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Prion Diseases
See all quizzes of Prion Diseases here:
1 Scrapie agent is synonym of ?
A. Amyloid
B. Prion
C. Myelin
D. None of the above
2 Which of the following about prions is false ?
A. Infectious proteins
B. Cause degeneration of CNS
C. Lacks nucleic acid
D. None of the above
3 The fundamental event underlying prion diseases is ?
A. -to- structural transition in PrP
B. -to- structural transition in PrPSC
C. -to- structural transition in PrP
D. -to- structural transition in PrPSC
4 Prion diseases may manifest as ?
A. Infectious disorders
B. Genetic disorders
C. Sporadic disorders
D. All of the above
5 Disease causing isoform of prion is ?
A. PrPC
B. PrPD
C. PrPSc
D. PrPSd
6 Which of the following statements about prions is false ?
A. Devoid of nucleic acid
B. Prion diseases result from accumulation of PrPSc
C. Pathologic hallmarks of CJD are spongiform degeneration and
astrocytic gliosis
D. CSF is nearly always abnormal in CJD
7 Which of the following is the precursor of PrPSc ?
A. PrP 27-30
B. PrPC
C. PrPD
D. PRNP
8 In humans, PrP gene (PRNP) is located on ?
A. Short arm of chromosome 12
B. Short arm of chromosome 14
C. Short arm of chromosome 16
D. Short arm of chromosome 20
9 Which of the following about prions is false ?
A. Prions reproduce by binding to PrPC
B. PrPC is rich in helix & has little structure
C. PrPSc has less helix & high amount of structure
D. PrP 27-30 is a fragment of PrPC
10 Which is the most common prion disorder in humans ?
A. Sporadic CJD (sCJD)
B. Familial CJD (fCJD)
C. Gerstmann-Straussler-Scheinker disease (GSS)
D. Fatal familial insomnia (FFI)
11 Inherited prion disease caused by mutations in PrP gene is ?
A. Familial CJD (fCJD)
B. Gerstmann-Straussler-Scheinker disease (GSS)
C. Fatal familial insomnia (FFI)
D. All of the above
12 Iatrogenic CJD can be caused by ?
A. Corneal transplantation
B. Contaminated EEG electrode implantation
C. Implantation of duramater grafts
D. All of the above
13 Pathologic hallmarks of Creutzfeldt-Jakob disease (CJD) is ?
A. Spongiform degeneration
B. Astrocytic gliosis
C. Lack of inflammatory response
D. All of the above
14 “Florid plaques” is characteristic feature of ?
A. sCJD (Creutzfeldt-Jakob disease)
B. fCJD
C. vCJD
D. iCJD
15 Dementia with myoclonus is seen in ?
A. Alzheimer’s disease (AD)
B. Unverricht-Lundborg disease
C. Creutzfeldt-Jakob disease (CJD)
D. All of the above
16 Which of the following about myoclonus in CJD is false ?
A. Persists during sleep
B. Elicited by loud sounds
C. Elicited by bright lights
D. None of the above
17 Most patients with CJD survive for what length of time after onset
of clinical signs & symptoms ?
A. 6 – 12 months
B. 12 – 24 months
C. 24 – 48 months
D. 48 – 60 months
18 Clinical abnormalities in CJD involve ?
A. CNS
B. PNS
C. ANS
D. All of the above
19 Which of the following is a finding in CJD ?
A. Fever
B. Leukocytosis
C. Pleocytosis in CSF
D. None of the above
20 Ataxia is a prominent & presenting feature in ?
A. Familial CJD (fCJD)
B. Gerstmann-Straussler-Scheinker disease (GSS)
C. Fatal familial insomnia (FFI)
D. sCJD
21 Insomnia & dysautonomia are a feature of ?
A. Familial CJD (fCJD)
B. Gerstmann-Straussler-Scheinker disease (GSS)
C. Fatal familial insomnia (FFI)
D. vCJD
22 Which of the following is false for Hashimoto’s encephalopathy ?
A. Subacute progressive encephalopathy
B. Tonic-clonic seizures
C. Periodic triphasic complexes on EEG
D. Fluctuations in severity
23 Sterilization for Creutzfeldt-Jakob disease (CJD) contaminated
materials is done by ?
A. Hcl
B. NaOH
C. NaHCo3
D. All of the above
24 Stress protein 14-3-3 is elevated in the CSF of ?
A. Herpes simplex virus encephalitis
B. Multi-infarct dementia
C. Stroke
D. All of the above
25 Stress protein 14-3-3 is elevated in the CSF of all except ?
A. Herpes simplex virus encephalitis
B. Multi-infarct dementia
C. AD
D. CJD
