Test 9

Degenerative diseases. Demyelinative diseases

Questions marked with * are more appropriate for residents.

1. Abnormal Tau deposits are found in:
A. Senile plaques
B. Neuropil threads
C. Both
D. Neither
2. The main component of neurofibrillary tangles is:
A. Tau protein
B. Ubiquitin
C. Synuclein
D. Beta amyloid
*3. Neurofibrillary tangles occur also in:
A. Friedreich ataxia
B. Huntington's disease
C. Creutzfeldt-Jacob disease
D. Progressive supranuclear palsy
4. Dementia can be caused by all of the following except:
A. Creutzfeldt-Jacob disease
B. MS
C. A large MCA infarct
D. Diffuse axonal injury
5. Most cases of Alzheimer's disease are:
A. Autosomal dominant
B. Autosomal recessive
C. Multifactorial
D. Environmental
6. The amount of beta amyloid made by cells of a patient with Down syndrome is:
A. The same as normal people
B. 1.5 times normal
C. 2 times normal
D. 3 times normal
*7. The earliest changes in Alzheimer's disease are usually found in:
A. Hippocampus
B. Entorhinal cortex
C. Amygdala
D. Association cortex
8. A patient with large ventricles, dementia, incontinence, and abnormal gait should:
A . Have a brain biopsy to rule out Alzheimer's disease
B . Have a shunt placed to relieve hydrocephalus
9. Which type of Alzheimer's disease plaque is more important clinically:
A. Diffuse plaque
B. Neuritic plaque
C. Both are equally important
10. The pathology shown in this Bielschowsky silver stain below occurs in:
neurofibrillary tangles
A. In post-traumatic dementia
B. In some cases of Parkinsonism
C. Both
D. Neither, it only occurs in Alzheimer's disease
11. The pathology shown below occurs in:
Creutzfeldt-jacob disease
A. Alzheimer's disease
B. Pick's disease
C. Creutzfeldt-Jacob disease
D. HIV encephalitis
12. The best match for the illustrated changes is:
Huntington's disease
A. An 83 year old person with memory loss and spacial disorientation
B. A 43 year old person with abnormal movements who committed suicide>
C. A 56 year old person with progressive paralysis
D. A 60 year old person with tremor, rigidity and dementia
13. The best match for the illustrated changes is:
Alzheimer's disease
A. A 76 year old person with memory loss and spacial disorientation
B. A 43 year old person with abnormal movements who committed suicide
C. A 56 year old person with progressive paralysis
D. A 60 year old person with tremor, rigidity and dementia
14. The best match for the illustrated changes is:
Parkinson's disease
A. An 83 year old person with memory loss and spacial disorientation
B. A 43 year old person with abnormal movements who committed suicide
C. A 56 year old person with progressive paralysis
D. A 60 year old person with tremor, rigidity and dementia

*15. Axonal swellings in diffuse axonal injury contain:
A. Beta amyloid precursor protein
B. Beta amyloid
C. Both
*16. The CSF in Alzheimer's disease shows:
A. Elevated Tau and decreased beta amyloid
B. Decreased Tau and elevated beta amyloid
*17. Elevation of protein 14-3-3 in CSF occurs in:
A. CJD
B. Alzheimer's disease
C. Both
D. Neither
18. Which of the following APO E genotypes confers the lowest risk for dementia:
A. APO E2/2
B. APO E2/3
C. APO E3/3
D. APO E3/4
E. APO E4/4
19. Many patients with Alzheimer's disease also have cerebral amyloid angiopathy:
A. True
B. False
20. The pathology shown is associated with:
Pick's disease
A. Dementia and severe memory impairment
B. Dementia, abnormal movements, and seizures
C. Dementia and tremor
D. Dementia with language dysfunction
21. Frontotemporal dementias may be accompanied by degeneration of all of the following structures except:
A. The substantia nigra
B. The cerebellum
C. The anterior horns
D. The amygdala
22. Ubiquitin is important for degradation of:
A. Nucleic acids
B. Lipids
C. Carbohydrates
D. Proteins
23. Which of the following is least likely to be affected in Parkinson's disease:
A. The cerebral cortex
B. The nucleus basalis of Meynert
C. The hypothalamus
D. The caudate nucleus
*24. The pathology shown in this ubiquitin immunostain of the cerebral cortex is associated with:
Diffuse Lewy body disease
A. Dementia and hallucinations
B. Dementia and motor neuron disease
C. Dementia and choreoathetosis
D. Dementia and severe memory loss
25. MPTP damages:
A. Lysosomes
B. Mitochondria
C. Proteasomes
D. Nuclei
*26. Parkinsonian manifestations may develop following poisoning with:
A. Manganese
B. Organophosphates
C. Lead
D. Acrylamide
27. Parkinsonian manifestations may develop with all of the following except:
A. HIE
B. Heavy metal poisoning
C. MS
D. Prion disease
28. Which of the following is not a trinucleotide repeat disorder:
29. The pathology shown below is least likely to be caused by:
Denervation atrophy
A. The Guillain-Barre syndrome
B. Diabetic neuropathy
C. Spinal muscular atrophy
D. ALS
30. The pathology shown below causes:
ALS
A. Spasticity and exaggerated reflexes
B. Muscle atrophy
C. Both
D. Neither
*31. The muscle pathology (ATPase stain) shown in this severely hypotonic infant is caused by:
Denervation atrophy
A. GAA trinucleotide repeat
B. SMN1 deletion
C. PMP22 duplication
D. PMP22 deletion
32. The pathology shown is most likely to occur in:
midline cerebellar degeneration
A. A 79 year old demented patient
B. A 60 year old patient with pes cavus and arrhythmias
C. A 61 year old patient with Parkinsonian manifestations
D. A 59 year old patient withextensive intestinal resection
33. Frataxin is located in the:
A. Mitochondria
B. Lysosomes
C. Nucleus
D. Cytosol
*34. Which of the following is not a polyglutamine disorder:
A. Huntington's disease
B. Alzheimer's disease
C. DRPLA
D. Machado-Joseph disease
*35. Which of the following is a polyglutamine disorder:
A. Friedreich's ataxia
B. Autosomal dominant spinocerebellar ataxia
C. OPCA
D. The Fragile X syndrome

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