-secretase

Front 99

Ways known to lead to an increased generation of A-beta (1-42) include:
A. Increase activity of alpha-secretase
B. Certain mutations in APP, PS1 and PS2 genes
C. None of the above.
D. All of the above.

Back 99

B. Certain mutations in APP, PS1 and PS2 genes

Front 100

A critical event in the formation of neurofibrillary tangles can be:
A. Increased production of A-beta (1-42).
B. Over-phosphorylation of microtubule resulting in its reduced affinity to tau and the formation of paired helical filaments.
C. Over-phosphorylation of tau resulting in its reduced affinity to microtubule and the formation of paired helical filaments.
D. Increased production of both tau and microtubule.

Back 100

C. Over-phosphorylation of tau resulting in its reduced affinity to microtubule and the formation of paired helical filaments.

Front 101

A person at the least risk to develop Alzheimer’s disease is the one who carries the following
ApoE alleles:
A. E2/E2
B. E2/E3
C. E3/E4
D. E4/E4

Back 101

A. E2/E2

Front 102

Down’s syndrome is due to:
A. Mutations in APP gene
B. Trisomy of chromosome 21 on which APP genes is located.
C. The duplication of functional APP genes on both chromosomes 21 and 12.
D. Mutations in PS1 and PS2 genes.

Back 102

B. Trisomy of chromosome 21 on which APP genes is located.

Front 103

Development of Huntington’s disease is a result of:
A. Exposure to specific neurotoxins in early life.
B. Unknown factors.
C. Mutations that change the nature of specific amino acids in one particular protein.
D. Mutations in a specific gene resulting in the appearance of numerous repeats of a particular amino acid.

Back 103

D. Mutations in a specific gene resulting in the appearance of numerous repeats of a particular amino acid.

Front 104

In patients with Huntington’s disease, neurodegeneration can be defined as:
A. Loss of dopamine neurons in the substantia nigra.
B. Loss of only hippocampal neurons
C. Loss of striatal medium size spiny neurons, cortical neurons and hippocampal neurons.
D. All of the above.

Back 104

C. Loss of striatal medium size spiny neurons, cortical neurons and hippocampal neurons.

Front 105

The emotional impact of Huntington’s disease on families with HD patients is enormous because:
A. It is a hereditary disease and off-spring have an equal chance of developing the disease at some point of life.
B. Female descendants have a greater chance of developing the disease.
C. The disease always attacks people over 65 years of age.
D. None of the above.

Back 105

A. It is a hereditary disease and off-spring have an equal chance of developing the disease at some point of life.

Front 106

The fact that in certain affected families some children can develop HD before parents is believed to be due to:
A. The CAG repeats affects certain children more than their parents.
B. The CAG repeat number can expand successively over generation, especially in paternal transmission.
C. Mutations in a second gene can double the effect of CAG repeat.
D. None of the above.

Back 106

B. The CAG repeat number can expand successively over generation, especially in paternal transmission.

Front 107

True/False
The fact that nearly 80 million babyboomers are retiring does not change the prediction on the incidence of Parkinson’s and Alzheimer’s disease in this country because we are enjoying an ever higher living standards, better health care and increasing self awareness of health and quality of life in general.

Back 107

False. changes the prediction on the incidence of Parkinson’s and Alzheimer’s disease in this country despite the fact that

Front 108

True/False
The onset time for Parkinson’s disease or Alzheimer’s disease in adults is unrelated to age and therefore everyone from 20 years of age and over has an equal chance to develop either one of the diseases.

Back 108

False. is related to age and is more common in people over the age of 60 in developing either one of the diseases.

Front 109

True/False
All cases of Alzheimer’s disease and Parkinson’s disease are due to mutations in several genes.

Back 109

False. Only some

Front 110

True/False
Because we fully understand the pathogenesis of Alzheimer disease and Parkinson’s disease and we have preventive measures for both diseases, the incidence for both disease is estimated to decrease dramatically over the next 30-50 years.

Back 110

False. we do not.. dramatically increase

Front 111

True/False
Beta-secretase is the only enzyme required to produce beta amyloid peptides from the APP precursor protein.

Back 111

False. and gamma-secretase

Front 112

True/False
Certain mutations in presenilin 1 or 2 genes represent a risk factor but not a causative factor to the development of Alzheimer’s disease.

Back 112

False. represent a causative factor

Front 113

True/False
Insufficient phosphorylation of tau by protein kinases causes it to have lower affinity to microtubles, dissociate from microtubles and forms paired helical filaments.

Back 113

False. Over-phosphorylation

Front 114

True/False
Parkinson’s disease is a result of significant degeneration of the dopaminergic pathway between the substantia nigra region and nuclear accumbens region of the brain that plays a key role in the regulation of movements.

Back 114

False. and striatum

Front 115

True/False
While glutamate is a stimulatory neurotransmitter, GABA and dopamine (DA) are both bono fide inhibitory neurotransmitters.

Back 115

False. GABA only

Front 116

True/False
While too much GABA release from neurons innervating between SNpr/GPi and thalamus is to blame for movement disorders in Huntington’s disease, too little GABA release from the same neurons is to blame for movement disorders in Parkinson’s disease.

Back 116

False. Too little GABA... too much GABA

Front 117

True/False
Mutations in most of the genes related to Parkinson’s disease do not lead to improper protein folding and processing in the affected neurons.

Back 117

False. leads to

Front 118

True/False
Huntington’s disease is a result of having too few CAG repeats at the 5’ end of the Huntingtin gene or glutamine repeats in the protein. For example, individual having less than (<) 40 CAG repeats will have a 100% chance of developing Huntington’s disease at some point of their life.

Back 118

False. too many... more than (>)