Deck 20: Neurodegenerative Diseases, by Jennifer R : Yates, Lander University

Question 1

Two of the most frequent symptoms associated with Parkinson's disease include _______ and _______.

A) difficulty initiating movement; slowness of movement
B) unwanted motor activity; dementia
C) pill-rolling tremors; retropulsion
D) resting tremor; urge to move certain body parts

Answer 1

A

Question 2

Which motor dysfunction is not seen in Parkinson's disease?

A) Bradykinesia
B) Pill-rolling tremor
C) Rigidity
D) Ballistic movements

Answer 2

D

Question 3

Although slowness of movement is a hallmark of Parkinson's disease, some patients experience an uncontrollable acceleration of gait, called

A) anteropulsion.
B) akathisia.
C) festination.
D) dystonia.

Answer 3

C

Question 4

Pharmacological treatments for Parkinson's disease include all of the following except drugs that

A) prevent motor complications.
B) are given in addition to l-DOPA therapy.
C) prevent the initial stages of neuronal degeneration.
D) treat a particular symptom.

Question 5

l-DOPA increases dopamine activity by _______, while the drug selegiline (Eldepryl) does so by _______. Pramipexole (Mirapex) does so by _______.

A) increasing synthesis; acting on DA receptors; inhibiting reuptake
B) acting on DA receptors; inhibiting reuptake; inhibiting metabolism
C) increasing DA cell firing; increasing synthesis; inhibiting reuptake
D) increasing synthesis; inhibiting metabolism; acting on DA receptors

Question 6

Which of the following is the correct order of degeneration in Parkinson's disease?

A) Motor nucleus of the vagus $\rightarrow$ substantia nigra $\rightarrow$ raphe nucleus and locus coeruleus $\rightarrow$ temporal neocortex $\rightarrow$ temporal mesocortex
B) Motor nucleus of the vagus $\rightarrow$ raphe nucleus and locus coeruleus $\rightarrow$ substantia nigra $\rightarrow$ temporal mesocortex $\rightarrow$ temporal neocortex
C) Raphe nucleus and locus coeruleus $\rightarrow$ motor nucleus of the vagus $\rightarrow$ temporal mesocortex $\rightarrow$ temporal neocortex $\rightarrow$ substantia nigra
D) Raphe nucleus and locus coeruleus $\rightarrow$ motor nucleus of the vagus $\rightarrow$ substantia nigra $\rightarrow$ temporal neocortex $\rightarrow$ temporal mesocortex

Question 7

The major consequence of degeneration of substantia nigra cells is a decrease in _______ input into the striatum, which results in less _______ of the globus pallidus and greater tonic inhibition of the _______, reducing _______ input to the cortex.

A) NMDA; excitation; thalamus; excitatory
B) dopamine; inhibition; subthalamic nucleus; inhibitory
C) dopamine; inhibition; thalamus; excitatory
D) acetylcholine; excitation; caudate/putamen; inhibitory

Question 8

Which statement about the "MitoPark" mouse model is true?

A) Mitochondrial transcription factor A (TFAM) is inactivated in this model.
B) The model does not support for the theory of loss of dopaminergic neurons PD.
C) Substantia nigra cells die because of deficiencies in cellular respiration.
D) Treatment with l-DOPA enhances the observed behavioral changes.

Question 9

Protein aggregation is an important component of neuronal degeneration in PD, with _______, composed of _______, formed in the affected cells.

A) Lewy bodies; α-synuclein
B) plaques; tau proteins
C) neurofibrillary tangles; Lewy bodies
D) proteosomes; ubiquitin

Question 10

Which surprising finding may have efficacy in early detection of PD and thus earlier and more effective treatment?

A) There is no genetic component associated with risk of PD.
B) Brain pathways that are similar in people with PD and those that have bipolar disorder.
C) PD is associated with Down syndrome.
D) A case in which a woman could actually "smell" PD

Question 11

Which statement about shortcomings of treatment for PD is true?

A) Although there are treatments that slow the disease process, there is currently no cure for PD.
B) Although symptoms can be treated, there are not currently treatments that actually slow the disease process.
C) There currently are no imaging techniques to visualize damaged DA neurons.
D) There are currently no drugs that treat primary motor symptoms of bradykinesia and tremor.

Question 12

The most significant risk factor for Parkinson's disease is

A) a history of boxing or other contact sports involving chronic skull impact.
B) exposure to illicit drugs or environmental toxins.
C) having family members with the disorder.
D) age.

Question 13

Parkinson's disease dementia affects approximately _______ of patients after 15 years of disease progression.

A) 15%
B) 15% to 40%
C) 70%
D) 100%

Question 14

The primary cellular abnormalities in AD are _______ and _______. These abnormalities are seen largely in _______ cortex.

A) amyloid precursor protein; β-amyloid; prefrontal
B) neurofibrillary tangles; amyloid plaques; frontotemporal association
C) amyloid precursor protein; neurofibrillary tangles; frontoparietal association
D) amyloid plaques; Aβ42; prefrontal

Question 15

_______ neurons are particularly susceptible to neurofibrillary tangles, which are primarily made of _______ proteins.

A) Pyramidal; tau
B) Cholinergic; Aβ
C) Prefrontal cortex; ubiquitin
D) Polygonal; microtubule

Question 16

The tau protein is normally associated with microtubules and is important in cellular function. In tangles, it is abnormally

A) acetylated.
B) folded.
C) methylated.
D) phosphorylated.

Question 17

The typical pattern of tangles in Alzheimer's disease follows which pattern?

A) Entorhinal cortex → hippocampus → neocortex
B) Neocortex → entorhinal cortex → hippocampus
C) Basal forebrain → entorhinal cortex → hippocampus → neocortex
D) None of the above; degeneration occurs primarily in cholinergic and monoaminergic systems, not brain regions.

Question 18

The two most basic risk factors for AD are

A) family history of dementia and advancing age.
B) history of illicit substance use and advancing age.
C) history of head trauma and bipolar disorder or PTSD.
D) diabetes and high cholesterol.

Question 19

Familial cases of AD (known as autosomal dominant Alzheimer's disease) are caused by mutations of _______ genes, including _______, and make up _______ of cases.

A) risk; presenilin-1; 0.5%
B) deterministic; apolipoprotein E; 5%
C) deterministic; presenilin-2; 5%
D) risk; apolipoprotein E; 15%

Question 20

Which statement about apolipoprotein E, which has the gene greatest influence on risk of AD development, is true?

A) Its receptor may be increased in the brains of AD patients.
B) The ApoE gene is a risk gene with only a minor influence on disease development.
C) ApoE is normally involved in moving excess cholesterol from the blood to the liver.
D) Early onset AD is not linked to the E4 form of the gene.

Question 21

Most people with _______ develop plaques and tangles and some develop AD, possibly because they _______.

A) heart disease; have high cholesterol
B) Parkinson's disease; develop Lewy bodies
C) Huntington's disease; carry the APP gene
D) Down syndrome; have three copies of the APP gene

Question 22

Which statement about the diagnosis of Alzheimer's disease is true?

A) A presence myeloid plaques allows for a positive determination of AD
B) It cannot be unequivocally determined until post-mortem examination shows NFTs and amyloid plaques.
C) Florbetapir (Amyvid), a small molecule that binds β-amyloid, can be used in combination with PET scan to confirm an AD diagnosis.
D) Fluortaucipir F18 (Tauvid) as a PET tracer to visualizes tau protein aggregates to confirm an AD diagnosis.

Question 23

A critical characteristic of a good animal model of AD is

A) the development of neurofibrillary tangles.
B) the presence of progressive neuropathology/symptomology.
C) that it be naturally occurring.
D) that it be in a mammalian species.

Question 24

Which statement about the cholesterol-fed rabbit model of AD is false?

A) The rabbits only developed AD symptoms if they also were administered copper.
B) The rabbits showed cognitive deficits.
C) Neurofibrillary tangles and αβ deposits were seen in the rabbits.
D) Neuronal loss occurred in the frontal cortex and hippocampus.

Question 25

In addition to memory problems, a person afflicted with Alzheimer's might exhibit all of the following except for

A) difficulty driving.
B) a lack of enjoyment of previously pleasurable activities.
C) difficulty swallowing.
D) impulse control issues and compulsive behaviors.

Question 26

Which of the following is not a psychiatric symptom seen in AD?

A) Delusions
B) Depressed mood
C) Manic episodes
D) Hallucinations

Question 27

NMDA receptor agonists and _______ are currently in use for the treatment of AD.

A) adenosine receptor agonists
B) D₂ receptor antagonists
C) cholinesterase inhibitors
D) acetylcholine receptor agonists

Question 28

In AD, Neurodegeneration leads to glutamate release and _______, so _______ can be used in treatment.

A) depolarization block; an NMDA antagonist
B) excitotoxicity; sodium channel blockers
C) excitotoxicity; an NMDA antagonist
D) excess neurotransmitter release; calcium channel blockers

Question 29

Delivering drugs to the brain for treatment of AD is difficult because of the blood-brain barrier. Which technology has been used to successfully open the barrier and thus provides hope for future treatment of AD?

A) Transcranial magnetic stimulation
B) Magnetic resonance-guided focused ultrasound
C) Cingulotomy
D) Electroconvulsive therapy

Question 30

Alzheimer's disease is the most common form of dementia, affecting _______ of the 25 million patients worldwide.

A) 25%
B) 30% to 50%
C) 50% to 70%
D) 90%

Question 31

The likelihood of developing Huntington's disease depends on

A) both inheriting the huntingtin gene and dietary factors.
B) inheriting a huntingtin gene with more than 35 CAG repeats.
C) both a family history of HD and advancing age.
D) inheriting a huntingtin gene with more than 40 CAG repeats.

Question 32

Huntington's disease is unusual compared to other neurodegenerative disorders in that

A) it has both motor and cognitive symptoms.
B) no cure exists.
C) it has a clear genetic cause.
D) HD patients may exhibit psychiatric symptoms.

Question 33

Which of the following is not a symptom of Huntington's disease?

A) Unwanted movements that cannot be suppressed
B) Jerky, writhing movements of the limbs
C) Problems swallowing
D) Disturbances in the sense of touch and proprioception

Question 34

Brains of HD patients show severe _______ and _______.

A) basal ganglia degeneration; enlarged lateral ventricles
B) cerebellar degeneration; basal ganglia abnormalities
C) enlargement of the lateral ventricles; degeneration of the prefrontal cortex
D) striatal degeneration; enlarged fourth ventricle

Question 35

The comorbidity of HD with psychiatric symptoms/disorders is problematic for treatment of HD symptoms. Decreasing excess movement is achieved therapeutically by _______ or _______, but these treatments can lead to _______ and _______.

A) decreasing DA synthesis; blocking DA receptors; mania; dysphoria
B) depleting vesicular DA; blocking DA receptors; depression; parkinsonism
C) depleting vesicular DA; enhancing DA reuptake; schizophreniform symptoms; cognitive deficits
D) increasing DA synthesis; increasing DA release; depression; mania

Question 36

_______ is a peculiar symptom of HD and manifests as _______.

A) Sialorrhea, patients' anxiety over their condition
B) Sialorrhea, oversleeping
C) Anosognosia; patients' denial or unawareness of their condition
D) Anosognosia; loss of the sense of smell

Question 37

The early symptoms of amyotrophic lateral sclerosis (ALS) result from

A) a loss of motor neurons.
B) degeneration of muscle tissue.
C) widespread destruction of the neuromuscular junction.
D) degeneration of primary motor cortex.

Question 38

Which statement about ALS is false?

A) Death is caused by respiratory failure as motor neurons controlling this process are lost.
B) Protein aggregates known as spheroids are present in degenerating neurons.
C) Abnormal eye movements may serve as a marker for the disease prior to other symptoms.
D) There is evidence of both hereditary and nonhereditary causes, but specific genes have not been identified.

Question 39

People with ALS may experience _______, which is exaggeration of emotional responses, including uncontrolled laughing or crying, or laughing in sad situations and crying when others are laughing.

A) hypophonia
B) the emotion affect
C) anosognosia
D) the pseudobulbar affect (PBA)

Question 40

Two very different approaches that are currently being used to treat ALS are _______ and _______.

A) DA agonists; glutamate agonists
B) glutamate antagonists; free radical scavengers
C) glutamate agonists; free radical scavengers increasing neurotransmission in motor control centers of the brain
D) DA antagonists; stem cell therapies to produce new motor neurons

Question 41

Multiple sclerosis

A) is thought to result from childhood vaccines.
B) is easily diagnosed based on a core set of symptoms, including fatigue and problems with coordination.
C) is thought to be a result from autoimmune destruction of the myelin produced by oligodendrocytes.
D) is more common in the tropics than in temperate zones.

Question 42

Which of the following is considered a potential risk factor of MS?

A) A predisposition to depression
B) Living in an extremely warm climate before age 15
C) Having a first-degree relative with MS
D) Lack of vitamin E

Question 43

Treatments for MS include _______ such as _______ and treatments for exacerbations, such as _______.

A) disease-modifying treatments; interferon beta-1a; corticosteroids
B) disease-modifying treatments; potassium channel blockers; corticosteroids
C) treatments for managing symptoms; Copaxone; Aubagio
D) treatments for managing symptoms; corticosteroids; interferon beta-1a

Question 44

Treatment with a potassium channel blocker such as Ampyra could improve neurotransmission in demyelinated neurons because it

A) enhances the generation of action potentials.
B) allows transmission through demyelinated fibers in the motor pathway.
C) hyperpolarizes the membrane.
D) modulates release of acetylcholine at the neuromuscular junction.

Question 45

_______ is indicated only for treatment-resistant MS (because of its risky side effects) and its mechanism of action is _______.

A) Interferon beta-1a; enhancing function of suppressor T cells
B) Interferon beta-1a; reducing function of suppressor B cells
C) Cladribine (Mavenclad); increase of B and T cells
D) Cladribine (Mavenclad); decrease of B and T cells

Question 46

Describe the factors that contribute to the "Parkinson's shuffle," including information about rigidity and postural instability.

Question 47

Explain why l-DOPA is the primary drug treatment for Parkinson's disease. What is its mechanism of action? Identify three other ways DA receptor activation could be increased and name one drug from each of these categories.

Question 48

Describe the stages of pathological changes in Parkinson's disease and explain how a loss of dopaminergic cells in the substantia nigra leads to motor symptoms.

Question 49

Name the processes thought to be involved in neuronal degeneration in Parkinson's disease.

Question 50

Explain the process involved in the formation of amyloid plaques and neurofibrillary tangles and the consequences of their development in PD. What is the normal function of beta-amyloid? Of tau protein?

Question 51

Many neurodegenerative disorders involve excess glutamate release from degenerating neurons. Why is this undesirable and how can the consequences be decreased?

Question 52

Describe animal models of AD in three different species. What are the advantages and disadvantages of each?

Question 53

Why might psychiatric disorders such as OCD and bipolar disorder be comorbid with Huntington's disease? Consider the areas of neurodegeneration and the circuits that may be interrupted.

Question 54

According to the updated McDonald Criteria, for a diagnosis of MS to be made, which criteria must be met?

Question 55

Explain the procedures used to assess disease progression in MS and identify/describe the four courses the disease can take, including the incidence of each.

Question 56

What is meant by secondary or tertiary symptoms of MS? Include two examples.

Question 57

Describe the different categories of treatments available for MS, give an example of each one and explain its mechanism of action.