58. Transmissive spongiform encephalopathies.

Question 1

transmissive spongiorm encephalopathies is also known as ?

Answer 1

prion disease

Question 2

what is the pathophysiology of prion disease

Answer 2

sporadic approach of infections with misfolded PrPsc leads to conformational change of physiological PrPsc - generates condensation areas – plaques forms – decrease of neurones - progression until there is clinical picture of spongiform enecaphlopathy

many tine holes appear in the cortex like a sponge under microscope

Question 3

what are the classical symptoms of prion disease ?

Answer 3

memory change , personality change , motion problem - myclonia
visual disturbnaces

later stages - cannot move or speak - akinetic mutism

Question 4

what is the diagnosis of prion disease ?

Answer 4

the abnormally folded PrPsc protein is found in low level of blood and urin which is easily accessible

final diagnosis is prooved after death

MRI - increase in signals of basal ganglia and the head of caudate nucleus

lumbar puncture

Question 5

what is the medication for prion disease ?

Answer 5

no curative therapy