Chapter 17
Neurodegeneration
Serge Przedborski
Questions
1.
Define neurodegeneration.
2.
What are the three broad categories of diseases of the nervous system?
3.
Provide examples of diseases from the three categories.
4.
What is expected to occur with the prevalence of neurodegenerative diseases in the forthcoming
generations and why?
5.
What is the effect of neurodegeneration on the life expectancy and for what reason(s) do patients with
neurodegenerative disease typically die?
6.
What is the common method used in classifying neurodegenerative diseases and what are the difficulties
inherent with this type of classification?
7.
Describe the different forms of genetic contributions among the neurodegenerative diseases.
8.
What does multisystemic neurodegeneration refer to?
9.
Why are neurodegenerative diseases progressive and what does it mean at the level of the whole
population of affected cells and a single affected cell?
10. Define the notion of non-cell autonomous neurodegeneration and provide an example.
11. What are the two most prevalent neurodegenerative diseases?
a. Alzheimer’s disease and stroke
b. Parkinson’s disease and brain tumor
c. Alzheimer’s disease and Parkinson’s disease
d. Huntington’s disease and amyotrophic lateral sclerosis
e. Mitochondrial encephalopathy and multiple sclerosis
12. What are typical neurodegenerative diseases without overt neuropathology?
a. Creutzfeldt-Jakob disease
b. Schizophrenia
c. Essential tremor
d. Torsion dystonia
e. All but a
13. What is the correct statement about the neuropathology of neurodegenerative diseases?
a. Neuropathologic changes in neurodegenerative diseases are always detectable in at least one region of the
nervous system.
b. Residual neurons look sick or normal.
c. In addition to the loss of neurons, there is always some gliosis and protein aggregates.
d. If different regions of the brain are affected, the type of neurons degenerating remains identical.
e. The multisystemic nature of a neurodegenerative process is explained by a trans-synaptic phenomenon.
17. Neurodegeneration
Serge Przedborski
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14. What is the correct statement about the etiology of neurodegenerative diseases?
a. The etiology of most neurodegenerative diseases is unknown.
b. Most neurodegenerative diseases are genetic.
c. Neurodegenerative diseases are only linked to nuclear gene defects.
d. Environmental toxic factors have never been implicated in the occurrence of neurodegenerative diseases.
e. Genetic/environmental interactions are unknown causes of neurodegeneration.
15. Which of the following statements is true about the pathogenesis?
a. The death of neurons is caused by a unique pathogenic mechanism triggered by an etiologic factor.
b. Neurodegenerative disorders never become multisystemic upon progression of the disease.
c. It is established that the neurodegenerative process starts in one area of the nervous system and then
spreads to others by a domino effect.
d. Before dying, compromised neurons never loose their functions and phenotypic markers.
e. The distribution and magnitude of neuronal loss in neurodegenerative disorders is often difficult to
establish with certainty.
16. Which of the following statements are most correct?
a. Onset of symptoms reflects the onset of the disease.
b. The prominent symptoms often reveal the main site of neuropathology.
c. Intercurrent infection can permanently exacerbate the symptoms.
d. Neurodegenerative diseases differ from non-degenerative diseases because the latter never progress in a
step-wise manner.
e. b and c
17. Which of the following statements are always correct?
a. Neurodegenerative diseases can involve either the central or the peripheral nervous systems, or both.
b. A familial occurrence of a neurodegenerative disease is the signature of its genetic origin.
c. Cardiovascular problems exacerbate the cognitive decline in patients with dementia and vise-versa.
d. Neurodegenerative diseases are associated with a shortened life-span because they eventually impair
respiratory or cardiovascular functions.
e. When available, symptomatic agents normalize life-span in patients suffering from neurodegenerative
diseases.
18. The transmissibility of a neurodegenerative phenotype can be?
a. Autosomal dominant
b. Autosomal recessive
c. X-linked
d. Mitochondrial
e. All of the above
19. Regarding the classification of neurodegenerative disorders which statement is not correct?
a. The clinico-anatomic classification is quite popular, but is fraught with many shortcomings.
b. The molecular classification groups together neurodegenerative diseases of very different clinical
expression.
c. A parkinsonism can be observed in patients suffering from a wide variety of disorders including
Parkinson’s disease.
d. Members of a same family carrying an identical mutation always exhibit the same clinical and
neuropathological phenotype.
e. A similar clinical phenotype can be caused by distinct etiologic factors.
20. The concept of non-cell autonomy in neurodegeneration implies.
a. A disease phenotype can be transmitted from a mutant cell to a wild-type cell.
b. The whole disease process does not rely solely on pathogenic events taking place in the degenerating cells.
c. The fate of neurons destined to die is, at least in part, determined by other cells such as neighboring glia.
d. This concept applies to infectious diseases but not to neurodegenerative disorders.
e. All but d
17. Neurodegeneration
Serge Przedborski
Answers to Multiple Choice Questions
11.
c
12.
e
13.
b
14.
a
15.
e
16.
e
17.
a
18.
e
19.
d
20.
e
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