Prions

Question 1

What are some of the neurocytological lesions you see in prion infection?

Answer 1

spongiform changes, astrocytic gliosis, and sometimes amyloid plaques

Question 2

Vacuoles in gray matter due to loss of neurons is called what?

Answer 2

spongiform changes

Question 3

astroglial enlargement and proliferation is known as what?

Answer 3

astrocytic gliosis

Question 4

What are the main symptoms of Kuru?

Answer 4

cerebellar ataxia, shivering-like tremor, dysarthria, and progresses to complete motor incapacity

Question 5

What are the main symptoms of CJD?

Answer 5

memory loss, depression, behavioral changes, dementia, hallucinations, myoclonus, cerebellar ataxia, cortical blindness (damage to occipital lobes), weakness of certain muscles, slowness of movement, rigidity. Death occurs usually within 6 mo of sx onset

Question 6

What are the 3 methods of developing CJD?

Answer 6

sporadic, inherited (familial) or infectious

Question 7

What are the main differences between Gerstmann-Straussler-Scheinker Syndrome (GSS) and other forms of CJD?

Answer 7

earlier onset (35-55) and illness evolves more slowly (3-5 yrs)